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Systemic Lupus Erythematosus

Systemic lupus erythematosus, abbreviated as SLE, is an autoimmune disease which primarily strikes women.


Lupus band test
Lupus band test
Lupus band test, CC BY 3.0
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Lupus erythematosus, Atlas der Hautkrankheiten
Lupus erythematosus, Atlas der Hautkrankheiten
Lupus erythematosus, Atlas der Hautkrankheiten, Public Domain

Presentation

Majority of the individuals with SLE experience joint pain, accompanied by swelling. Arthritis is also a common complaint. In addition to these, individuals also experience pain in chest, hair loss, and development of sores inside mouth, anemia, malaise, fatigue, fever and sensitivity to light. Affected individuals also develop “butterfly” skin rash. The rash gets worse, when exposed to sunlight, and it commonly appears in areas of bridge of nose and check [8].

Other symptoms of SLE vary with the type of body part that is affected. Individuals also experience numbness, headache, seizures, abdominal pain, arrhythmias, swelling in legs, difficulty in breathing and Raynaud phenomenon [9].

Easy Bruising
  • bruising Anxiety Depression Memory loss[web.archive.org]
  • Sometimes changes in blood counts may contribute to symptoms of fatigue (low red blood cell count, anemia), serious infections (low white blood cell count), or easy bruising (low platelet count).[my.clevelandclinic.org]
  • Continued Sometimes, changes in blood counts (low red cell count, or anemia ), may cause fatigue , serious infections (low white cell count), or easy bruising or bleeding (low platelet count).[webmd.com]
Splenomegaly
  • Presentation SLE is a remitting and relapsing illness, with a variety of different presentations. [ 5 , 6 ] Symptoms and signs are often nonspecific - eg, fatigue (can be severe and debilitating), malaise, fever, splenomegaly, lymphadenopathy, weight[patient.info]
  • Splenomegaly occurs in 10% of patients. Neurologic symptoms can result from involvement of any part of the central or peripheral nervous system or meninges. Mild cognitive impairment is common.[merckmanuals.com]
  • Splenomegaly occurs in 10% of patients. Neurologic manifestations Neurologic symptoms can result from involvement of any part of the central or peripheral nervous system or meninges. Mild cognitive impairment is common.[merckmanuals.com]
  • ( Lrdm1 and Lrdm2 ) mapped to murine chromosomes 7 and 12, as well as the lpr mutation to murine chromosome 19. 135 Vidal et al 25 later attempted a genome scan using the (MRL- Fas lpr B6-Fas lpr )F 2 intercrossed strain to examine the phenotype of splenomegaly[doi.org]
Generalized Lymphadenopathy
  • Systemic lupus erythematosus with an unusual presentation of generalized lymphadenopathy was diagnosed in both patients.[ncbi.nlm.nih.gov]
Fever
  • PATIENT CONCERNS: The patient showed a high spiking fever and myalgia. Laboratory data revealed pancytopenia and immunological abnormalities.[ncbi.nlm.nih.gov]
  • This FDG distribution pattern should be kept in mind when PET is performed for etiological investigation of fever of unknown origin.[ncbi.nlm.nih.gov]
  • We present a case of a previously healthy, 17 year old Asian female who presented to the ED with 14 days of fever and fatigue after administration of the meningococcal vaccine, as well as 5 days of facial rash.[ncbi.nlm.nih.gov]
  • CASE: A 16-year-old male was referred to us with fever, a 1-month history of violaceous red papules and erythematous plaques on his face and a painful nodule in his right neck.[ncbi.nlm.nih.gov]
  • Prolonged fever was the most common non-specific symptom found in 27 (84%), followed by pallor in 13 (41%) patients. Twenty-two (69%) children were found to be anemic and 18 patients (56%) having signs of arthritis at presentation.[ncbi.nlm.nih.gov]
Fatigue
  • Disease activity as measured by SLE Disease Activity Index (SLEDAI) did not significantly predict self-reported levels of fatigue. Medication usage did not predict fatigue levels. Pain and depression were both unique positive predictors of fatigue.[ncbi.nlm.nih.gov]
  • The study identified that pain (p 0.001), body mass index (p 0.026), Chalder's fatigue scale (p 0.001), fatigue severity scale (p 0.001), and anxiety (p 0.001) are all positively correlated with depression in SLE patients.[ncbi.nlm.nih.gov]
  • However, 24% of the patients reported high degree of SLE-related pain, more fatigue, anxiety and depression, and worse health-related quality of life (HRQoL).[ncbi.nlm.nih.gov]
  • It also suggests that pediatricians should be aware of occult onset of SLE, such as unclear marasmus and fatigue found in this case.[ncbi.nlm.nih.gov]
  • Fatigue Chronic fatigue is a common symptom in SLE and usually does not relate to objective muscular effort (i.e., walking up stairs may seem no harder than walking on level ground). [20] Fatigue may contribute to both self-perceived and to measurable[emedicine.medscape.com]
Anemia
