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)

A lupus-like syndrome involving chronic urticaria with cutaneous vasculitis, systemic symptoms, hypocomplementemia with preferential depletion of C1q, and low m.w. (7S) C1q-precipitins has recently been defined. The C1q-precipitin activity (C1q-p) seems to represent a diagnostic marker of the disease, but its chemical nature is not yet clear. We have partially purified and characterized C1q-p from the serum of two patients with this syndrome and compared its activity with the C1q-precipitating activity of aggregated human gamma-globulin (AHGG) anti-C1q antibodies, and several polynucleotides including DNA and polyinosinic acid. C1q-p was found to partition with IgG during precipitation by ammonium sulfate and low ionic strength buffer as well as during column chromatography on DEAE-cellulose and G-200 Sephadex. Like AHGG, but in complete contrast to the polynucleotides, the C1q-precipitating activity of C1q-p was sensitive to pepsin, trypsin, and acidic conditions, but unaffected by DNAse or RNAse; the C1q-precipitating activity of anti-C1q antibody was not diminished by any of these procedures. Thus, C1q-p consists of gamma-migrating protein of low m.w., and its C1q-precipitating activity is indistinguishable from that of AHGG. These results are consistent with the concept that C1q-p is comprised, at least in part, of IgG that binds C1q via the Fc portion of the molecule.
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PMID:Low molecular weight C1q-precipitins in hypocomplementemic vasculitis-urticaria syndrome: partial purification and characterization as immunoglobulin. 2 69

The C1q precipitin test was performed in serum samples from five groups of patients: (1) 20 patients with acomplementemic systemic lupus erythematosus glomerulonephritis (SLE), (2) 2 patients with serum sickness due to the administration of horse serum, (3) 2 patients with serum sickness preceding hepatitis B, (4) 50 patients with chronic urticaria, and (5) 30 normal controls. Positive C1q precipitin tests were found in all patients with SLE and the four cases of serum sickness. Positive tests correlated with depressed serum complement (C3 and C4) levels and were found only in the early phase of serum sickness. Urticaria patients uniformly had negative C1q precipitin tests and normal serum complement levels.
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PMID:Studies of urticaria and acute serum sickness with the C1q precipitin test. 13 55

Twelve of 54 patients with systemic lupus erythematosus (SLE) had nonpuric, chronic urticaria-like lessions. Skin biopsy of the lesions was performed in 11 cases, and nine showed necrotizing vasculitis. The 54 patients, in general, had severe disease, and laboratory and clinical data suggested a postivite relationship between the urticaria-like lesions and disease severity. There was no consistent relationship between the course of the urticaria-like lesions and the serologic findings and clinical activity of the SLE. The frequency and importance of urticaria-like lesions in SLE deserve further study.
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PMID:Chronic urticaria-like lesions in systemic lupus erythematosus. A review of 12 cases. 35 70

During metabolism studies of radiolabeled proteins in 126 participants four patients were suspected of being sensitive to potassium iodide (Kl) because they repeatedly developed urticaria and other symptoms after Kl administration. Two of the four patients suspected of Kl sensitivity and 10 control patients were orally challenged with Kl to document and characterize Kl sensitivity and to evaluate the possible association(s) of Kl sensitivity with urticaria, hypocomplementemia, and vasculitis. The Kl challenges in the two sensitive patients precipitated urticaria, angioedema, polymyalgias, conjunctivitis, and coryza. One of these two patients also developed a severe systemic illness characterized by fever, headache, peritonitis, episcleritis, and pneumonitis. The four sensitive patients were strikingly similar in that they exhibited hypocomplementemia and dermal vasculitis associated with chronic urticaria or systemic lupus erythematosus, suggesting that other patients with similar clinical features may be sensitive to Kl and that Kl may precipitate severe systemic illness in them.
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PMID:Potassium iodide sensitivity in four patients with hypocomplementemic vasculitis. 51 84

