Immunogenetics of HLA class II genes in primary Sjögren's syndrome in Israeli Jewish patients.
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Biomedical subjects
Publications and source records attributed to A Rubinow.
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A case of late haematogenous pneumococcal infection of a prosthetic hip joint is presented. In view of the relatively benign nature of pneumococcal arthritis, the patient was treated with parenteral antibiotics and surgical drainage without removal of the prosthesis. This was followed by complete resolution.
There are inherent technical difficulties in measuring IgG rheumatoid factor (IgG-RF) in the serum of patients with rheumatoid arthritis (RA). These arise from measuring a reaction between two IgG molecules and the interference of IgM-RF in the reaction. We compared the prevalence of IgG-RF in whole sera and purified IgG fractions from 58 RA patients (43 of whom were latex or sheep cell agglutination positive). Methods of purification were: ammonium sulphate precipitation and DEAE cellulose or protein A-Sepharose chromatography. IgG-RF was measured by two methods: (1) radioimmunoassay and ELISA with a monoclonal myeloma IgG (IgG4,K) as the antigen and radiolabelled rabbit anti-human IgG (previously absorbed on a column with IgG4,K) as the second antibody; (2) ELISA using rabbit IgG as the antigen and a peroxidase conjugated goat anti-human IgG as the second antibody. When whole sera were assayed, 18 (31%) contained IgG-RF. In contrast, only three of the IgG fractions (5%) were positive for IgG-RF by all methods, while the remainder were uniformly negative. These results suggest that IgG-RF determination in whole sera does not accurately reflect IgG-RF activity.
The in-vitro proliferation reaction of peripheral blood lymphocytes (measured by [3H]thymidine incorporation) to autologous pokeweed mitogen (PWM)-induced lymphoblasts (PWM-lymphoblast-stimulated autologous mixed leucocyte reaction, PWM.AMLR) was used as a measure of immune hyperreactivity for comparison of atopic with non-atopic individuals. Accordingly, 10/24 non-atopics responded in the PWM.AMLR, and 19/19 atopics reacting to inhaled allergens responded. Autologous stimulation was associated with release of mitogenic factors from the PWM-activated stimulating cells (2/15 non-atopics, 9/15 atopics). For non-atopics, stimulation delivered by staphylococcus A (SAC)-activated cells was similar to that delivered by PWM-induced cells, while in atopics, the SAC.AMLR was never more than 50% of the PWM.AMLR, indicating a possible T cell component. Separation by panning of the stimulation cells into lymphocyte subsets supported the notion that stimulation involved a cooperation between B and T4+ T cells. It is proposed that a positive PWM.AMLR is dependent upon an initial B cell activation followed by the PWM stimulus dependent upon a previous T cell activation, where atopics have more lymphocytes in an activated state than healthy non-atopics. Such a baseline priming may contribute to an innate sensitivity of atopics to environmental allergens.
A 26 year old woman with systemic lupus erythematosus, including malar rash, photosensitivity, and arthritis, developed a cerebrovascular accident and acute myocardial infarction. High titres to antinuclear factor, anti-DNA antibodies, positive Venereal Disease Research Laboratory (VDRL) test, and anticardiolipin antibodies were found in her serum. A possible association between the presence of anticardiolipin antibodies and the two major thrombotic events is discussed.
A patient with seropositive rheumatoid arthritis and a carrier of hepatitis B surface antigen developed angiofollicular hyperplasia (multicentric Castleman's disease). The hepatitis B virus and the rheumatoid factor may have had a role in the aetiology of this lymphatic disorder. The development of Castleman's disease in association with these factors may provide another clue supporting the reactive nature of this disease.
A 20-year-old woman with nasopharyngeal carcinoma, in clinical remission, developed pains in the wrists and knees. Radiological and scintigraphic evaluation revealed tumor recurrence and bone metastases. No pulmonary involvement was observed. Reinstitution of combined chemotherapy resulted in abolition of the metastatic lesions and resolution of her rheumatic complaints.
Two cases of polyarteritis nodosa (PAN) in patients with familial Mediterranean fever (FMF) are reported. These and another 11 cases found in the literature suggest that PAN occurs more commonly in patients with FMF than would be expected in the general population. Perirenal hematoma, which is surprisingly high in patients with FMF, is a life threatening complication of PAN. The diagnosis of PAN in patients with FMF may be delayed due to the similarity of the clinical manifestations of both diseases.