  • She was diagnosed with SLE with pernicious anemia and iron deficiency anemia. The rare association of SLE with pernicious anemia was reported previously in few patients. Treatment of SLE along with B12 supplementation is necessary for such patients.[ncbi.nlm.nih.gov]
  • Anemia is a common finding in children with systemic lupus erythematosus (SLE). Anemia in SLE may result from several mechanisms and more than one may be operative at any time.[ncbi.nlm.nih.gov]
  • […] assoc w SLE Hemolytic anemia associated with systemic lupus erythematosus Hemolytic anemia with Systemic lupus erythematosus Inflammatory myopathy due to lupus Inflammatory myopathy due to SLE Lung disease with systemic lupus erythematosus Lupus erythematosus[icd9data.com]
  • The most common laboratory finding was anemia, found in 22 (69%) of cases.[ncbi.nlm.nih.gov]
  • The further clinical course was complicated by anemia, chest wall shingles, hypertension, and progressive cervical shortening.[ncbi.nlm.nih.gov]
Raynaud Phenomenon
  • phenomenon, pulmonary, hematological, gastrointestinal, ocular.[web.archive.org]
  • phenomenon) Shortness of breath Chest pain Dry eyes Easy bruising Anxiety Depression Memory loss[web.archive.org]
  • ’s phenomenon) mouth or nose ulcers Many of these symptoms occur in other illnesses besides lupus.[web.archive.org]
  • Raynaud phenomenon, arthritis, and leukopenia were more common in women, whereas skin manifestations, serositis, and renal involvement were more common in men.[ncbi.nlm.nih.gov]
Weight Loss
  • But, in general, lupus signs and symptoms may include: Fatigue Fever Weight loss or gain Joint pain, stiffness and swelling Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose Skin lesions that appear or worsen[web.archive.org]
  • The most common depressive symptoms in participants were fatigue and weakness (88.2%), irritability (82.3%), sadness (77.6%), and somatic preoccupation (76.4%), while the least common symptoms were weight loss (34.1%), low level of energy (28.2%), and[ncbi.nlm.nih.gov]
  • A 50-year-old woman of Chinese origin came to the emergency room with symptoms of high fever, nausea, vomiting and weight loss of 10 kg. Three months before, an episode with abdominal pain due to ileal invagination in the colon occurred.[ncbi.nlm.nih.gov]
  • INTERVENTIONS: An 82-year-old woman complained about anorexia, weight loss, fatigue, and mild night fever.[ncbi.nlm.nih.gov]
  • Prior to admission, she had also had a cough, fever and weight loss and was under investigation for suspected TB infection.[ncbi.nlm.nih.gov]
Pleural Effusion
  • Antinuclear antibodies were subsequently found in the serum and in the pleural effusion. Anti-nucleosome antibodies were also present without antiphospholipid antibodies.[ncbi.nlm.nih.gov]
  • Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage.[ncbi.nlm.nih.gov]
  • We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion.[ncbi.nlm.nih.gov]
  • Pleural effusions may occur, and the lung parenchyma may be involved. These conditions have only recently been recognized and differentiated; accurate diagnosis has been much improved by refinements in radiological methods, by the use… Read More[britannica.com]
Dyspnea
  • INTERVENTIONS: Initially the patient was treated for medically unexplained dyspnea (MUD) without much improvement.[ncbi.nlm.nih.gov]
  • Patients present with dyspnea alone or associated with chest pain and orthopnea, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on pulmonary function tests.[ncbi.nlm.nih.gov]
  • We describe the case of a child, aged 14 years, with fever, asthenia, haemoptysis, dyspnea, anaemia, increased inflammatory markers, positivity to ANA, nDNA, direct Coombs tests, anticardiolipin antibodies and complement factors consumption.[ncbi.nlm.nih.gov]
  • His vital signs were stable. 5 days later, his condition aggravated and mechanical ventilation was necessitated owing to severe dyspnea.[ncbi.nlm.nih.gov]
Pleuritic Pain
  • Serositis a) Pleuritis--convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion OR b) Pericarditis--documented by ECG or rub or evidence of pericardial effusion 7.[pedclerk.bsd.uchicago.edu]
  • Acute lupus pneumonitis presents as cough, dyspnea, pleuritic pain, hypoxemia, and fever. Infiltrates on chest radiographs may be unilateral or bilateral.[emdocs.net]
  • Typically, the patient seeks medical help for relief of fever, weight loss, joint pain, the characteristic butterfly rash, pleural effusion and pleuritic pain, and nephritis.[medical-dictionary.thefreedictionary.com]
  • SLE can cause pleuritic pain as well as inflammation of the pleurae known as pleurisy, which can rarely give rise to shrinking lung syndrome involving a reduced lung volume.[en.wikipedia.org]
Hepatomegaly
  • Other signs are: Pancreatitis, Lupus Enteropathy, Hepatitis and Hepatomegaly 5. Systemic Fever, Malaise/Fatigue, Lymphadenopathy, Weight loss 6. Cardio-Vascular Pericarditis is the most common cardiac manifestation, occurs up to 30% of patients.[fidanoski.ca]
Psychiatric Manifestation
  • In clinical practice, differential diagnosis of the two diseases is necessary for patients with hepatic, neurological, and psychiatric manifestations.[ncbi.nlm.nih.gov]