An unusual case of photosensitive psoriasis and systemic lupus erythematosus-related syndrome was characterized by erythroderma, chronic urticaria, angioneurotic edema, intermittent low-grade fever, and polyarthralgias. Investigation revealed no measurable total hemolytic complement and markedly diminished levels of C4, C2, and C3. Microscopic examination of three skin biopsy sections of sun-exposed skin showed psoriasis. Skin biopsy sections of sun-exposed psoriatic plaques and of non-sun-exposed, uninvolved skin (which were stained with fluorescein-tagged anti-IgG, anti-IgM, anti-IgA, and anti-C3) showed granular deposits of IgM and C3 at the dermal-epidermal junction in the sun-exposed plaques, and IgM alone in a granular pattern at the dermal-epidermal junction in uninvolved skin. Antibodies to single-stranded but not double-stranded DNA were detected in the patient's serum. In addition, serum immune complex-like material was detected by sucrose density-gradient ultracentrifugation, standard anticomplementary assays, and radioimmunoassays using both C1q and monoclonal rheumatoid factor.
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PMID:Development of photosensitivity and an SLE-like syndrome in a patient with psoriasis. 67 15

Twelve cases of chronic urticaria with histopathologic features of lecocitoclastic allergic angitis are studied. The type of cutaneous lesion, personal and familiar atopic history and the presence of autoimmune disease are described. Light microscopy, direct immunofluorescence, anti DNA, antinuclear, antithyroid, Ro, La, Rnp and Sm antibodies, total complement levels, C3 and C4, rheumatoid factor, latex, ASTO, cryoglobulines and complete workup were investigated, taking into account natural progression and response to therapy. Two different groups are defined: 1) normocomplementemic (5 patients) and 2) hypocomplementemic (7 patients). They were all women except one. The cutaneous lesions were indistinguishable in the two groups. Only in the second group there was an associated disease (systemic lupus erythematosus, Sjogren syndrome disease, lupus-Sjogren overlap, autoimmune thyroid disease). Urticaria had been present from the onset of the disease in 4 patients, and occurred later during its course in 8 others. Five patients had thyroid disease (Hashimoto thyroiditis or Graves disease), two of them being mother and daughter. Another patient had a family history of Grave's disease and urticaria. Anti DNA antibodies were found in 7 cases, and anti Ro + La + in 3 cases. Response to treatment was variable with spontaneous cycles of worsening and remissions. One of the patients found a relationship with certain foods. Histopathologic results are related in both clinical normocomplementemic and hypocomplementemic groups. No significant differences were found between the two groups, but Ro+ and La+ patients exhibit more intense cariorexis and neutrophilic infiltrates.
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PMID:[Vasculitic urticaria: study of 12 cases]. 226 94

The identification of hereditary and acquired complement deficiencies in humans has led to a better understanding of the biologic importance of the complement system in immunity and autoimmune disease. Although the understanding of the relevance of complement in the pathogenesis of disease is incomplete, several characteristic clinical syndromes associated with complement deficiencies have been recognized and should be known to the practicing clinician. In allergic diseases, one need recognize the C1 inhibitor deficiency syndromes which can present as severe, recurrent angioedema in childhood or in the adult as recurrent angioedema in association with a lymphoid malignancy or autoimmune disease. Complement analyses allow one to readily diagnose C1 inhibitor deficiency in angioedema. Correct diagnosis is critical because safe effective therapy is available. Chronic urticaria is also uncommonly associated with complement deficiencies, particularly acquired C1q deficiency. Again, effective therapy for hypocomplementemic urticarial vasculitis and C1q deficiency is available and differs significantly from the usual management of chronic urticaria. Homozygous and acquired deficiencies of C3 are associated with severe immune deficiency and recurrent infections with gram-positive and gram-negative bacteria. Recurrent meningococcemia and gonococcemia are being identified frequently in patients with a deficient membrane attack mechanism relating to deficiency of C5, C6, C7, or C8. Nearly one third of the patients developing meningococcemia may have an associated complement deficiency indicating the importance of complement determinations in understanding the treatment and prognosis for these patients. Deficiency of almost every complement component has been reported in association with one or more rheumatic diseases, particularly systemic lupus erythematosus. Extensive studies of C2 deficiency and limited studies of C4 deficiency indicate that these components of the classical pathway of complement are important in preventing the development of SLE or are linked to other genes predisposing to SLE. The clinical presentations of SLE in association with C2 or C4 deficiency are relatively uniform. The patients exhibit typical skin manifestations suggestive of SLE and DLE and often exhibit antibodies to SSA (Ro). The association of complement deficiencies with clinical syndromes is important for today's physician. The syndromes and deficiencies described here are the beginning of an expanding knowledge relating to the pathobiology of complement in human disorders.
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PMID:The hereditary and acquired deficiencies of complement. 389 88

