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Systemic Lupus Erythematosus

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


Lupusfoto
Lupusfoto
Lupusfoto, CC BY-SA 4.0

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
  • 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]
  • Hematologic • Anemia (chronic disease) • Leukopenia • Lymphopenia • Thrombocytopenia • Lymphadenopathy • Splenomegaly • Auto Immune Haemolytic anemia 44.[slideshare.net]
  • 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]
  • Differential Diagnosis of Systemic Lupus Erythematosus Differential diagnosis Distinguishing features Diagnostic approach Adult-onset Still disease Arthralgia, fever, lymphadenopathy, splenomegaly Tests for elevated ESR, leukocytosis, and anemia Behçet[aafp.org]
Generalized Lymphadenopathy
  • Systemic lupus erythematosus with an unusual presentation of generalized lymphadenopathy was diagnosed in both patients.[ncbi.nlm.nih.gov]
Pleural Effusion
  • Eosinophilic pleural effusion in elderly patients is most commonly due to malignancies and infections. In rare cases, pleural eosinophilia is associated with connective tissue disease.[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]
  • Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage.[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
  • A 43-year-old male came to our hospital with the sole symptom of dyspnea after thymoma resection initially. However, other atypical lesions of SLE occurred over time.[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]
  • Acute lupus pneumonitis presents as cough, dyspnea, pleuritic pain, hypoxemia, and fever. Infiltrates on chest radiographs may be unilateral or bilateral.[emdocs.net]
  • Musculoskeletal System : Arthritis- typically affects hand, wrists, and knees Arthralgia Tenosynovitis Tendon ruptures Swan-neck deformity Ulnar drift Cardiopulmonary/Cardiovascular System: Pleuritis Pericarditis Dyspnea Hypertension Myocarditis Endocarditis[physio-pedia.com]
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]
  • Diagnostic Criteria for Systemic Lupus Erythematosus System ACR criteria* SLICC criteria † Cardiac/pulmonary Pleuritis (pleuritic pain or rub, or pleural effusion), or pericarditis (documented by electrocardiography, rub, or pericardial effusion) Serositis[aafp.org]
  • Acute lupus pneumonitis presents as cough, dyspnea, pleuritic pain, hypoxemia, and fever. Infiltrates on chest radiographs may be unilateral or bilateral.[emdocs.net]
  • Serositis a) Pleuritis – pleuritic pain, pleural rub, pleural effusion b) Pericarditis – ECG changes, rub, pericardial effusion 7. Renal disorder a) Proteinuria ( 3 or 0.5 g/day) b) Cellular casts in urine 8.[ojrd.biomedcentral.com]
Fever
  • 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]
  • The patient showed a high spiking fever and myalgia. Laboratory data revealed pancytopenia and immunological abnormalities. Pulse methylprednisone plus intravenous immunoglobulin (IVIG) failed to improve the clinical symptoms and laboratory data.[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]
  • 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
  • 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]
  • Relationship assessment and fatigue severity scale scores were found to be the best indicators of depression for the SLE patients (P   .042 and .028, respectively).Fatigue Severity, relationship satisfaction, and IL-10 concentrations are indicators of[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]
  • A 27-year-old previously healthy woman was admitted with echymotic patches over the lower limbs for six months, multiple joint pain and fatigue for 2 months. She had severe pallor and multiple echymotic patches over the lower limbs.[ncbi.nlm.nih.gov]
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]
  • 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]
  • Laboratory findings on admission revealed proteinuria, microscopic hematuria, anemia, leukopenia, hypoalbuminemia, hypocomplementemia, and slightly elevated levels of serum creatinine.[ncbi.nlm.nih.gov]
  • A 46-year-old man suffered from chronic fever, nephrotic syndrome, acute kidney injury, anemia, thrombocytopenia and serositis, as well as hypocomplementemia and negative anti-nuclear antibody.[ncbi.nlm.nih.gov]
Raynaud Phenomenon
  • They include immune complexes, Toll-like receptors activation, antiphospholipid antibodies and ischemia-reperfusion associated with Raynaud phenomenon.[ncbi.nlm.nih.gov]
  • […] 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 few are RF[orthobullets.com]
  • phenomenon, IHD, arteritis - ischaemia RESPIRATORY: infections and PE’s, pleuralitis, pleural effusion, pulmonary fibrosis NEUROLOGICAL: cranial and peripheral nerve lesions, transverse myelitis - weakness and paraplegia, depression, psychosis, seizures[lifeinthefastlane.com]
