Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Selective IgA deficiency may be defined as an inborn state characterized by a decrease of serum IgA levels below 8 IU/1 (approximately 5 mg/dl) which may be associated with clinical symptoms of disease. The frequency of this condition in the general population varies between 1 : 400 and 1 : 3000 in different countries. Patients with defects of chromosome 18, ataxia teleangiectatica and with connatal rubella syndrome have a high incidence of IgA deficiency. Inspite of the decrease in circulating IgA there are B-lymphocytes containing IgA molecules in the peripheral blood. Thus it has been concluded that transformation of B-lymphocytes into IgA bearing plasmacells is stunted by another mechanism. While small amounts of IgA may be released by transformed plasmacells the capacity of B-lymphocytes to mature into fully functioning plasmacells releasing normal amounts of IgA is defective. T-cells acting as suppressor cells for IgA differentiation have been demonstrated in peripheral blood and are a possible explanation for this phenomenon. The majority of individuals with IgA deficiency are healthy. Evaluations of increased susceptibility for infections have to consider the fact that 6 respiratory tract infections per year are the average for any preschool child. However a number of children with IgA deficiency suffer from recurrent bacterial infections such as sinusitis, bronchitis and pneumonia, usually responding well to antibiotic treatment. IgA deficiency has an established correlation with atopic disease. There is an 40 fold increase in incidence of allergies and autoimmune diseases such as rheumatoid arthritis, lupus erythematodes and thyroiditis in individuals with IgA deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Selective IgA deficiency]. 636 66

We observed two sisters with lupus-like syndrome with homozygous C3 deficiencies. A 19-year-old woman and her 15-year-old sister developed malar rash, arthralgia, and photosensitivity, but antinuclear antibodies and LE cell preparations were negative. The older sister experienced recurrent bronchitis in her childhood, but the younger sister had no recurrent infections. Serum C3 was not detected immunochemically in either sister, and total complement activity and C3 hemolytic activity were extremely low.
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PMID:Hereditary deficiency of the third component of complement in two sisters with systemic lupus erythematosus-like symptoms. 730 27

The following paper discusses the aspect of respiratory system diseases, that are present in the course of systemic lupus erythematosus. Taking into consideration that SLE is a nonspecific systemic autoimmune condition, lungs as well as respiratory tract could be affected in the process of the disease. The main contributing factors are circulating antibodies and immune complexes, that are deposited in tissues. SLE affects mostly women in reproductive age and is characterized by variable intensity of symptoms. The most commonly observed are: hyperthermia, malar rash, erythematous-cicatrical skin changes, photosensitivity, oral ulcers, polyarthralgia, nonerosive arthritis with no visible changes in radiogram, pleurisy and pericarditis, neuropsychiatric disorders, renal changes, hematologic disorders, as well as, abnormalities in circulatory and respiratory system. The respiratory conditions include pleuritis with or without pleural effusion, lupus pneumonitis, pulmonary hemorrhage, interstitial lung disease, pulmonary thromboembolic disease, pulmonary arterial hypertension, alveolitis, bronchitis obliterans and inflammatory myopathy, as well as acute alveolar-capillary injury, which in the course of SLE is associated with deposits of immune complexes in pulmonary alveoli, capillaries and interstitial tissue.
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PMID:[Diseases of the respiratory system in systemic lupus erythematosus]. 2434 Aug 94