Rodney Bluestone

High Association of an HL-A Antigen, W27, with Ankylosing Spondylitis

Abstract The frequencies of 24 HL-A antigens were examined in 40 patients with ankylosing spondylitis, 119 with rheumatoid arthritis, and 66 with gout. No significant deviation from control frequen...

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.

Polymyositis and dermatomyositis: combined methotrexate and corticosteroid therapy.

Abstract Twenty-two patients with polymyositis and dermatomyositis were treated with combined prednisone and intravenous methotrexate when moderate to high-dose cortisone alone was ineffective in c...

HL-A W27 — A Clue to the Diagnosis and Pathogenesis of Reiter's Syndrome

RECENTLY, an extraordinary correlation between the second-segregant series histocompatibility antigen (HL-A), W27, and a well characterized rheumatic disease, ankylosing spondylitis was reported.1 ...

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.

Polyarticular versus monoarticular gout: a prospective, comparative analysis of clinical features.

This investigation was undertaken to define prospectively the clinical characteristics of patients with crystal-documented gouty arthritis simultaneously involving multiple joints. Of 106 consecutive patients with gouty arthritis (GA), 42 (40%) had articular inflammation at 2 or more sites. Comparison of these 42 patients with GA with the 64 patients with GA who presented with monoarthritis yielded the following conclusions: 1) Polyarticular gout represents one end of a generally predictable spectrum of GA, reflecting chronicity associated with poor patients understanding, poor patient compliance, and suboptimal physician management. 2) Polyarticular patients with GA tend to develop attacks of more smoldering onset and increasing duration, while joint involvement tends to occur in an ascending but asymmetrical fashion, with upper extremity joints later added to repeatedly active lower extremity sites. 3) There may be a significant discrepancy between the site (or sites) of the GA patients chief complaint and clinically involved joints on careful physical examination. 4) Recognition of polyarticular joint involvement increases the number of sites for potential joint and/or tophus aspiration, permitting greater ease of establishing a definitive diagnosis. 5) No single laboratory or synovial fluid value meaningfully distinguishes patients with polyarticular from those with monoarticular gout.

Hyperuricemic Nephropathy: Pathologic Features and Factors Influencing Urate Deposition

The three general types of renal pathology associated with hyperuricemia are reviewed. Factors influencing urate solubility are discussed, as well as the effect of uricosuric drugs on renal urate handling, with particular emphasis upon their efficacy in competing with urate for protein binding sites. In addition, a new experimental model of hyperuricemic nephropathy is described which could yield valuable data concerning the complex relationships between hyperuricemia, urate deposition and renal function.

Acute gastric mucosal injury during continuous or interrupted aspirin ingestion in humans

The effect of continuous versus interrupted high-dose aspirin (ASA) for 14 days was evaluated in a randomized double-blind study in 8 rheumatoid arthritis patients. Acute gastric mucosal injury was measured by serial gastroscopy and gastric biopsy. Significant gross mucosal damage was seen in all patients following 3 days of ASA (P<0.01) and persisted without significant change in severity to the end of the study. Histologic gastritis in areas free of hemorrhages and erosions was not increased significantly by ASA. In spite of gross mucosal injury, symptoms occurred infrequently. Serum pesinogen I, but not serum gastrin, increased significantly following 3 days of ASA, and the elevation persisted to the end of the study. The extent of mucosal injury at 14 days was not significantly different in those receiving ASA continuously from those on an interrupted schedule. Thus, gastric mucosal adaptation to ASA in man was not demonstrated.

Effect of drugs on urate binding to plasma proteins

The effect of various drugs on urate binding to plasma proteins was investigated in normal subjects. Whereas allopurinol, aspirin, phenylbutazone, probenecid, and sulphinpyrazone all significantly reduced plasma urate concentrations, only aspirin, phenylbutazone, and probenecid significantly impaired urate binding. Colchicine and indomethacin in the doses administered had no significant effect on plasma urate concentrations or binding. In the case of aspirin, urate binding was reduced to 25% of normal, and this effect was quickly abolished after cessation of therapy. Phenylbutazone reduced urate binding to 56% and probenecid to 46% of normal; this impairment was still detected four days after cessation of therapy. Drugs may impair urate binding by competition for plasma protein binding sites, with displacement of bound urate. Impairment of urate binding in vivo by administration of certain drugs may be relevant to the precipitation of acute gouty arthritis, to the formation of gouty tophi, and to the augmentation of uricosuria. Furthermore, the role of drugs must be seriously considered during all studies on urate binding in patients with gout.

Juvenile rheumatoid arthritis: A serologic survey of 200 consecutive patients*

A search for serologic abnormalities in 200 patients with juvenile rheumatoid arthritis revealed rheumatoid factor in 12 per cent and antinuclear antibody in 4 per cent. Elevated levels of serum IgG were noted in 25 per cent of patients, of IgA in 17 per cent, and of IgM in 10 per cent. From our observations, it appears that immunologic abnormalities are associated with specific clinical manifestations, hip disease, subcutaneous nodules, lymphadenopathy, and greater degrees of functional impairment.