  • Individuals also experience numbness, headache, seizures, abdominal pain, arrhythmias, swelling in legs, difficulty in breathing and Raynaud phenomenon.[symptoma.com]
  • Children and teens with SLE may have fatigue, pain or swelling in joints, skin rashes, fevers, hair loss, mouth sores or skin color changes due to the cold (Raynaud's phenomenon). Fatigue is one of the most prominent and life-affecting symptoms.[arthritis.org]
Weight Loss
  • 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]
  • An 82-year-old woman complained about anorexia, weight loss, fatigue, and mild night fever. Clinical examination disclosed a left pleural effusion without parenchymal lesion on high contrast thoraco-abdomino-pelvic computed tomography scan.[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]
  • Kidney problems may become apparent when lupus patients feel ill with arthritis, have a rash, fever and weight loss. Less often, kidney disease may occur when there are no other symptoms of lupus.[my.clevelandclinic.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]
  • 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]
  • Symptoms SLE may cause general symptoms such as fever, fatigue, mouth sores, hair loss, stomach pain, nausea and vomiting, abnormal heart rhythm, chest pain with deep breaths, headaches, seizures, difficulty breathing, and hemoptysis.[rheumatologyspecialistcare.com.au]
  • Gastrointestinal-Stomach & Digestion: Lupus can cause or exacerbate ulcerative colitis, pancreatitis, and liver conditions, resulting in nausea, vomiting, recurring and persistent abdominal pain, bladder infections, and blood in urine.[kaleidoscopefightinglupus.org]
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]
  • 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]
  • 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]
  • 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]
Hepatosplenomegaly
  • Hepatosplenomegaly can come and go with disease activity. Mesenteric vasculitis is very rare, but can be life-threatening, especially if it leads to perforation, and may only be diagnosed at laparotomy.[ojrd.biomedcentral.com]
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]
Red Eye
  • Posterior scleritis does not cause a red eye (unless it extends anteriorly) but may cause visual problems, with blurring, change in refraction and double vision [ 16 ].[academic.oup.com]
  • Very rarely, lupus can affect the eyes, causing a painful red eye or changes in the eyesight. Related conditions About one third of people with lupus develop an additional autoimmune disease.[arthritisresearchuk.org]
Arthritis
  • She was treated with increasing immunosuppression as for lupus related arthritis. Subsequently she developed a wrist effusion with high inflammatory markers, and was treated as septic arthritis. Synovial biopsy features suggested tuberculosis.[ncbi.nlm.nih.gov]
  • Despite also suffering from the pain and disability that accompanies chronic autoimmune disease, the rheumatoid arthritis patients had less anxiety and better relationship scores.[ncbi.nlm.nih.gov]
  • Arthritis in SLE is defined as arthritis of two or more peripheral joints, as evidenced by joint tenderness, swelling, or effusion.[practicalpainmanagement.com]
  • The primary endpoint was the proportion of patients achieving resolution of arthritis or rash at week 24, as defined by Systemic Lupus Erythematosus Disease Activity Index-2000 (SLEDAI-2K).[ncbi.nlm.nih.gov]
  • The most common systemic rheumatologic conditions are connective tissue diseases (including rheumatoid arthritis [RA]) followed by spondyloarthropathy.[ncbi.nlm.nih.gov]
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]
  • A 42-year-old woman was admitted to our hospital with complaints of exanthema, arthralgia, shortness of breath, and hemoptysis.[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]
  • Fever, arthralgia and pallor are the most frequent clinical manifestations among the children. Hemolytic anemia (HA) is the most common laboratory abnormality, with ANA and anti ds-DNA antibodies positivity in the majority of padiatric patients.[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]
  • 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]
  • In active SLE arthritis, joints are tender to palpation with mild joint swelling, evidenced by difficulty palpating the joint line. Certain laboratory abnormalities suggest active SLE as well.[practicalpainmanagement.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]
  • stiffness lasting more than one hour; affected joints are usually symmetric, tender, and swollen Positive tests for rheumatoid factor and anticyclic citrullinated antibodies; synovial fluid reflects inflammatory state Sarcoidosis Cough, dyspnea, fatigue[aafp.org]
  • 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 haematological abnormality haemolytic anaemia or leukopenia: 3 on more than two occasions lymphopenia: 3 on more[radiopaedia.org]