A 58 year-old woman had atypical chronic urticaria, arthralgias and abdominal pain. Attacks of angioneurotic edema occurred. Proteinuria was discovered. She had clinical and biological signs of inflammation, leukoneutropenia , antileukocyte antibodies and low CH 50, C1q and C4 levels without functional C1 esterase deficiency. C1q precipitins were not detectable. Skin biopsy disclosed angiitis and by immunofluorescence a lupus band test was positive. Serologic investigations in search of SLE were negative. Renal biopsy showed mesangial deposits, capillary loop thickening and mesangial fixation of anti-IgG, C3 C1q and C4 antisera. In the interstitium, voluminous perivenular inflammatory infiltrates were visible. With corticosteroid treatment clinical manifestations subsided and proteinuria disappeared. This observation of McDuffie 's angiitis with renal venulitis leads to a review of the literature with discussion of the mechanisms of hypocomplementemia.
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PMID:[Hypocomplementemic urticarial vasculitis with glomerulopathy and renal venulitis]. 672 99

Antinuclear antibodies (ANA) by indirect immunofluorescence and antibodies against native deoxyribonucleic acid (anti-nDNA) by radiommunoassay were measured simultaneously in 6,000 sera from about 5,000 patients. Usual findings about 5,000 patients. Usual findings were: (a) both tests negative (75%), (b) only ANA positive (20%), and (c) both tests positive (3%). The unusual combination of ANA-negative/anti-nDNA positive was found in 117 sera from 24 patients. These patients were examined more closely clinically and the mentioned tests repeated. In five with systemic lupus erythematosus, in two with chronic rheumatoid arthritis and one with chronic urticaria this finding occurred repeatedly over some time. In five additional patients (two with chronic hepatitis, one each with drug-induced systemic lupus erythematosus, chronic rheumatoid arthritis, and drug related haemolytic anaemia) this unusual finding occurred only once but with high levels of anti-nDNA. In 11 patients with various diseases the combination of ANA-negative/anti-nDNA positive occurred only once, with the anti-nDNA value being low. In a control group of patients with mononucleosis, cytomegalic disease, acute or chronic hepatitis or hepatoma, anti-nDNA results were never positive.
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PMID:[Antibodies against native deoxyribonucleic acid (anti-nDNA) without antinuclear antibodies: clinical significance (author's transl)]. 699 92

Circulating immune complexes (C.I.C.) were investigated in 244 patients with various skin diseases and 100 healthy subjects. C.I.C. were detected by the PEG-C4 assay, firstly proposed by Digeon et al. using the precipitation by polyethylene glycol (PEG 3,5 p. 100) and the determination by laser nephelometry of complement component C4 in sera and in precipitates. The percentage of C4 precipitated and of positive subjects were significantly increased in numerous cutaneous diseases: systemic lupus erythematosus, scleroderma, pemphigus, bullous pemphigoid, dermatitis herpetiformis, psoriasis, contact dermatitis and lichen planus. Two cases of dermatomyositis, 3 cases of post herpetic erythema multiformis and 2 cases of Kaposi-Juliusberg syndroma were also positives but no definite conclusion can be given because of the few patients tested. On the contrary, the values of precipitated C4 are normal in most cases of atopic dermatitis (the method does not detect IgE-C.I.C.) scabies, porphyria cutanea tarda, cutaneous epithelioma and discoid lupus. In chronic urticaria and in mycosis fongoides the mean values of precipitated C4 are significantly increased but the number of positive subjects is low and the significance of these results is uncertain because of the wide range of the values. The results of the present study are compared with the literature data. The value of C.I.C. determination in determining the evolutivity of skin diseases and their possible role in pathogenesis are discussed.
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PMID:[Circulating immune complexes in skin disease patients. Study and literature data (author's transl)]. 730 15


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