Systemic lupus erythematosus, abbreviated as SLE, is an autoimmune disease which primarily strikes women.
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].
Hematological
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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]
Signs and symptoms include easy bruising or bleeding due to a decrease in the number and size of platelets ; susceptibility to infections and to immune and inflammatory disorders; and an increased risk for some cancers (such as lymphoma). [rarediseases.info.nih.gov]
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]
Immune System
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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 loss [patient.info]
Enlargement of the spleen (splenomegaly) occurs in about 10% of people. [merckmanuals.com]
Hematologic • Anemia (chronic disease) • Leukopenia • Lymphopenia • Thrombocytopenia • Lymphadenopathy • Splenomegaly • Auto Immune Haemolytic anemia 44. [slideshare.net]
Entire Body System
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Fever
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]
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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]
Search terms included systemic lupus erythematosus, antiphospholipid syndrome, lupus nephritis, central nervous system disease in lupus, and fatigue. Articles were selected according to their impact on clinical practice. [doi.org]
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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]
[…] 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]
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Weight Loss
A 44-year-old Chinese female was admitted with three months history of painless abdominal distension accompanied by watery diarrhea 5-6 times daily, shortness of breath, fatigue, lower limb swelling, and 10 kg weight loss. [ncbi.nlm.nih.gov]
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Weakness
The patient's neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids. [ncbi.nlm.nih.gov]
Respiratoric
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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]
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]
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Dyspnea
INTERVENTIONS: Initially the patient was treated for medically unexplained dyspnea (MUD) without much improvement. [ncbi.nlm.nih.gov]
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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]
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]
Liver, Gall & Pancreas
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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]
Psychiatrical
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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]
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. [doi.org]
Neurologic
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Seizure
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 Sometimes lupus first starts in the most dramatic way with a seizure or a series of epileptic fits. This is usually when the patient is untreated and the disease fairly active. [thelupussite.com]
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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]
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. [thelupussite.com]
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]
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Confusion
When these two conditions happened to have similar clinical spectrum features, the confusion with each other occurred. [ncbi.nlm.nih.gov]
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Peripheral Neuropathy
Moreover, peripheral neuropathy can also occur in Sjögren's syndrome (SS) regardless of the presence or absence of vasculitis. [doi.org]
In addition to headache, lupus can cause other neurological disorders, such as mild cognitive dysfunction, organic brain syndrome, peripheral neuropathies, sensory neuropathy, psychological problems (including personality changes, paranoia, mania, and [malacards.org]
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Guillain-Barré Syndrome
He was clinically diagnosed with the acute motor and sensory axonal neuropathy variant of Guillain-Barré syndrome. Despite intravenous immunoglobulin therapy, he deteriorated with loss of all voluntary motor function and cranial nerve reflexes. [ncbi.nlm.nih.gov]
A case of systemic lupus erythematosus presenting as Guillain-Barré syndrome. Br J Rheumatol, 28 (1989), pp. 440-442 [12] R.M. Fazio, I. Chen, N. Somal. [elsevier.es]
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]
Urogenital
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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]
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]
failure and dialysis; blood abnormalities, etc. [azarthritis.com]
Despite advances in therapy, a minority of patients with lupus nephritis will develop kidney failure. These patients must undergo dialysis. [hss.edu]
As lupus becomes more active, the level of anti-dsDNA autoantibodies can rise, indicating a greater risk of lupus nephritis, which can lead to kidney failure. [nyulangone.org]
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Hematuria
At present, the level of complement restored to normal, hematuria and proteinuria disappeared, and liver function returned to normal. SLE may be a novel phenotype of GOF mutation in PI3CKD gene (GOF PIK3CD). [ncbi.nlm.nih.gov]
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Irregular Menstruation
Observational studies analysing the hormonal stage in a group of Japanese women showed that irregular menstruation was associated with an increased risk of the development of this disease [ 2 ]. [ncbi.nlm.nih.gov]
Gastrointestinal
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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]
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]
