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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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Granulomatosis with polyangiitis - the incomplete puzzle

Ana Catarina Duarte1, Ana Cordeiro1, Pedro Gonçalves1

  • 1Serviço de Reumatologia, Hospital Garcia de Orta.

Acta Reumatologica Portuguesa
|December 28, 2021
PubMed

Insights

Granulomatous with polyangiitis (GPA) can initially present with musculoskeletal symptoms, mimicking rheumatic diseases. Early recognition of these signs is crucial for timely GPA diagnosis and management.

Area of Science:

  • Rheumatology
  • Vasculitis
  • Immunology

Background:

  • Granulomatous with polyangiitis (GPA) is a small-vessel vasculitis characterized by necrotizing granulomas.
  • Renal and respiratory tract involvement are common, but musculoskeletal manifestations occur in approximately 50% of patients.
  • Musculoskeletal symptoms can precede other GPA signs, potentially delaying diagnosis.

Purpose of the Study:

  • To highlight the diagnostic challenge posed by early musculoskeletal manifestations in GPA.
  • To illustrate how GPA can be mistaken for other rheumatic conditions when joint and muscle symptoms are the initial presentation.

Main Methods:

  • Case series describing three patients with GPA.
  • Focus on the initial presentation of musculoskeletal symptoms.
  • Review of clinical course and diagnostic process.

Main Results:

  • All three patients presented with musculoskeletal symptoms as their earliest manifestation of GPA.
  • Diagnosis of GPA was delayed in these cases due to initial misinterpretation as other rheumatic diseases.
  • The described cases underscore the variability of GPA presentation.

Conclusions:

  • Musculoskeletal symptoms can be the primary presenting feature of GPA.
  • Clinicians should consider GPA in patients with unexplained musculoskeletal complaints, especially when other systemic signs are absent.
  • Prompt recognition of GPA in this context is essential to prevent disease progression and organ damage.

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