[DIAGNOSIS OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-NEGATIVE EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS BASED ON...

Area of Science:

• Nephrology
• Rheumatology
• Pathology

Background:

• Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis usually associated with asthma and eosinophilia.
• Atypical EGPA presentations, particularly those lacking asthma, are rarely documented.

Purpose of the Study:

• To report a case of EGPA in a patient without asthma, highlighting diagnostic challenges and considerations.
• To emphasize the importance of considering EGPA in the differential diagnosis of renal impairment with eosinophilia.

Main Methods:

• A case presentation of a middle-aged male with fever, dyspnea, rash, eosinophilia, and multi-organ dysfunction.
• Diagnostic workup included peripheral blood tests, myocardial, gastrointestinal, and renal biopsies.
• Treatment involved systemic corticosteroids, cyclophosphamide, and intravenous immunoglobulin.

Main Results:

• Renal biopsy revealed pauci-immune necrotizing crescentic glomerulonephritis with eosinophilic interstitial nephritis.
• Despite negative ANCA, the patient met EGPA classification criteria.
• The patient achieved remission following treatment and hospital discharge.

Conclusions:

• Eosinophilia and characteristic renal pathology are crucial diagnostic indicators for EGPA, even in ANCA-negative patients without asthma.
• EGPA should be considered in the differential diagnosis of renal impairment accompanied by eosinophilia.
• Early renal biopsy is recommended for patients with suspected EGPA and renal involvement.