[…] have: Fatigue Fever without signs of infection Weight loss Swollen and painful joints Swollen muscles Skin rashes over areas exposed to sunlight, especially a butterfly shaped rash over the nose and cheeks Sensitivity to light Mouth sores Hair loss Nausea [winchesterhospital.org]
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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]
Jaw & Teeth
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Aphthous Stomatitis
Cutaneous Manifestations Over 90 percent of patients with systemic lupus erythematosus eventually have a cutaneous manifestation of the disease, including malar rash, discoid lupus erythematosus, alopecia or aphthous stomatitis. [aafp.org]
Cardiovascular
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Chest Pain
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]
Inflammation of the lining of the lungs (pleuritis) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body position. [web.archive.org]
Non-specific symptoms of fatigue, malaise, oral ulcers, arthralgia, photosensitive skin rashes, lymphadenopathy, pleuritic chest pains, headache, paraesthesiae, symptoms of dry eyes and mouth, Raynaud's phenomenon, and mild hair loss are more likely … [doi.org]
Musculoskeletal
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Arthritis
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 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]
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]
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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]
Reduced arthralgia/arthritis has been reported in Chinese ( P 0.02) [ 23 ], Greek ( P 0.004) [ 24 ] and Caucasian ( P 0.03) [ 25 ] populations. [doi.org]
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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]
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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]
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Symmetrical Arthritis
arthritis in both large and small joints malar "butterfly" rash (considered characteristic) kidney disorders, most often glomerulonephritis (in up to 50% of cases) cardiac effects: pericarditis or myocarditis pulmonary effects: pleurisy, pneumonitis [epilepsy.com]
Eyes
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Visual Impairment
OBJECTIVES: We aimed at evaluating morphological and functional visual abnormalities in a cohort of SLE and SS patients in the absence of an overt clinical visual impairment. [ncbi.nlm.nih.gov]
Any apparent visual impairment or eye disease should be first confirmed by an optometrist, and then referred on to the local ophthalmologist before starting treatment. [academic.oup.com]
Ischemic optic neuropathy is characterized by acute-onset and progressive binocular visual impairment as a consequence of occlusion of the small vessels of the optic nerves due to immune complex vasculitis. [doi.org]
The severity of the visual impairment depends on the involved tissue and its location. Signs include eyelid oedema, proptosis, limitation of ocular movements and, if anterior scleritis is present, redness. [medical-dictionary.thefreedictionary.com]
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Red Eye
Very rarely, lupus can cause a painful red eye or changes in your eyesight. If you have any symptoms affecting your eyes, it’s important to see a doctor as soon as possible. [versusarthritis.org]
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]
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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]
Swelling in the legs or around the eyes. Pain when breathing deeply or lying down. Headaches, dizziness, depression, confusion, or seizures. Stomach pain. [niams.nih.gov]
Skin
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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]
Photosensitivity in lupus erythematosus, UV photoprovocation results compared with history of photosensitivity and clinical findings. Br J Dermatol 1997 ; 136: 699 – 705. Google Scholar Medline ISI 16. Gambichler, T, Terras, S, Kreuter, A. [dx.doi.org]
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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]
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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]
[…] that resolve without scarring, sometimes with postinflammatory dyspigmentation or telangiectasias) Chronic cutaneous lupus Classic discoid rash, localized (above the neck) discoid rash, generalized (above and below the neck) discoid rash, hypertrophic [merckmanuals.com]
Discoid rash. A raised rash found on the head, arms, chest, or back. [stanfordchildrens.org]
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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]
[…] 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]
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]
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Erythema
In 1851 Cazenave renamed erythema centrifugum, calling it lupus erythematosus and gave a classic description of discoid lupus erythematosus. [ncbi.nlm.nih.gov]
1971, the American Rheumatism Association (ARA) Committee on Therapeutic Criteria proposed "Preliminary Criteria for the Classification of Systemic Lupus Erythematosus [SLE]".1Any four of the 14 items listed below, when present, indicate SLE.1 Facial erythema [jamanetwork.com]
Face, Head & Neck
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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]
There is consistent evidence for a reduced incidence of RP, alopecia, malar rash and arthralgia/arthritis in men at presentation and in the subsequent disease course. [doi.org]
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Facial Redness
Patients may have a distinctive pattern of facial redness and oral lesions. [medical-dictionary.thefreedictionary.com]
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].
