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Systemic Lupus Erythematosus

SLE

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].

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]
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]
  • Hematologic • Anemia (chronic disease) • Leukopenia • Lymphopenia • Thrombocytopenia • Lymphadenopathy • Splenomegaly • Auto Immune Haemolytic anemia 44.[slideshare.net]
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]
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.[dx.doi.org]
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]
Raynaud Phenomenon
  • We present a case of a young Pakistani boy who presented with cervical lymphadenopathy, fever, blackish discoloration of finger tips, and Raynaud's phenomenon. His lymph node biopsy was suggestive of KFD.[ncbi.nlm.nih.gov]
  • phenomenon, pulmonary, hematological, gastrointestinal, ocular.[web.archive.org]
  • […] 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]
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]
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]
Dyspnea
  • INTERVENTIONS: Initially the patient was treated for medically unexplained dyspnea (MUD) without much improvement.[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]
  • 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]
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]
  • 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]
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]
  • failure SLE can have serious negative effects on your body during pregnancy.[healthline.com]
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]
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]
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 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]
  • 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]
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 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.[thelupussite.com]
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]
Peripheral Neuropathy
  • 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
  • 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]
  • 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.[37] More rare manifestations are acute confusional state, GuillainBarré[en.wikipedia.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]
  • 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]
  • 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]
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]
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 …[dx.doi.org]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
  • An 8-year-old girl had presented with fever, arthralgia, alopecia, anasarca and bleeding from multiple sites.[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]
Erythema
  • In 1851 Cazenave renamed erythema centrifugum, calling it lupus erythematosus and gave a classic description of discoid lupus erythematosus.[ncbi.nlm.nih.gov]
  • 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]
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]
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]
  • Its symptoms can include: "butterfly" rash across the nose and cheeks skin rashes on parts of the body exposed to the sun sores in the mouth or nose painful or swollen joints fever weight loss hair loss fatigue chest pain when taking deep breaths purple[news-medical.net]
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].

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]
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]
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[dx.doi.org]
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[nejm.org]
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]
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]
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]
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-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]
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]
  • 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]
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.[dx.doi.org]
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].

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

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Last updated: 2019-07-11 20:17