Immunologic abnormalities in hemodialysis patients: improvement after pyridoxine therapy.

8 male patients undergoing maintenance hemodialysis were studied to determine the effect of administering supplements of pyridoxine hydrochloride, 50 mg/day for 3-5 weeks, on tests of immune function. In the 3 patients who initially had abnormal nitroblue tetrazolium reduction tests, the values returned to normal with therapy (p less than 0.05). The generation of chemotactic factors from plasma was defective in all evaluated patients and improved after pyridoxine therapy in 4 of 5 patients (p less than 0.01). The lymphocyte subpopulations changed with a rise in the populations of null cells after supplementation with pyridoxine. In addition, lymphocyte transformation in response to mitogens improved in the 3 patients who initially showed low values in these assays. The improvements occurred with pyridoxine therapy even though some patients who responded had no evidence for vitamin B6 deficiency before therapy, as indicated by a normal erythrocyte glumatic-pyruvic transaminase index. We conclude that several parameters of immune function are improved with pyridoxine supplementation. Studies are necessary to establish the minimum daily intake of pyridoxine which will maintain improved values of these tests of immune function in hemodialysis patients.

Scleromyxedema myopathy. Histochemical and electron microscopic observations.

A biopsy-proven case of scleromyxedema (papular mucinosis) with IgG lambda light chain paraproteinemia, eosinophilia and severe proximal myopathy is presented. Muscle biopsy revealed an atypical necrotizing vacuolar myopathy. Histochemical studies of cryostat sections revealed fiber necrosis, severe type II fiber atrophy, and fiber vacuolization with NADH tetrazolium reductase hyperactivity. Electron microscopy showed myocytolysis, reduplication of the basement membrane, and unit membrane-lined vacuoles negative for acid mucopolysaccharide.

Papular mucinosis with myopathy, arthritis, and eosinophilia. A histopathologic study.

A patient with biopsy-proven papular mucinosis, plus the characteristic IgG lambda light chain paraproteinemia, also developed a severe proximal myopathy, seronegative inflammatory polyarthritis, and marked eosinophilia. Muscle enzymes were elevated, EMG was compatible with polymositis, and muscle biopsy revealed an atypical necrotizing vacuolar myopathy. Synovial biopsy revealed an inflammatory synovitis with Class II synovial fluid. No mucin deposition was detectable in muscle or synovium. During 7 years of observation, corticosteroids and various immunosuppressive agents were successively administered with little benefit. Recently, weekly intravenous methotrexate and low-dose oral corticosteroids have resulted in clinical and laboratory improvement. It is suggested that the pathology in papular mucinosis may include serious rheumatic manifestations in addition to the cutaneous involvement.

Relapsing polychondritis: prospective study of 23 patients and a review of the literature.

Relapsing polychondritis (RP) is not a totally rare rheumatic disease. We have seen 23 patients from 1960-1975, and there are now a total of 159 reported cases, which form the basis of this study. RP occurs equally in both sexes, and has a maximum frequency in the fourth decade. 2) Empirically defined diagnostic criteria are proposed, to include the most common clinical features: a) Bilateral auricular chondritis b) Nonerosive sero-negative inflammatory polyarthritis c) nasal chondritis d) Ocular inflammation e) Respiratory tract chondritis f) Audiovestibular damage The diagnosis is based primarly upon the unique clinical features, and is quite certain if three or more criteria are present together with histologic confirmation. 3) Fifty percent of patients present with either auricular chondritis or the arthropathy of RP; but with prolonged follow-up, a majority of patients develop four or more of the above mentioned criteria. 4) Approximately 30 percent of patients have a preceding or coexistent rheumatic or autoimmune disease, which can lead to initial diagnostic confusion. 5) Laboratory and radiographic investigations help mainly to rule out other diagnostic possibilities, with no characteristic abnormalities being present in a majority of patients. 6) On follow-up, three-fourths of our patients required chronic corticosteroid therapy with an average dose of 25 mg per day of prednisone. Corticosteroids decrease the frequency, duration, and severity of flares, but do not stop disease progression in severe cases. 7) The mortality rate has been 30 percent in our series and 22 percent in the other 136 reported cases. Of the 29 cases where the cause of death was known, 17 were from respiratory tract involvement and 9 from cardiac valvular or vasculitic involvement, emphasizing the need to search for critical involvement of either of these organ systems in each patient. 8) Detailed reports of selected cases are presented to illustrate the clinical diagnosis and differential diagnosis, and to demonstrate the need for careful prolonged follow-up. 9) Although the etiology remains unknown, there is a frequent association with, and clinical similarity to, other rheumatic diseases. 10) Careful clinicopathological study of our 23 patients leads us to postulate an underying systemic vascultis as an important pathologic mechanism in RP.

The arthropathy of relapsing polychrondritis.

Twenty-three adults (11 males and 12 females) with well-defined relapsing polychondritis (RP) are studied in order to characterize the arthropathy of RP. Arthritis was found in 19 patients-as the presenting feature in 8 and as a significant symptom in 11 others. The usual pattern of involvement was migratory, asymmetric, non-nodular, nonerosive, and seronegative, and affected large and small joints as well as parasternal articulations. In addition RP was seen in 3 patients with preexisting chronic polyarthritis or associated rheuamtic disease.