UCTD or U1 RNP-associated connective tissue syndrome.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M F Kahn.
Explore the source record for details and available documents.
Psoriatic arthritis is an inflammatory arthropathy associated with psoriasis, and its clinical presentation varies from case to case. Distal interphalangeal involvement is characteristic but not seen in all patients. Enthesopathy, including that of the spine, is common and contributes to the classification of psoriatic arthritis as a seronegative spondyloarthropathy. The etiopathogenesis of psoriatic arthritis is not well understood, and evolution as measured by follow-up is variable. Treatment includes nonsteroidal anti-inflammatory drugs and some of the drugs used in the treatment of rheumatoid arthritis. Sulfasalazine and, in the more severe cases, cyclosporine, are being studied for efficacy and tolerance. Some cases of psoriatic arthritis are associated with an inflammatory bone disease, frequently seen on the anterior chest wall, which is part of the newly described SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome. SAPHO syndrome is characterized by this bone involvement, which can affect the spine and peripheral bones. Various skin conditions are associated with this syndrome, but they do not necessarily occur in all cases. Chronic recurrent multifocal osteomyelitis, which is seen mostly in children, may be a presentation of SAPHO syndrome. Associations with sacroiliitis, bowel disease, and psoriasis link SAPHO syndrome with the spondyloarthropathies.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Epithelioid hemangioendothelioma is a rare, low-grade vascular malignancy reported for the first time in 1982 by Weiss and Enzinger. It involves one or, more rarely, several organs. We report a case involving the lungs and liver, in which the first manifestation was symptomatic hypertrophic osteoarthropathy. Findings four years after the diagnosis included very slow tumor spread, resolution of symptoms, and stabilization of radiological changes.
The interactions between pregnancy and adult-onset Still's disease are unclear. Nine pregnancies which occurred from 1983 through 1991 in seven women (mean age 27.7 years; range 23-40) were studied retrospectively. Five patients had one pregnancy and two had two pregnancies. Onset of the joint disease occurred before or during pregnancy. The joint disease occurred at the 5th or 6th month of the first pregnancy in two patients; one of these patients had a second pregnancy which was not associated with a flare. In five patients, onset of the joint disease occurred 4, 15, 42, 44 and 58 months before the first pregnancy, respectively; any effects of the joint disease on the pregnancy were extremely variable. 6 infants were full-term and two were premature (35 weeks); the remaining pregnancy was electively terminated; Apgar scores were normal in all eight infants, who were all male. Adult-onset Still's disease did not influence the outcome of the pregnancy in our patients; no clear-cut effects of pregnancy on adult-onset Still's disease were seen.
Intravesical administration of Calmette-Guérin bacillus (BCG) is effective in the treatment of superficial bladder carcinoma. Transient arthritis or migratory arthralgia has been reported in 0.5% of cases. The authors report on four men (mean age 65 years), who developed an oligoarthritis in three cases after the 2nd, 5th, and 6th weekly instillation of Pasteur BCG, respectively, and symmetrical polyarthritis in one after 48 BCG instillations over a three-year period. Joints involved were the knees (3/4), ankles (3/4), shoulders (1/4), wrists, and hands (1/4). Features included morning stiffness and local evidence of inflammation. All four patients had an elevated erythrocyte sedimentation rate and negative tests for rheumatoid factor. Neutrophils were the main cell type in synovial fluid. Synovial biopsy performed in two cases revealed non-specific inflammation. Erosions of the metatarsophalangeal joints were observed in one patient. Locoregional or systemic symptoms included transient fever (3/4), cystitis (4/4), urethritis (1/4), epididymoorchitis (2/4), conjunctivitis (1/4), and pleural effusion with pericarditis (1/4). The BCG was recovered from an epididymal specimen in one patient. Two patients were positive for the HLA B27 antigen. Outcome was favorable in every case (range: 15 days-6 months) after discontinuation of BCG therapy and administration of isoniazid, rifampin, and prednisone (3 patients) or NSAID (1 patient). Various pathogenic hypotheses for BCG-induced arthritis are discussed, including infection, immune responses to heat shock proteins, and reactive arthritis.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A case of severe peripheral nerve lesions in a patient with eosinophilia-myalgia syndrome induced by L-Tryptophan is reported. Both superficial radial nerves were very tender on palpation. Biopsy of the radial nerve showed myelin abnormalities of the type observed in experimental chronic nerve entrapment. Nerve lesions included asymmetrical distortion of the myelin sheath, segmental demyelination, axonal degeneration and regeneration. Such abnormalities suggest that mechanical factors, presumably due to proliferation of connective tissue, play a role in the nerve lesions observed in this patient.