Photosensitivity
  • 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]
  • 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]
  • For patients with photosensitivities, flare-ups can be reduced if patients are cautious about the amount of sunlight or UV light that they are exposed to.[physio-pedia.com]
Discoid Rash
  • 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]
  • Discoid rash. A raised rash found on the head, arms, chest, or back.[stanfordchildrens.org]
  • rash photosensitivity oral ulcers non-erosive arthritis serositis: pleuritis, pericarditis renal: proteinuria, casts central nervous: seizures, psychosis haematological: haemolytic anaemia, leucopenia, thrombocytopenia immune: anti-dsDNA, anti-smooth[lifeinthefastlane.com]
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]
  • 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]
  • 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]
  • A 10-year-old girl was admitted to our hospital for marasmus and fatigue without other typical manifestations of SLE well before the appearance of skin lesions.[ncbi.nlm.nih.gov]
  • Photosensitivity, fever, skin lesions on the neck, and alopecia where the skin lesions extend beyond the hairline may occur. The skin lesions may spread to the mucous membranes and other tissues of the body.[medical-dictionary.thefreedictionary.com]
Alopecia
  • […] 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]
  • Total activity score is made up of: Degree of redness (0–3) and scale (0–2) Mucous membrane involvement (0–1) Recent hair loss (0–1), nonscarring alopecia (0–3) Total damage score is made up of: The degree of dyspigmentation (0–2), and scarring (0–2)[dermnetnz.org]
  • Cutaneous • Photosensitivity • Malar rash • Oral Ulcers • Alopecia • Discoid Rash • Vasculitis rash • Urticaria 41.[slideshare.net]
Erythema
  • 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 of SLE.[ncbi.nlm.nih.gov]
  • Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds 2.[pedclerk.bsd.uchicago.edu]
  • M34.0 ) rheumatoid arthritis ( M05.00- M06.9 ) systemic lupus erythematosis ( M32.0- M32.9 ) Type 2 Excludes: A18.4 ICD-10-CM Diagnosis Code A18.4 Tuberculosis of skin and subcutaneous tissue 2016 2017 2018 2019 Billable/Specific Code Applicable To Erythema[icd10data.com]
  • Vasculitic skin lesions may include mottled erythema on the palms and fingers, periungual erythema, nail-fold infarcts, urticaria, and palpable purpura. Petechiae may develop secondary to thrombocytopenia. Photosensitivity occurs in some patients.[merckmanuals.com]
  • Acute CLE Central face malar or "butterfly" violaceous erythema with sharp cutoff at lateral margins, resolves without scarring (may result in persistent telangiectasia ) Bullous lupus: a blistering rash , if severe may resemble toxic epidermal necrolysis[dermnetnz.org]
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]
  • 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]
  • 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 of SLE.[ncbi.nlm.nih.gov]
  • Clinical features Relatively common autoimmune disease of women, due to anti-DNA antibodies Fatigue, fever, arthritis, erythematous, bilateral butterfly (malar) rash of the face, renal disease, lymphadenopathy, and serositis Associated with altered immunity[pathologyoutlines.com]
  • The classic malar rash is absent in two-thirds of individuals.[pedclerk.bsd.uchicago.edu]
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
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]
  • Stojanovich , L , Zandman-Goddard , G , Pavlovich , S et al ( 2007 ) Psychiatric manifestations of systemic lupus erythematosus . Autoimmunity Review ; 6 : 421–6.[cambridge.org]
Seizure
  • 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]
  • 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]
  • Raynauds phenomenon, IHD, arteritis - ischaemia RESPIRATORY: infections and PE’s, pleuralitis, pleural effusion, pulmonary fibrosis NEUROLOGICAL: cranial and peripheral nerve lesions, transverse myelitis - weakness and paraplegia, depression, psychosis, seizures[lifeinthefastlane.com]
  • Lupus can be triggered by certain types of blood pressure medications, anti-seizure medications and antibiotics. People who have drug-induced lupus usually get better when they stop taking the medication.[mayoclinic.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]
  • Here, we report one case of meningoencephalitis by Listeria monocytogenes (LM) in a patient with SLE who presented with fever and developed headache and altered in consciousness in the hospital.[ncbi.nlm.nih.gov]
  • Headaches. Dizzy spells. Feeling sad. Confusion. Seizures. What causes it? What causes systemic lupus erythematosus (lupus)? No one knows what causes systemic lupus erythematosus (lupus).[niams.nih.gov]
  • If your brain is affected by lupus, you may experience headaches, dizziness, behavior changes, vision problems, and even strokes or seizures. Many people with lupus experience memory problems and may have difficulty expressing their thoughts.[mayoclinic.org]
