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[Systemic scleroderma and sarcoidosis: 3 new cases]

M D Bandt1, O Meyer, C Masson

  • 1Service de Rhumatologie, Hôpital Bichat, Paris.

Annales De Medecine Interne
|January 1, 1996
PubMed
Summary

This study reports on three patients with successive scleroderma and sarcoidosis, highlighting the diagnostic challenges and poor corticosteroid response in pulmonary involvement.

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Pathology

Background:

  • Scleroderma (SS) and sarcoidosis (SA) are systemic autoimmune diseases.
  • Co-occurrence of SS and SA presents diagnostic complexities, particularly in pulmonary manifestations.

Observation:

  • Three patients presented with sequential diagnoses of SS followed by SA.
  • SS diagnosis included Raynaud's phenomenon, sclerodactyly, joint pain, and positive antinuclear antibodies.
  • SA diagnosis involved Löfgren's syndrome, parotiditis, sicca syndrome, granulomas in various organs, and elevated ACE levels.

Findings:

  • Two patients developed SA 5-9 years after SS diagnosis.
  • All patients initially had restrictive pulmonary disease.
  • Pulmonary involvement was a significant feature, with one patient developing pulmonary artery hypertension.

Implications:

  • The rare association of SS and SA underscores overlapping systemic disease features.
  • Differentiating pulmonary involvement between SS and SA is challenging.
  • Pulmonary manifestations in this combined condition are severe and poorly responsive to corticosteroids.

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