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A Rare Occurrence of Microscopic Polyangiitis and Aortitis

Akshat Sahai1, Atul Bhasin2, Chittimalla Gopinath3

  • 1Medical Officer, Department of Internal Medicine, BLK-Max Super Speciality Hospital, New Delhi, India, Corresponding Author.

The Journal of the Association of Physicians of India
|March 12, 2026
PubMed

Insights

A rare case of fever of unknown origin (FUO) was diagnosed as perinuclear antineutrophil cytoplasmic antibodies (p-ANCA)-associated microscopic polyangiitis (MPA) and aortitis. This highlights diagnostic challenges and the need for clinician awareness in managing severe multisystemic vasculitis.

Area of Science:

  • Internal Medicine
  • Rheumatology
  • Vascular Medicine

Background:

  • Fever of unknown origin (FUO) presents diagnostic challenges, especially with rare multisystemic involvement.
  • Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are uncommon but can lead to severe systemic complications.
  • Microscopic polyangiitis (MPA) and aortitis require timely diagnosis and management to prevent organ damage.

Purpose of the Study:

  • To report a unique case of FUO diagnosed as p-ANCA-associated MPA and aortitis.
  • To emphasize the diagnostic complexities associated with rare vasculitic syndromes.
  • To highlight the importance of recognizing and managing severe multisystemic inflammatory conditions.

Main Methods:

  • A comprehensive diagnostic workup was performed for a 60-year-old male presenting with FUO.
  • Diagnostic investigations included serological tests for ANCA, imaging studies, and potentially biopsy (details not specified in abstract).
  • Treatment strategies were tailored to the specific diagnosis of p-ANCA-associated MPA and aortitis.

Main Results:

  • The patient was diagnosed with perinuclear antineutrophil cytoplasmic antibodies (p-ANCA)-associated microscopic polyangiitis (MPA) and aortitis.
  • The diagnosis explained the patient's prolonged fever and systemic symptoms.
  • Effective management strategies were initiated based on the confirmed diagnosis.

Conclusions:

  • This case illustrates the diagnostic difficulties in FUO when rare conditions like p-ANCA-associated MPA and aortitis are present.
  • Clinician awareness is crucial for the prompt recognition of severe, multisystemic vasculitic presentations.
  • Early and accurate diagnosis is essential for effective therapeutic intervention and improved patient outcomes in complex inflammatory diseases.

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