Dermatomyositis without creatine kinase elevation. A poor prognostic sign.

Serum muscle enzyme levels are usually elevated in patients with untreated polymyositis and dermatomyositis. Creatine kinase is the muscle enzyme most often used to diagnose inflammatory myopathies. Seven patients with dermatomyositis and normal creatine kinase levels are described. Five of the seven patients had either an associated malignancy or severe interstitial lung disease. The one-year survival of the six patients followed for that length of time was 33 percent. A lack of creatine kinase elevation in patients with dermatomyositis is a poor prognostic sign.

Transient effect of nifedipine on pulmonary hypertension of systemic sclerosis.

A 64-year-old woman with systemic sclerosis and pulmonary hypertension underwent Swan-Ganz catheterization to test her response to the calcium channel blocker nifedipine. Pulmonary vascular resistance decreased from 2095 to 1333 dynes sec cm-5 with an initial dose of the drug. She developed tolerance to nifedipine within 12 h and subsequent doses had no effect on her pulmonary circulation. Our study confirms that acute administration of nifedipine reduces pulmonary vascular resistance in systemic sclerosis. However, an initial response to nifedipine does not predict benefit from continued administration of the drug.

Demonstration of a chemotactic factor receptor on macrophages.

Certain synthetic N-formylated peptides are potent chemotactic agents for phagocytic cells. We have identified a specific, high affinity receptor for the chemotactic peptide fMet-Leu-[3H]Phe on inflammatory as well as on resident guinea pig peritoneal macrophages. The receptor on inflammatory macrophages has an equilibrium dissociation constant (KD) of 11 nM at room temperature, and there are approximately 10,000 binding sites per cell. The receptor on resident peritoneal macrophages has a KD of 7 nM with approximately 12,000 sites per cell. The increased chemotactic responsiveness of inflammatory macrophages as compared to resident macrophages is probably not due to differences in the fMet-Leu-[3h]phe receptor since the number of binding sites per cell and the KD are quite similar. The specificity of the binding site on both cell types for a series of N-formylated peptides correlates well with the ability of the peptides to initiate macrophage chemotaxis. These studies suggest that the chemotactic response of guinea pig peritoneal macrophages to N-formylated peptides is initiated by the binding of the peptides to a specific cell-surface receptor.

Scleromyxedema with systemic involvement mimics rheumatic diseases.

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud's phenomenon. Scleromyxedema with systemic involvement may mimic rheumatic diseases.

Deferoxamine induced decreases of lipid peroxides in rheumatoid arthritis.

Eleven patients with rheumatoid arthritis (RA) were treated with intraarticular deferoxamine or placebo to test the hypothesis that iron chelation decreased hydroxyl radical mediated lipid peroxidation in RA. Intraarticular administration of deferoxamine 100 mg resulted in predominantly systemic effects with decreased serum ferritin and decreased serum levels of lipid peroxidation products. Similar changes were not detected in synovial fluid at this dose. Iron chelation with deferoxamine may provide a novel approach to preventing tissue injury in RA by inhibiting hydroxyl radical production and lipid peroxidation.

Acromegalic arthropathy. Characteristics and response to therapy.

Arthropathy was assessed in 19 patients with active acromegaly. Axial or peripheral arthropathy was present in 10 patients, was located most often in large joints or the lumbosacral spine, and was osteoarthritic in nature. The mean duration of acromegaly in patients with arthropathy was 21.6 years, while the mean duration in patients without arthropathy was 7.9 years. A mild-to-moderate improvement in symptoms, estimated functional ability, and crepitus occurred in 8 of 9 patients who were prospectively examined during therapy to lower production of growth hormone. We conclude that this therapy did improve the symptoms of acromegalic arthropathy. Whether objective structural improvements occur remains unclear.

Comparison of azathioprine, methotrexate, and the combination of both in the treatment of rheumatoid arthritis. A controlled clinical trial.

OBJECTIVE: To compare the relative safety and efficacy of azathioprine (AZA), methotrexate (MTX), and the combination of both in the treatment of active rheumatoid arthritis (RA). METHODS: Two hundred twelve patients with active RA were entered into a 24-week prospective, controlled, double-blind, multicenter trial and were randomly assigned to 1 of 3 treatment groups. RESULTS: One hundred fifty-eight patients finished 24 weeks of the study. There were no remissions seen but response rates were greater than 30% for all outcome measures. Combination therapy was not statistically superior to MTX therapy alone, but both combination therapy and MTX alone were superior to AZA alone when patients were analyzed by intent-to-treat and with withdrawals treated as therapy failures. If only patients who continued taking the therapy were analyzed, the mean improvement was greater for AZA therapy than for MTX, while the combination remained the most active. Adverse effects on the gastrointestinal tract and elevations of liver enzyme levels were the most frequent causes for discontinuations. CONCLUSION: Both combination therapy and MTX alone were superior to therapy with AZA alone for active RA but were not statistically different in their effect on outcome assessment.