  • Symptoms SLE may cause general symptoms such as fever, fatigue, mouth sores, hair loss, stomach pain, nausea and vomiting, abnormal heart rhythm, chest pain with deep breaths, headaches, seizures, difficulty breathing, and hemoptysis.[rheumatologyspecialistcare.com.au]
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]
  • 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]
  • How we care for lupus If your child has lupus, it can be a confusing and scary diagnosis to deal with.[childrenshospital.org]
  • 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
  • Peripheral neuropathy in SLE should be given greater recognition, and rarer forms of presentation should be taken seriously in the differential diagnosis when the clinical picture is atypical.[ncbi.nlm.nih.gov]
  • 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]
  • neuropathy, cranial neuropathy, cerebrovascular accidents, organic brain syndrome, psychosis) Mouth, nose, or vaginal ulcers” [1] The most common signs and symptoms of SLE in children and adolescents are: "fever, fatigue, weight loss, arthritis, rash[physio-pedia.com]
  • Nervous system problems that cause vision disturbance, dizziness, muscle weakness in the face, arms, or legs, or loss of temperature or pain sensation in the feet, hands, arms, or legs (cranial or peripheral neuropathy ). Seizures .[northshore.org]
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]
  • […] and headache with occasional abducens nerve paresis , absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. [36] More rare manifestations are acute confusional state , Guillain–Barré[en.wikipedia.org]

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].

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]
  • 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]
  • Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage.[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]
  • In addition to ascites, pleural and pericardial effusions had developed. 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]
Esophageal Motility Disorder
  • Esophageal motility disorders in the rheumatic diseases: a review of 150 patients. Clin Exp Rheumatol 1994;12:515–21 86. Vergara-Fernandez O, Zeron-Medina J, Mendez-Probst C, Salgado-Nesme N, Borja-Cacho D, Sanchez-Guerrero J, Medina-Franco H.[journals.lww.com]
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]
  • Pathological studies demonstrate infarction of the optic nerve secondary to extensive arteriolar fibrinoid necrosis [ 35 ]. Acute optic neuritis may also be bilateral and associated with transverse myelopathy.[academic.oup.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]
  • AIH is characterized by the elevated levels of liver enzymes, hypergammaglobulinemia, the presence of autoantibodies and typical histological changes [ 25 ], and association with the Human Leukocyte Antigens (HLA) DR3 or DR4 [ 24 ].[omicsonline.org]
  • TNFAIP3, FAM167A-BLK, BANK1 and KIAA1542); however, it was determined that these loci had a lower significance level and a lower contribution to individual risk for SLE. [31] Studies of human leukocyte antigens (HLAs) reveal that HLA-A1, HLA-B8, and HLA-DR3[emedicine.medscape.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]
  • […] associations with SLE (TNFAIP3, FAM167A-BLK, BANK1 and KIAA1542); however, it was determined that these loci had a lower significance level and a lower contribution to individual risk for SLE. [31] Studies of human leukocyte antigens (HLAs) reveal that HLA-A1, HLA-B8[emedicine.medscape.com]
Pleural Effusion
  • Eosinophilic pleural effusion in elderly patients is most commonly due to malignancies and infections. In rare cases, pleural eosinophilia is associated with connective tissue disease.[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]
  • Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage.[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
  • . • Serositis – Typical pleurisy for 1 day OR pleural effusions OR pleural rub – Typical pericardial pain (pain with recumbency improved by sitting forward) for 1 day OR pericardial effusion OR pericardial rub OR pericarditis by electrocardiography. •[slideshare.net]
  • 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]
  • rub; pericardial pain more than one day, pericardial effusion, pericardial rub, or pericarditis) Hematologic Hemolytic anemia, or leukopenia ( 4,000 cells per mm 3 ), or lymphopenia ( 1,500 cells per mm 3 ), or thrombocytopenia ( 100,000 cells per mm[aafp.org]
  • Serositis a) Pleuritis – pleuritic pain, pleural rub, pleural effusion b) Pericarditis – ECG changes, rub, pericardial effusion 7. Renal disorder a) Proteinuria ( 3 or 0.5 g/day) b) Cellular casts in urine 8.[ojrd.biomedcentral.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