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Related Experiment Videos

Skeletal findings in progressive systemic sclerosis (scleroderma).

L W Bassett, K L Blocka, D E Furst

    AJR. American Journal of Roentgenology
    |June 1, 1981
    PubMed
    Summary
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    Progressive systemic sclerosis can cause significant bone and soft tissue changes, including osteolysis and inflammatory arthritis. Radiographic review of 55 patients revealed common hand and foot deformities and joint destruction not linked to other connective tissue diseases.

    Area of Science:

    • Rheumatology
    • Radiology
    • Scleroderma Research

    Background:

    • Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease characterized by fibrosis, vascular abnormalities, and immune dysregulation.
    • Radiographic manifestations in PSS are diverse, but their specific patterns and prevalence require further elucidation, especially in patients without overlap syndromes.
    • Differentiating PSS-related musculoskeletal changes from other connective tissue diseases is crucial for accurate diagnosis and management.

    Purpose of the Study:

    • To characterize the spectrum of radiographic findings in the chest, hands, and feet of patients with progressive systemic sclerosis.
    • To identify common soft tissue and bony changes associated with PSS, excluding overlap syndromes like mixed connective tissue disease (MCTD).
    • To investigate the prevalence and nature of inflammatory arthritis in PSS patients without confounding autoimmune conditions.

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    Main Methods:

    • Retrospective review of radiographic images (chest, hands, feet) from 55 patients diagnosed with progressive systemic sclerosis.
    • Patient selection criteria excluded individuals with overlap syndromes, particularly mixed connective tissue disease.
    • Detailed analysis of soft tissue abnormalities (deformities, atrophy, calcifications) and bony changes (osteolysis, resorption).

    Main Results:

    • Common soft tissue changes included flexion deformities, generalized/localized atrophy, and dystrophic calcifications.
    • Resorption of distal phalanges was the most frequent bony alteration; osteolysis was also noted in feet, ribs, and mandibles.
    • Twelve out of 55 patients exhibited radiographic evidence of inflammatory arthritis, ranging from isolated to generalized joint destruction.

    Conclusions:

    • Progressive systemic sclerosis is associated with a distinct pattern of soft tissue and bony abnormalities on radiography.
    • Osteolysis and inflammatory arthritis can occur in PSS, even in the absence of rheumatoid arthritis or mixed connective tissue disease.
    • Radiographic assessment plays a vital role in identifying and characterizing the musculoskeletal manifestations of progressive systemic sclerosis.