  • Patients with SS or SLE present with various neurological symptoms and psychiatric manifestations.[ncbi.nlm.nih.gov]
  • This study investigates the interplay between physical and psychiatric manifestations of lupus. To this end, an SLE patient cohort was examined for correlations between clinical presentation, laboratory tests, and psychological indicators.[ncbi.nlm.nih.gov]
  • Psychiatric manifestations in systemic lupus erythematosus. Autoimmun Rev, 2007, 6(6):421–426 PubMed CrossRef Google Scholar 3. Ogawa M, Ishimaru K, Shiroto T, et al.[dx.doi.org]
Kidney Failure
  • Kidney inflammation in SLE can cause leakage of protein into the urine, fluid retention, high blood pressure, and even kidney failure . This can lead to further fatigue and swelling of the legs and feet.[web.archive.org]
  • Overwhelming infection and kidney failure are the two most common causes of death in people with lupus. Recently there is new information which indicates heart disease may be another leading cause of death among people with lupus.[web.archive.org]
  • Historically, lupus caused people to die young, primarily from kidney failure. Today, with careful treatment, 80 to 90 percent of people with lupus can expect to live a normal lifespan.[healthline.com]
  • Lupus most often affects your skin, joints and kidneys — which can lead to kidney damage and kidney failure. More than 16,000 new cases are reported each year in the United States.[hopkinsmedicine.org]
Hematuria
  • Immunosuppressant therapy relieved him of the edema and hematuria, but he regained the symptoms after a cold.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: In this study, we describe a 55 year old patient who developed hematoma and hematuria due to acquired hemophilia secondary to systemic lupus erythematosus (SLE). Then, she developed arthritis and thrombosis after some evaluations.[ncbi.nlm.nih.gov]
  • She also had concomitant massive hematuria and proteinuria, fever, multiple positive autoimmune antibodies, hypocomplementemia, abnormal lumbar puncture findings and evidence of Sjögren syndrome, which are all rare in WD.[ncbi.nlm.nih.gov]
  • Laboratory findings on admission revealed proteinuria, microscopic hematuria, anemia, leukopenia, hypoalbuminemia, hypocomplementemia, and slightly elevated levels of serum creatinine.[ncbi.nlm.nih.gov]
  • Script Download on iTunes Related Content: Podcast: Approach to Pediatric Rashes Podcast: Approach to Pediatric Anemia Part 1 and Part 2 Podcast: Evaluation of Hematuria Podcast: Evaluation of Proteinuria Podcast: Juvenille Idiopathic Arthritis[pedscases.com]
Seizure
  • A seizure focus may result from an acute insult or from the development of a seizure focus in an area of previous brain insult. Partial or secondarily generalized seizures are most common, but all seizure types have been reported.[emedicine.medscape.com]
  • We found that APS was strongly associated with neurological manifestations and in particular with cerebrovascular diseases and seizures.[ncbi.nlm.nih.gov]
  • All of these seizures were generalized, with focal onset in five. 3 In another series of 161 SLE patients, 16 (10%) had seizures during the entire course of illness.[epilepsy.com]
  • Fits or seizures are one of the non-specific ways the brain reacts to severe illness. Once the lupus is treated further fits are the exception rather than the rule. Movement disorders The same applies to movement disorders.[web.archive.org]
Headache
  • The first patient presented with significant headache at time of SLE diagnosis and negative brain imaging studies on initial evaluation. The headache recurred with findings of cerebellar ataxia and obstructive hydrocephalus.[ncbi.nlm.nih.gov]
  • Neurological syndromes occurring most frequently in SLE patients include headache, polyneuropathy and cerebrovascular diseases. Antiphospholipid syndrome increases the risk of nervous system involvement.[ncbi.nlm.nih.gov]
  • Headaches Headaches are common in lupus. In some patients a history of headache going back to their early teens is a feature of the disease. They may be a part of the lupus itself or may be associated with a clotting syndrome.[web.archive.org]
  • Headaches Headaches are common in lupus. In some patients a history of headache going back to their early teens is a feature of the disease. They may be a part of the lupus itself or may be associated with a clotting (antiphospholipid) syndrome.[uklupus.co.uk]
Confusion
  • The clinical triad of mild confusion, ataxia and ophthalmoplegia also raised the possibility of Wernicke's encephalopathy (WE). The diagnosis of WE was further supported by the magnetic resonance imaging features.[ncbi.nlm.nih.gov]
  • When these two conditions happened to have similar clinical spectrum features, the confusion with each other occurred.[ncbi.nlm.nih.gov]
  • DILE Should Not Be Confused With Medication Side Effects Drug-induced lupus should not be confused with the drug side-effects that often occur after short-term therapy for gastrointestinal, neurologic, or allergic symptoms.[web.archive.org]
