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Acro-osteolysis and mononeuritis multiplex as a presenting symptom of systemic angiitis of Wegener's type

M Modi1, A K Vats, S Prabhakar

  • 1Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Indian Journal of Medical Sciences
|April 3, 2007
PubMed

Insights

This case study highlights a rare limited form of Wegener's granulomatosis presenting with digit resorption and nerve damage. Early immunosuppressive therapy led to significant improvement in this oligosymptomatic vasculitis case.

Area of Science:

  • Rheumatology
  • Vasculitis Research
  • Immunopathology

Background:

  • Wegener's granulomatosis (WG) is a systemic vasculitis affecting small-to-medium vessels, typically involving the respiratory tract and kidneys.
  • Limited or oligosymptomatic forms of WG can present with atypical manifestations and unusual site involvement.
  • Early diagnosis and treatment are crucial for managing WG to prevent severe organ damage.

Observation:

  • A rare case of limited WG presented with sequential spontaneous resorption of digits and acro-osteolysis.
  • The patient also developed mononeuritis multiplex over a 10-month period.
  • High proteinase 3 antibodies (c-ANCA) were detected, with minimal lung involvement evident only on high-resolution computed tomography (HRCT).

Findings:

  • The patient's presentation mimicked other conditions due to oligosymptomatic and atypical manifestations.
  • Diagnostic workup confirmed WG with characteristic c-ANCA positivity.
  • Aggressive immunosuppressive therapy resulted in marked clinical improvement.

Implications:

  • This case underscores the importance of considering limited WG in patients with unexplained digital resorption, acro-osteolysis, and mononeuritis multiplex.
  • It highlights the diagnostic utility of c-ANCA testing and HRCT in identifying subtle respiratory involvement.
  • Effective management with immunosuppression can lead to favorable outcomes even in atypical WG presentations.

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