Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Microscopic polyangiitis, a condition recently differentiated from macroscopic periarteritis nodosa, is characterized by small vessel damage, pauciimmune necrotizing glomerulonephritis and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). Arthralgia is a common symptom often present early in the disease, and other joint manifestations have also been reported. We report a case with polyarthritis as the first manifestation. Perinuclear ANCA was found in a moderate titer. A renal biopsy done six months after the onset of joint symptoms to investigate rapidly progressive renal failure established the diagnosis. The p-ANCA exhibited antimyeloperoxidase specificity. In the discussion we review the diagnosis of microscopic polyangiitis and of concomitant polyarthritis and p-ANCA production. ANCA is present in some patients with rheumatoid arthritis or systemic lupus erythematosus. It is important to determine the specificity of the ANCA since presence of p-ANCA with antimyeloperoxidase specificity in a patient with polyarthritis is highly suggestive of systemic vasculitis.
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PMID:Polyarthritis with perinuclear antineutrophil cytoplasmic antibody inaugurating microscopic polyangiitis. Report of a case. 952 94

Rheumatological conditions can sometimes present as emergencies. These can occur due to the disease process or may be iatrogenic. Some of the important articular emergencies are septic arthritis, acute polyarthritis and atlanto-axial dislocation. Classical polyarteritis nodosa may present with massive gastro-intestinal bleeding, intestinal perforation or acute pancreatitis. Adult respiratory distress syndrome, bilateral pneumonitis and diffuse alveolar haemorrhage due to systemic lupus erythematosus or systemic necrotising vasculitis and ventilatory failure due to polymyositis are some of the respiratory emergencies. Scleroderma is well known to cause renal crisis which can be fatal if not diagnosed and managed promptly. Microscopic polyangiitis and Wegener's granulomatosis may cause rapidly progressive renal failure. Cerebrovascular accident, cortical vein thrombosis, seizures and acute psychosis are important neurological complications of rheumatic disease. Cardiac emergencies include tamponade, acute myocarditis and acute myocardial infarction. Vision can be threatened in Behcet's disease, temporal arteritis and seronegative spondylarthritis. Catastrophic antiphospholipid syndrome is a devastating emergency. The management of above emergencies includes critical care, immunosuppression when indicated and withdrawal of the offending drug. Anticoagulants have to be used in the management of antiphospholipid syndrome. A good understanding of these conditions is of paramount importance for proper management.
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PMID:Emergencies in rheumatology. 1516 86

Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis that affects small vessels, especially renal glomeruli. We recently demonstrated that the abnormal formation and impaired degradation of neutrophil extracellular traps (NETs) may be crucially involved in the generation of myeloperoxidase (MPO)-ANCA and subsequent development of MPA. This study assessed the formation and regulation of NETs in patients with MPO-ANCA-associated MPA. Peripheral blood samples were obtained from 38 patients with MPO-ANCA-associated MPA, 23 patients with systemic lupus erythematosus (SLE), and 8 healthy controls. IgG eluted from MPO-ANCA-associated MPA sera demonstrated the highest ability to induce NETs, and this ability correlated with disease activity and paralleled ANCA affinity for MPO. Moreover, addition of recombinant human MPO to these IgG samples reduced NET induction. Additionally, MPO-ANCA-associated MPA sera exhibited lower rates of NET degradation that recovered partially upon depletion of IgG. The activity of DNase I, an important regulator of NETs, was also lower in MPO-ANCA-associated MPA and SLE sera. IgG depletion from MPO-ANCA-associated MPA sera partially restored the rate of NET degradation, and addition of DNase I synergistically enhanced this restoration. Addition of anti-MPO antibodies did not inhibit DNase I activity, and some MPO-ANCA-associated MPA sera contained anti-NET antibodies at levels not correlated with MPO-ANCA titers, suggesting the involvement of unidentified autoantibodies as well. The collective evidence suggests a vicious cycle involving MPO-ANCA and the regulation of NETs could be critically involved in the pathogenesis of MPO-ANCA-associated MPA.
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PMID:Enhanced formation and disordered regulation of NETs in myeloperoxidase-ANCA-associated microscopic polyangiitis. 2438 92