  • Despite this, she developed confusion, dizziness, blurred vision and fluctuating consciousness. Magnetic resonance imaging (MRI) and lumbar puncture revealed CNS TB infection resulting in meningitis.[ncbi.nlm.nih.gov]
  • Confusion. Seizures. What causes it? What causes systemic lupus erythematosus (lupus)? No one knows what causes systemic lupus erythematosus (lupus).[niams.nih.gov]
Peripheral Neuropathy
  • GBS is an autoimmune peripheral neuropathy usually triggered by an antecedent bacterial or viral infection, with SLE being a rare cause.[ncbi.nlm.nih.gov]
  • For neurologic manifestations, females with SLE experienced more psychiatric symptoms and headaches, whereas males with SLE experienced more seizures and peripheral neuropathy.[ncbi.nlm.nih.gov]
  • Peripheral neuropathy Peripheral neuropathy occurs in as many as 18% of patients with SLE.[emedicine.medscape.com]
  • Moreover, peripheral neuropathy can also occur in Sjögren's syndrome (SS) regardless of the presence or absence of vasculitis.[doi.org]
  • Neurologic system involvement may create painful peripheral neuropathy or headache syndromes. Raynaud’s phenomenon occurs in many people with SLE and can be painful.[practicalpainmanagement.com]
Guillain-Barré Syndrome
  • It may cause neurologic damage which is mainly characterized by central and mental system, while peripheral sexual damage is relatively rare in which Guillain-Barré Syndrome (GBS) as the first performance is more rare .[ncbi.nlm.nih.gov]
  • Additional symptoms include demyelinating syndrome, movement disorders, a confusional state, aseptic meningitis, Guillain-Barré syndrome, plexopathy, myelopathy, cranial neuropathy and myasthenia gravis.[pubs.sciepub.com]
  • […] papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents.More rare manifestations are acute confusional state, Guillain–Barré[en.wikipedia.org]
  • Kamatani , Guillain-Barré syndrome accompanied by central nervous system lupus in a patient with juvenile rheumatoid arthritis , Modern Rheumatology , 11 , 2 , (155) , (2001) . George A. Heckman, Alexandra Papaioannou, Jonathan D.[doi.org]
Nausea
  • Gastrointestinal manifestations of SLE are described as being common in SLE, with nausea and vomiting occurring in 50% of cases in some series. Poor eating habits and vomiting are well-described causes of non-alcoholic WE.[ncbi.nlm.nih.gov]
  • A 50-year-old woman of Chinese origin came to the emergency room with symptoms of high fever, nausea, vomiting and weight loss of 10 kg. Three months before, an episode with abdominal pain due to ileal invagination in the colon occurred.[ncbi.nlm.nih.gov]
  • Side/Adverse Effects Dermatologic: rash, mild skin reaction at injection site Gastrointestinal: abdominal cramps, nausea, vomiting Musculoskeletal: chest, back or hip pain; muscle pain; joint pain Neurologic: anxiety, chills, dizziness, fever, headache[web.archive.org]
  • PATIENTS CONCERNS: The 23-year nulliparous patient was admitted to a district hospital with abdominal pain, nausea, vomiting and bloody diarrhea at a gestational age (GA) of 10 weeks.[ncbi.nlm.nih.gov]
Vomiting
  • Gastrointestinal manifestations of SLE are described as being common in SLE, with nausea and vomiting occurring in 50% of cases in some series. Poor eating habits and vomiting are well-described causes of non-alcoholic WE.[ncbi.nlm.nih.gov]
  • Here is a case of a 40-year lady who presented with diarrhea and vomiting that was unresponsive to treatment with intravenous antibiotics.[ncbi.nlm.nih.gov]
  • She also had associated abdominal pain and vomiting. Imaging revealed a gall bladder (GB) mass with hepatic extension, which was histologically consistent with adenocarcinoma of the GB. Subsequently she succumbed to death during chemotherapy.[ncbi.nlm.nih.gov]
  • A 50-year-old woman of Chinese origin came to the emergency room with symptoms of high fever, nausea, vomiting and weight loss of 10 kg. Three months before, an episode with abdominal pain due to ileal invagination in the colon occurred.[ncbi.nlm.nih.gov]
  • However, diarrhea, vomiting, and fever occurred for approximately 3 months.[ncbi.nlm.nih.gov]
Eye Pain
  • […] which are the same for females and males, are: extreme fatigue (tiredness) headaches painful or swollen joints fever anemia (low numbers of red blood cells or hemoglobin, or low total blood volume) swelling (edema) in feet, legs, hands, and/or around eyes[web.archive.org]
Arthritis
  • The term for this is arthritis. In the affected joint, arthritis can cause: pain swelling tenderness a feeling of warmth fluid collection. Pain in and around the joints is not always due to lupus arthritis.[web.archive.org]
  • Arthritis Excerpt Complete Article Rheumatoid Arthritis Remission Rheumatoid Arthritis Response Sjögren's Syndrome Classification 2016 ACR/EULAR Sjögren's Syndrome Classification Criteria Complete Article 2012 ACR Sjögren's Syndrome Classification Criteria[rheumatology.org]
  • Arthritis Rheum 1997; 40: 804-808. Costenbader KH, Feskanich D, Stampfer MJ, et al. Reproductive and menopausal factors and risk of systemic lupus erythematosus in women. Arthritis Rheum 2007; 56: 1251-1262. . Petri M.[dx.doi.org]