A 16-year-old girl meeting the criteria for SLE is described. Salient features of the clinical course included active glomerulonephritis with dense subepithelial deposits on electron microscopy, pulmonary embolism, axillary vein thrombosis, arthritis, serositis and fever. Disease activity correlated with the presence of lupus anticoagulant as measured by VDRL and partial thromboplastin time (PTT). Her serum was consistently negative to ANA, anti-DS-DNA, anti-SS-DNA, ENA, anti-Ro, anti-La, and LE cells for the entire 4-year course. She responded remarkably to prednisone and azathioprine. Reappearance of VDRL and elevated PTT preceded exacerbation of disease activity and served as a serological guide for modifying medical treatment.
The brown pigmentation of the skin associated with venous ulceration is caused by increased local iron deposition. Diagnostic x-ray spectrometry, a method based on x-ray fluorescence analysis, was used for the noninvasive determination of iron levels in the skin of patients with venous ulceration. The mean (+/- SEM) iron concentration in the skin around the venous ulcer was elevated, compared with control values of nonulcerated skin (250 +/- 54 vs 128 +/- 39 micrograms) and compared with normal skin from the forearm (250 +/- 54 vs 14 +/- 2.5 micrograms). These data suggest that dermal iron deposition may not be an incidental by-product of increased venous pressure, but may actively perpetuate tissue damage in venous ulcerations.
Thirty-one patients with documented cardiac amyloidosis were compared to 39 control subjects with left ventricular hypertrophy to determine specific 2-dimensional echocardiographic features of amyloid. In 16 patients, increased myocardial echogenicity was present when a single short-axis view was examined, and had a sensitivity of 63% and a specificity of 74% for the diagnosis of amyloidosis. When complete echocardiograms were reviewed (15 patients), an improved sensitivity of 87% and specificity of 81% based on increased echogenicity was seen. Increased atrial septal thickness was present in 60% of amyloid patients and no controls. The combination of increased myocardial echogenicity and increased atrial thickness was 60% sensitive and 100% specific for the diagnosis of amyloidosis. The ratio of electrocardiographic voltage (S in V1 + R in V5 or V6) to left ventricular cross-sectional area also was examined. A ratio of less than 1.5 was 82% sensitive and 83% specific for amyloid (excluding the 2 patients with left bundle branch block), but added little to the diagnosis as determined from the 2-dimensional echocardiogram.
Primary amyloidosis has a variable course, but is generally associated with a short life expectancy. To date, no specific therapy has been available. Fifty-three patients with AL amyloidosis seen between 1976 and 1983 were treated with colchicine, and their clinical course and survival were compared with that in 29 other patients seen between 1961 and 1973. Of the variables measured, the treatment, the patient's sex, and the time interval from diagnosis to referral of treatment were significantly associated with length of survival. Median survival for the colchicine-treated patients was 17 months, compared with six months for the non-colchicine-treated patients. A surprising finding was the longer life span in female patients (median eight months versus four and a half months in the non-colchicine-treated group, and 25.5 months versus 10 month in the colchicine-treated group). The study suggests that colchicine has improved the life expectancy in AL amyloidosis. Although it is not a specific therapy, it may be a reasonable form of adjunctive treatment in this complex disorder.
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Codeine and other opiates can induce immediate type wheal and flare skin reactions. Calcium channel blockers including nifedipine have been shown to inhibit mast cell degranulation in different systems. The oral administration of nifedipine (10 mg) did not affect the size of codeine-induced skin reactions in ten normal volunteers. Mean wheal over flare sizes were 11.7 mm/29.2 mm before nifedipine and 11.5 mm/31.0 mm at peak nifedipine blood levels. Similar observations were made when codeine was injected locally with or without 20 micrograms nifedipine (12.1/29.2 mm and 13.2/29.2 mm, respectively). These data suggest that codeine-induced mast cell degranulation may be mediated by a calcium-independent mechanism. Alternatively, mast cells in the human skin may differ in their reactions to secretagogues when compared with basophils and mast cells from other human tissues or other species.
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A 21-month-old child with acute febrile dermatosis, leukocytosis and skin biopsy showing intradermal neutrophil infiltrate was diagnosed as suffering from Sweet syndrome. This syndrome is rare in children, described heretofore in only seven paediatric patients. The clinical and histological features, differential diagnosis, as well as the potential association with more widespread systemic diseases are discussed.
Three patients developed severe incapacitating diffuse pain and tenderness of the skeletal muscles after acute streptococcal infection. There was no evidence of concomitant arthritis, glomerulonephritis, or inflammatory muscle disease in any of the cases. All patients responded promptly to anti-inflammatory therapy. Severe myalgia should be considered an additional complication of immunologically mediated poststreptococcal response.