Explore the source record for details and available documents.
Congenital heart blocks due to immunological causes are rare. A case is reported of a fetus with auriculo-ventricular block diagnosed at 22 weeks of amenorrhoea and intrauterine death at 32 weeks. The authors discussing the case find the most likely link: an anti-RO (SS-A) and anti-LA (SS-B) immunological block and they suggest that there are minor localised lesions in the nodal tissue which gives rise to benign disturbances of cardiac rhythm and they point out ways of preventing intrauterine auriculo-ventricular block.
Explore the source record for details and available documents.
Lupus mastitis is an infrequent dermatologic disease. We describe a case of bilateral lupus mastitis heralding a severe systemic lupus erythematosus with the antiphospholipid syndrome.
We describe 3 cases of hypocomplementemic urticarial vasculitis syndrome (HUVS) with Jaccoud's hands deformity and cardiac valve disease (aortic regurgitation, mitral regurgitation, mitral disease). In one case, the valve lesions required valve replacement and later a heart transplant. Valve disease and articular deformities developed 2 and 4 years, respectively, after the onset of HUVS. This as yet undescribed combination of diseases suggests a new syndrome. Pathogenesis of periarticular and cardiac lesions is unknown. The role of Clq and anti-Clq antibody is discussed.
A number of occupational exposure risks have been suspected as potential causes of scleroderma (systemic sclerosis), particularly in males. These include silica dust and numerous organic chemicals. With particular reference to the type of employment, we have reviewed the charts of male scleroderma patients receiving treatment in our Rheumatology Department since 1976. Of 39 patients, 25 could be included in the present study. Among these, 14 (56%) had an occupational activity which put them at risk: 11 were exposed to silica dust, one to organic chemicals and two to both silica dust and chemicals. Mean exposure time was 14.5 years (range 4-33 years); and the interval between the beginning of exposure and the onset of scleroderma averaged 24.4 years (range 4-45 years). Considering these 14 cases as silica- or chemically-induced scleroderma, we found that, except for the absence of muscular involvement, the clinical features and prognosis were similar to those of "idiopathic" scleroderma patients. Antinuclear antibodies and autoantibodies directed against Scl-70 were also found in both occupationally induced and idiopathic scleroderma. Our results emphasize the unusually high frequency of occupational exposure in scleroderma in males. Hence, a detailed professional history should be taken for all scleroderma patients. Our results also suggest that, in certain cases, scleroderma should be classified as an occupational disease.
Sciatica caused by intervertebral disc herniation can be treated with intradiscal injection of chymopapain. A search for a cheaper and less allergizing product led to triamcinolone hexacetonide, this procedure being known as "nucleorthesis". The first results at 6 months were encouraging. In 3 centres where triamcinolone hexacetonide was tested with a more than 2 years' follow-up 92 patients could be evaluated. The results obtained were considered satisfactory in 34 patients (36.9 percent), but they were poor in 19 patients (20.6 percent), and 39 patients (42 percent) had to be operated upon within 2 years. Return to surgery took place within the 6 months following nucleorthesis in 18 patients (19.56 percent) and beyond this period in 17 patients (22.8 percent) with degradation of the results. Moreover, calcifications were found in 19 out of 38 patients; they were of varying size, sometimes detected only at computerized tomography, and some of them appeared to produce symptoms. All considered, the failure rates, the number of patients who required surgery and the occurrence of large and sometimes symptomatic calcifications make triamcinolone nucleorthesis unacceptable compared with the recognized percentages of success with papain nucleolysis and surgical operations. For these reasons, we consider that this treatment should be abandoned.