  • Arthritis Rheum 2009 ; 61: 1396 – 1402 . Google Scholar Medline 6. Manzi, S, Selzer, F, Sutton-Tyrell, K. Prevalence and risk factors of carotid plaque in women with systemic lupus erythematosus . Arthritis Rheum 1999 ; 42: 51 – 60 .[dx.doi.org]
  • Arthritis Rheum 2001 ; 45: 191 – 202 . Google Scholar Medline[doi.org]
Arthralgia
  • Here we report a case in which an elderly female presented with malar rash, intermittent fever, and arthralgia. Her diagnosis was significantly delayed due to a close clinical resemblance to systemic lupus erythematosus.[ncbi.nlm.nih.gov]
  • While classic manifestations consist of arthralgia, myalgia, frank arthritis, a malar rash and renal failure to name a few, cardiac tamponade, however, is a far less common and far more dangerous presentation.[ncbi.nlm.nih.gov]
  • She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone.[ncbi.nlm.nih.gov]
  • A 42-year-old woman was admitted to our hospital with complaints of exanthema, arthralgia, shortness of breath, and hemoptysis.[ncbi.nlm.nih.gov]
  • It seems that signs of nephropathy, elevated ANA-titers and arthralgias may serve as prognostic predictors for transition into SLE. Characteristic features of cutaneous LE are photosensitivity and female predominance.[ncbi.nlm.nih.gov]
Joint Swelling
  • While denying a history of any rash, photosensitivity, oral ulcers, or seizures, his physical examination did reveal metacarpal phalangeal joint swelling along with noted pulsus paradoxus of 15-200mmHg.[ncbi.nlm.nih.gov]
  • Your doctor will ask about symptoms like: Fatigue (feeling tired often) Painful or swollen joints Swelling in the hands, feet, or around the eyes Headache Low-grade fever Sensitivity to sunlight or fluorescent light Chest pain when breathing deeply Learn[lupus.org]
  • swelling and synovitis hand and wrist manifestation are common (90%) swelling and synovitis of PIPs, MCPs, and carpus ligamentous laxity Raynaud's phenomenon dorsal subluxation of ulna at DRUJ Imaging Studies Radiographs usually no evidence of joint[orthobullets.com]
  • Common symptoms include: severe fatigue joint pain joint swelling headaches a rash on the cheeks and nose, which is called a “butterfly rash” hair loss anemia blood-clotting problems fingers turning white or blue and tingling when cold, which is known[healthline.com]
Joint Stiffness
  • Joint stiffness is common and is usually worse first thing in the morning. Mild joint swelling may occur but severe arthritis with joint damage is unusual.[patient.info]
  • Heat packs relieve joint stiffness and pain, and regular gentle exercise helps to maintain full range of motion. Physical and occupational therapy consultations are provided as appropriate.[medical-dictionary.thefreedictionary.com]
Joint Effusion
  • effusions; can occur in 80% of cases 1 pleuritis and/or pericarditis: present in 35% (range 17-50%) of cases 1 proteinuria: 0.5 grams/day haematologic abnormality hemolytic anemia or leukopenia: 3 on more than two occasions lymphopenia: 3 on more than[radiopaedia.org]
Photosensitivity
  • In addition to corroborating the lower risk of musculoskeletal involvement, in the latter study men were protected from malar rash, photosensitivity, oral ulcers, alopecia and RP ( P 0.05).[doi.org]
  • The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity .[web.archive.org]
  • We reported two cases of SLE with psoriasis vulgaris with clinical manifestations as recurrent erythroderma with photosensitivity.[ncbi.nlm.nih.gov]
  • While denying a history of any rash, photosensitivity, oral ulcers, or seizures, his physical examination did reveal metacarpal phalangeal joint swelling along with noted pulsus paradoxus of 15-200mmHg.[ncbi.nlm.nih.gov]
  • […] anemia (low numbers of red blood cells or hemoglobin, or low total blood volume) swelling (edema) in feet, legs, hands, and/or around eyes pain in chest on deep breathing (pleurisy) butterfly-shaped rash across cheeks and nose sun- or light-sensitivity (photosensitivity[web.archive.org]
Skin Lesion
  • She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone.[ncbi.nlm.nih.gov]
  • Various skin lesions are seen, which can resolve or leave scars; the syndrome usually resolves; however, cardiac manifestations can be fatal.[medical-dictionary.thefreedictionary.com]
  • But, in general, lupus signs and symptoms may include: Fatigue Fever Weight loss or gain Joint pain, stiffness and swelling Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose Skin lesions that appear or worsen[web.archive.org]
  • We describe a highly unusual case of SLE occurring after prodromal idiopathic thrombocytopenic purpura (ITP) and presenting with skin lesions more typical of malignant atrophic papulosis, a rare and often fatal vasculopathy.[ncbi.nlm.nih.gov]
  • There was complete remission with dapsone, with no recurrence of skin lesions throughout one year of follow-up. We highlight the rarity and early age of occurrence.[ncbi.nlm.nih.gov]
Discoid Rash
