Geraldine Z. Racaza, Lauro L. Abrahan, Katrina Angela Z. Reyes,
Ester G. Penserga Related Institution
Department of Medicine - University of the Philippines - Philippine General Hospital Publication Information
Publication Sub Type
Philippine Journal of Internal Medicine
Background: Polyangiitis overlap syndrome (POS), a systemic vasculitis not classifiable into well-defined syndromes, is diagnosed based on combined characteristics of two or more primary systemic vasculitides, such as Takayasu arteritis and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but not two AAVs. Our objective is to present such, with associated multiple infections.
Case: A 25-year-old Filipino female had six years of recurrent purpura, debilitating joint pains, hemoptysis, rhinorrhea, epistaxis, eye redness, dyspnea, and abdominal cramps. She was cachectic, had oral ulcers, bibasal crackles, polyarthritis, and generalized purpura. Work-up showed anemia, eosinophilia excluding parasitism, elevated inflammatory markers,and positive cytoplasmic (c)-ANCA and ant i - proteinase 3. There was chronic sinusitis on rigid rhinoscopy, middle to lower lung field interstitial infiltrates on radiographs, pulmonary vasculitis on chest CT, moderate pulmonary hypertension on echocardiography, but normal spirometry. Skin biopsy showed leukocytoclastic vasculitis without necrotizing granulomas. Serology showed chronic hepatitis B infection. Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus grew from bronchial washings and nasal swabs, respectively. ASO titer was high. Human immunodeficiency virus infection was ruled out. She was diagnosed with POS, with features of two AAVs – limited granulomatosis with polyangiitis (GPA) and Churg-Strauss syndrome (CSS), associated with multiple infections, and was given naproxen and culture-guided antibiotics (ciprofloxacin, clindamycin, penicillin), with partial resolution of symptoms. Prednisone 10 mg once a day (OD) was started two weeks after hepatitis B reactivation prophylaxis with lamivudine 100 mg OD. Complete resolution of skin lesions and upper airway symptoms and inflammatory marker improvement were observed after 12 weeks. She is maintained on prednisone 5.0 mg daily.
Conclusion and Recommendations: This is the first reported case of POS involving two AAVs – limited GPA and incomplete CSS, associated with multiple pathogens. Identifying POS is important; inability to classify patients into well-recognized vasculitic syndromes delays treatment. The infections likely perpetuated the vasculitis, and both antimicrobials and immunosuppression were necessary to induce disease remission.