Skin Test
X-Ray
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Pericardial Effusion
After 2 months, the symptoms were disappeared almost completely and TTE showed his pericardial effusion had decreased significantly. We should also keep SLE in mind when assessing male patients with pericardial effusions. [ncbi.nlm.nih.gov]
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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]
Urine
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Pyuria
[…] increases in the SLEDAI of 3 or more or 12 or more points, respectively, from the previous visit. 29 The data were also analyzed with the use of a new version of the SLEDAI (SLEDAI-2K) 30 and a modified SLEDAI (SLEDAIm) that excludes microhematuria and pyuria [content.nejm.org]
Serum
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Antinuclear Autoantibodies
Systemic lupus erythematosus is characterised by the presence of antinuclear autoantibodies (ANA). [hungary.pure.elsevier.com]
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]
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 [doi.org]
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Decreased Platelet Count
platelet count in 22.3%, hemolytic anemia in 4.8%, glomerulonephrites in 42.3%, anti-dsDna in 33.3%, anti-Ro in 37.3%, anti-La in 21.2%, anti-Sm in 23.5%, and anti-RNP in 27.3%. [scielo.br]
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Hyperglobulinemia
[…] resembling rheumatoid arthritis, diffuse erythematous skin lesions on the face, neck, or upper extremities, with liquefaction degeneration of the basal layer and epidermal atrophy, lymphadenopathy, pleurisy or pericarditis, glomerular lesions, anemia, hyperglobulinemia [medical-dictionary.thefreedictionary.com]
Other Pathologies
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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]
Pleura
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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]
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]
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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]
• 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; 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]
HLA Type
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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]
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]
[…] been noted in some populations without observing an association with HLA-DR3, thereby providing evidence that C4 may have an effect that is independent of HLA. 60, 61, 63, 64, 65 The most frequent homozygous complement deficiency in humans is that of [doi.org]
The presence of human leukocyte antigen (HLA)–B8 and HLA-DR3 in the mother may predispose the infant to NLE and congenital heart block. [emedicine.medscape.com]
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HLA-B8
Factors leading to SLE include: Genetic predisposition, including haplotype HLA-B8, -DR3 Exposure to sunlight Viral infection, particularly Epstein-Barr virus Hormones Toxins such as cigarette smoke Drugs in drug-induced LE Emotional upset. [dermnetnz.org]
The presence of human leukocyte antigen (HLA)–B8 and HLA-DR3 in the mother may predispose the infant to NLE and congenital heart block. [emedicine.medscape.com]
[…] 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]
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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. [doi.org]
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HLA-DRw3
Increased frequency of HLA-DRw3 in systemic lupus erythematosus Tissue Antigens 1980 15 : 283–288 41 Gladman KK, Urowitz MB, Darlinkton GA. [doi.org]
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].
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
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- Petri M. Epidemiology of systemic lupus erythematosus. Best Pract Res Clin Rheumatol 2002; 16:847.
- Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15(5):308-18.
- Lo MS, Tsokos GC. T cells in systemic lupus erythematosus: progress toward targeted therapy [August 2011]. The Rheumatologist [serial online].
- Trager J, Ward MM. Mortality and causes of death in systemic lupus erythematosus. Curr Opin Rheumatol. Sep 2001;13(5):345-51
- Pons-Estel GJ, Alarcón GS, Scofield L, et al. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum 2010; 39:257.
- 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.
- 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
- Tierney LM, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis and Treatment. 40th ed. New York, NY: McGraw-Hill; 2001:841-4.
- 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.
- 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.