  • Malar rash – a rash over the cheeks and nose, often in the shape of a butterfly Discoid rash – a rash that appears as red, raised, disk-shaped patches Photosensitivity – a reaction to sun or light that causes a skin rash to appear or get worse Oral ulcers[web.archive.org]
  • Symptoms may include: malar rash- a rash shaped like a butterfly that is usually found on the bridge of the nose and the cheeks. discoid rash- a raised rash found on the head, arms, chest, or back. raynaud's phenomenon- a condition in which the blood[web.archive.org]
  • A raised oval discoid rash is also seen in some cases. Diagnosis For diagnosis of SLE various tests are done including blood tests, urine analysis, chest X-ray and kidney biopsy.[rheumatologyspecialistcare.com.au]
  • Lupus is diagnosed when four of the following 11 criteria are present in a patient: Malar “butterfly” rash across the nose and cheeks Rash consisting of raised red patches (discoid rash) Rash resulting from sensitivity to the sun (photosensitivity) Ulcers[emedicinehealth.com]
  • Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions 3.[pedclerk.bsd.uchicago.edu]
Erythema
  • In 1851 Cazenave renamed erythema centrifugum, calling it lupus erythematosus and gave a classic description of discoid lupus erythematosus.[ncbi.nlm.nih.gov]
  • The patient reported that hand swelling and erythema developed soon after the diagnosis of SLE was established. This episode resolved quickly without recurrence or immediate sequelae.[ncbi.nlm.nih.gov]
  • PATIENT CONCERNS: A 16-year-old girl presenting with polyarthritis, malar rash, and palmar erythema was indicated for steroid therapy on the basis of positive results for antinuclear, anti-Smith, and antiphospholipid antibodies, which confirmed the diagnosis[ncbi.nlm.nih.gov]
  • 8 - 10 Due to the difficulties encountered in diagnosis, some criteria were proposed 7 for guidance and the patient in case 1 presented enough features to be diagnosed with DIL: continuous use of the drug for at least 60 days; sudden and persistent erythema[doi.org]
Alopecia
  • Thai males have also been shown to have less arthralgia than females in addition to less RP and alopecia and more prevalent thrombocytopenia ( P 0.05 for all) [ 18 ].[doi.org]
  • […] may include: Fatigue Fever Weight loss or gain Joint pain, stiffness and swelling Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose Skin lesions that appear or worsen with sun exposure Mouth sores Hair loss (alopecia[web.archive.org]
  • […] successively excluded patients with (1) at least one "SLE-specific" manifestation (biopsy-proven SLE nephropathy, arthritis, cutaneous, or neurologic SLE manifestations, pericarditis, autoimmune haemolytic anaemia, oral and nasal ulcers, non-scarring alopecia[ncbi.nlm.nih.gov]
  • Longitudinal analysis indicated that the disease related complaint alopecia (p 0.008) and relationship assessment scale scores (p 0.004) may also be correlated to depression in SLE patients.[ncbi.nlm.nih.gov]
  • Diffuse thinning or hair fragility with visible broken hairs (in the absence of other causes such as alopecia areata, drugs, iron deficiency, and androgenic alopecia) Oral or nasal ulcers Palate, buccal, and tongue ulcers or Nasal ulcers (in the absence[merckmanuals.com]
Malar Rash
  • Here we report a case in which an elderly female presented with malar rash, intermittent fever, and arthralgia. Her diagnosis was significantly delayed due to a close clinical resemblance to systemic lupus erythematosus.[ncbi.nlm.nih.gov]
  • KEYWORDS: Antinuclear antibody; Jaccoud arthropathy; Lupus nephritis; Malar rash; Pericarditis; Pleuritis; Systemic lupus erythematosus[ncbi.nlm.nih.gov]
  • But, in general, lupus signs and symptoms may include: Fatigue Fever Weight loss or gain Joint pain, stiffness and swelling Butterfly-shaped rash (malar rash) on the face that covers the cheeks and bridge of the nose Skin lesions that appear or worsen[web.archive.org]
  • While classic manifestations consist of arthralgia, myalgia, frank arthritis, a malar rash and renal failure to name a few, cardiac tamponade, however, is a far less common and far more dangerous presentation.[ncbi.nlm.nih.gov]
  • Malar rash – a rash over the cheeks and nose, often in the shape of a butterfly Discoid rash – a rash that appears as red, raised, disk-shaped patches Photosensitivity – a reaction to sun or light that causes a skin rash to appear or get worse Oral ulcers[web.archive.org]
Rash of the Nose
  • SLE also causes a characteristic butterfly shaped rash on the nose and cheeks called malar rash with exposure to the sun. A raised oval discoid rash is also seen in some cases.[rheumatologyspecialistcare.com.au]
  • Lupus is diagnosed when four of the following 11 criteria are present in a patient: Malar “butterfly” rash across the nose and cheeks Rash consisting of raised red patches (discoid rash) Rash resulting from sensitivity to the sun (photosensitivity) Ulcers[emedicinehealth.com]
  • An erythematous rash ("butterfly rash") over the nose and malar eminences, weakness, fatigue, and weight loss also are frequently seen early in the disease.[medical-dictionary.thefreedictionary.com]
Facial Redness

