The Nature of Joint Involvement in Progressive Systemic Sclerosis (Diffuse Scleroderma)

Clinical Study and Pathologic Examination of Synovium in Twenty-nine Patients

  1. GERALD P. RODNAN, M.D.
  1. Requests for reprints should be addressed to Gerald P. Rodnan, M.D., Department of Medicine, University of Pittsburgh School of Medicine,
    Pittsburgh 13, Pennsylvania
    .

Excerpt

Involvement of the joints in progressive systemic sclerosis was first noted in 1847 by Forget who described a 33-year-old woman whose initial symptoms were pain and swelling of many joints and whose "wrists still bore the traces of leeches and of cupping glasses" (1). The great frequency of these and other joint complaints at the onset or during the course of scleroderma was soon recognized and interpreted variously as indicative of rheumatism or simply related to change in the overlying skin. In 1896 Dercum secured roentgenograms of the hands of a patient with scleroderma and noted narrowing of the proximal

Acknowledgment

The author wishes to thank Dr. Joseph J. Bunim for permission to include cases 1 through 6, patients seen at the National Institute of Arthritis and Metabolic Diseases between 1953 and 1955.

Summario in Interlingua

Le examine de specimens de synovio obtenite—per biopsia o post morte—ab 29 patientes con ben-documentate progressive sclerosis systemic (scleroderma diffuse) ha revelate alterationes pathologic de frequentia e de intensitate sufficiente pro explicar, al minus in parte, le symptomas articular que occurre si communmente in iste disordine.

Esseva trovate, a generalmente parlar, que le specimens ab patientes con acute symptomas e signos de inflammation monstrava un synovitis characterisate per le infiltration de lymphocytos e de plasmocytos que esseva presente como aggregatos focal o in dispersion diffuse in omne partes del tissue.

Plus tarde in le curso del morbo il occurre un intense fibrosis del synovium, atrophia del cellulas de revestimento interior, e sclerosis del vasos de sanguine. A iste stadio le lesion del synovio resimila le lesion vidite in le derme (scleroderma) e pare esser satis distinctive pro progressive sclerosis systemic. Patientes con tal sclerosis del synovio se plange usualmente de rigiditate e de restriction del mobilitate articular. Un numero de illes ha notate e reportate le disveloppamento de ruitos articular, e un peculiar crepito corioide poteva esser evocate in illes per palpitar le patella durante un movimento del genu.

Alterationes roentgenographic in le articulationes major es restringite in grande mesura a grados moderate de reduction del spatio de cartilagine e de osteoporosis juxta-articular.

Positive reactiones hemagglutinatori o de fixation a latex con basse titros (o ambes) esseva presente in novem de 21 patientes testate (43%).

Reguardate globalmente, le characteristicas clinic e pathologic del affection synovial in iste disordine differe ab illos de arthritis rheumatoide e de altere formas de systemic morbo rheumatic. Il pare que illos es un parte integral e distinctive de progressive sclerosis systemic.

Article and Author Information

  • From the Department of Medicine, University of Pittsburgh School of Medicine.

  • Supported in part by a special research fellowship, National Heart Institute (HF-5493) and a grant (A-97) from the Health and Research Services Foundation of Allegheny County.

    • Received November 17, 1961.
    • Accepted December 26, 1961.
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