Workup

A preliminary physical examination is done, to carefully study the signs and symptoms of the disease. To be diagnosed with SLE, the individuals should exhibit at least 4 out of 11 common signs and symptoms of the disease.

Antinuclear antibody test is done using the indirect immunofluorescence technique. The pattern of flurorescence that is obtained suggests SLE. In addition, complete blood count, chest X-ray, urinalysis and kidney function tests are also carried out. Depending on the organ involved, other tests would also be required to arrive at a definite conclusion [10].

Anergy
  • […] leads to X-linked agammaglobulinemia and a complete lack of B cells. 36 However, deficiency of Btk in mice is associated with a much milder phenotype. 37 B-cell–receptor signaling is important for establishing the B-cell repertoire through induction of anergy[nejm.org]
Pericardial Effusion
  • Pericardial mesothelioma accounts for only 0.7 % of all malignant mesotheliomas and it usually presents with pericardial effusion, mimicking serositis.[ncbi.nlm.nih.gov]
  • A bedside echocardiogram revealed a significant pericardial effusion concerning for pericardial tamponade. An emergent pericardiocentesis performed drained 800mL of serosanguinous fluid.[ncbi.nlm.nih.gov]
  • Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage.[ncbi.nlm.nih.gov]
  • We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion.[ncbi.nlm.nih.gov]
  • In addition to ascites, pleural and pericardial effusions had developed. DIAGNOSIS: Diagnosis of SLE was finally established at GA 16 after an autoimmune workup revealed positive antinuclear, anti- Sm, anti-dsDNA and anti-U1RNP antibodies.[ncbi.nlm.nih.gov]
Antinuclear Autoantibodies
  • Press RI, Peebles CL, Kumagai Y, Ochs RL, Tan EM (1992) Antinuclear autoantibodies in women with silicone breast implants. Lancet 340:1304–1307 PubMed CrossRef Google Scholar 57.[dx.doi.org]
  • In the second phase, autoantigens (nucleic acid–protein complexes) and antinuclear autoantibodies form complexes serving as secondary IFN-α inducers.[doi.org]
  • The Sle1 z interval is home to three subloci: Sle1a z , Sle1b z and Sle1c z 22 Among these, the NZM2410/NZW-derived ‘ z ’ allele of Sle1b z leads to the highest levels and penetrance of antinuclear autoantibodies (ANAs). 23 , 24 Studies employing crosses[dx.doi.org]
Fibrinoid Necrosis
  • necrosis at dermoepidermal junction with liquefactive degeneration and atrophy of epidermis More mucin deposition in reticular dermis than discoid lupus Edema, small hemorrhages and a mild infiltrate of inflammatory cells, principally lymphocytes, in[pathologyoutlines.com]
HLA-DR3
  • MCPs, and carpus ligamentous laxity Raynaud's phenomenon dorsal subluxation of ulna at DRUJ Imaging Studies Radiographs usually no evidence of joint destruction osteonecrosis of hips is common Labs Usually positive for ANA (95%) anti-DNA antibodies HLA-DR3[orthobullets.com]
  • It is important to note that the HLA-DQ2 haplotype found in 95 % of celiac patients shares a strong linkage to the HLA-DR3 haplotype associated with AIH [ 52 ].[dx.doi.org]
  • A significant increase of the C4B null allele ( C4B*Q0 ) in lupus patients has been demonstrated in a Spanish SLE population (OR: 6.0). 61 The C4A null allele is in linkage disequilibrium with HLA-DR3. 62 However, C4A allelic associations with SLE have[doi.org]
  • […] associations with SLE (TNFAIP3, FAM167A-BLK, BANK1 and KIAA1542); however, those loci had a lower significance level and a lower contribution to individual risk for SLE. [33] Studies of human leukocyte antigens (HLAs) reveal that HLA-A1, HLA-B8, and HLA-DR3[emedicine.com]
HLA-B8
  • Factors leading to SLE include: Genetic predisposition, incuding haplotype HLA -B8, -DR 3 Exposure to sunlight Viral infection , particularly Epstein-Barr virus Hormones Toxins such as cigarette smoke Drugs in drug-induced LE Emotional upset The manifestations[dermnetnz.org]
  • […] other loci associations with SLE (TNFAIP3, FAM167A-BLK, BANK1 and KIAA1542); however, those loci had a lower significance level and a lower contribution to individual risk for SLE. [33] Studies of human leukocyte antigens (HLAs) reveal that HLA-A1, HLA-B8[emedicine.com]
HLA-DR2
  • Specific combinations of HLA-DR2 and DR3 class II haplotypes contribute graded risk for disease susceptibility and autoantibodies in human SLE. Eur J Hum Genet. 2007; 15 :823–830. [ PubMed ] [ Google Scholar ] 17.[ncbi.nlm.nih.gov]
  • Specific combinations of HLA-DR2 and DR3 class II haplotypes contribute graded risk for disease susceptibility and autoantibodies in human SLE. Eur J Hum Genet 2007 ;15: 823 - 830 7. Sigurdsson S, Nordmark G, Goring HH, et al.[nejm.org]
HLA-DRw3
  • HLA-DRw3 and systemic lupus erythematosus Arthritis Rheum 1980 23 : 954–957 50. So AK , Fielder AH , Warner CA , Isenberg DA , Batchelor JR , Walport MJ .[doi.org]
Pleural Effusion
  • Antinuclear antibodies were subsequently found in the serum and in the pleural effusion. Anti-nucleosome antibodies were also present without antiphospholipid antibodies.[ncbi.nlm.nih.gov]
  • Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage.[ncbi.nlm.nih.gov]
  • We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion.[ncbi.nlm.nih.gov]
  • Pleural effusions may occur, and the lung parenchyma may be involved. These conditions have only recently been recognized and differentiated; accurate diagnosis has been much improved by refinements in radiological methods, by the use… Read More[britannica.com]
Pleural Rub
  • rub or Typical pericardial pain (pain with recumbency improved by sitting forward) for 1 day, pericardial effusion, pericardial rub, or pericarditis by electrocardiography in the absence of other causes (eg, infection, uremia, Dressler syndrome) Renal[merckmanuals.com]

Treatment

Systemic lupus erythematosus cannot be cured. The symptoms can however be managed, with effective treatment regimes. For mild forms, treatment includes administration of non-steroidal anti-inflammatory drugs (NSAIDs) which are indicated for joint pain, and pleurisy. For skin rashes, corticosteroid creams are also given for topical application.

For severe forms of SLE, high doses of corticosteroids are administered along with cytotoxic drugs. Cytotoxic drugs work by blocking the cell growth and by suppressing the immune system. These drugs are recommended, when corticosteroids do not seem to produce favorable results [11].

Prognosis

The past few decades have witnessed a significant decline in the mortality rates in patients with SLE [6]. It has also been estimated, that with introduction of better treatment methods, about 80% individuals have a 15–year survival rate and more than 90% individuals have a 10-year survival rate. The prognosis of the disease depends on the severity of the condition. Individuals with mild symptoms generally do well with proper treatment. Women with SLE can successfully become pregnant and deliver a baby. However, individuals with underlying disease conditions have poor outcome [7].

Etiology

The exact cause that triggers the immune system to behave in an abnormal fashion is not clearly understood. However, several factors that can trigger the development of SLE are as follows:

  • Genetic predisposition: Individuals with family history of SLE are at an increased risk of developing the same.
  • Environmental stimuli such as exposure to ultraviolet rays, stress, drugs, trauma and viral agents, are known to play a role in causation of the disease.
  • Hormonal involvement: The hormone estrogen can to some extent, trigger attacks of SLE in women of child bearing age [2].

Epidemiology

Annually, about 5 cases of SLE occur in 100,000 populations. According to CDC, in the US, about 1.8 to 7.6 cases occur per 100,000 persons every year. As per the statistics provided by the Lupus Foundation of America, it has been estimated that about 1.5 million Americans live with SLE [3].

SLE is a common phenomenon amongst the women population. In addition, black women are 4 times more likely to develop the autoimmune disease than white women [4].

Sex distribution
Age distribution

Pathophysiology

Under normal conditions, the immune system produces antibodies that protect the body against infections. SLE occurs due to abnormalities in apoptosis, which disturbs the immune tolerance ability and speeds up cell death. T cells also have a major role to play in development of SLE. Abnormalities in T cells cause defect in signaling which in turn, leads to development of abnormalities in immune response [5].

In the condition of SLE, the body’s immune system attacks the healthy cells, considering them as invaders, and in the process destroys them. Such sequence of events causes tissue damage, giving rise to various symptoms of swelling and pain. Interplay of environment and genetic factors, are known to play a role in causation of SLE.

Prevention

Onset of SLE cannot be prevented; however, the associated complications can be kept at bay by following certain preventive steps. It is necessary, that individuals with SLE should be closely monitored for several other disease conditions. Individuals should receive all immunizations to prevent development of new disorders. In addition, they are also advised to get regularly tested for osteoporosis. SLE also predisposes an individual to develop heart diseases, and therefore preventive steps should be taken to protect the heart [12].

Summary

In women, systemic lupus erythematosus (SLE) usually begins during the child bearing age. SLE can affect the joints, brain, kidney and other body organs. Apart from women of child bearing age, the condition can also affect children and adults [1]. SLE is a chronic disease condition, wherein the affected individuals experience alternate bouts of mild and severe symptoms. SLE cannot be cured; however the symptoms can be well managed with an appropriate treatment regime.

Patient Information

Definition

Systemic erythematosus lupus (SLE) is an autoimmune disorder, in which the body’s immune system mistakenly attacks the healthy tissues of the body. Such a kind of phenomenon gravely affects the various organs of the body.

Cause

The exact cause of SLE is not known. However interplay of genetic, and environmental factors, is known to trigger abnormal immune response. Individuals with family history of the disease are at an increased risk of developing SLE.

Symptoms

Symptoms of SLE include onset of joint pain and swelling in majority of the cases. In addition, individuals also experience fever, fatigue, malaise, sensitivity to light and development of butterfly shaped rashes.

Diagnosis

A preliminary physical examination followed by antinuclear antibody test is done. This is followed by blood tests, and urinalysis to diagnose underlying disease conditions. Liver function and kidney function tests are also necessary.

Treatment

Mild forms of SLE are treated through non-steroidal anti-inflammatory drugs (NSAIDs) for managing joint pain and pleurisy. Corticosteroid cream is also administered to be applied on the skin rashes. In more severe cases, high dose corticosteroids are administered. If these do not work then cytotoxic drugs are given.

References

Article

  1. Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med. Feb 28 2008;358(9):929-39.
  2. Cooper GS, Dooley MA, Treadwell EL, et al. Hormonal, environmental, and infectious risk factors for developing systemic lupus erythematosus. Arthritis Rheum 1998; 41:1714.
  3. Petri M. Epidemiology of systemic lupus erythematosus. Best Pract Res Clin Rheumatol 2002; 16:847.
  4. Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15(5):308-18.
  5. Lo MS, Tsokos GC. T cells in systemic lupus erythematosus: progress toward targeted therapy [August 2011]. The Rheumatologist [serial online].
  6. Trager J, Ward MM. Mortality and causes of death in systemic lupus erythematosus. Curr Opin Rheumatol. Sep 2001;13(5):345-51
  7. Pons-Estel GJ, Alarcón GS, Scofield L, et al. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum 2010; 39:257.
  8. Edworthy SM. Clinical Manifestations of Systemic Lupus Erythematosus. In: Harris ED, et al, eds. Kelley's Textbook of Rheumatology. 7th ed. Philadelphia, Pa: WB Saunders; 2005:1201-24.
  9. Rothfield N. Clinical features of systemic lupus erythematosus. In: Textbook of Rheumatology, Kelley WN, Harris ED, Ruddy S, Sledge CB (Eds), WB Saunders, Philadelphia 1981
  10. Tierney LM, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis and Treatment. 40th ed. New York, NY: McGraw-Hill; 2001:841-4.
  11. Bertsias G, Ioannidis JP, Boletis J, et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis 2008; 67:195.
  12. Schmajuk G, Yelin E, Chakravarty E, Nelson LM, Panopolis P, Yazdany J. Osteoporosis screening, prevention, and treatment in systemic lupus erythematosus: application of the systemic lupus erythematosus quality indicators. Arthritis Care Res (Hoboken). Jul 2010;62(7):993-1001.

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Last updated: 2018-06-21 19:52