A 71-year-old male presented with 4 months of fatigue, low-grade fevers, night sweats, dry cough, and a 10-pound weight loss. He also recalled developing a central ulcerating pustular lesion on his right anterior shin a few months ago, that spontaneously resolved. Significant history included chronic sinusitis with nasal polyposis requiring 4 nasal polypectomies, as well as adult-onset asthma since his 20s. He had been on a “Mexican medication” for 20 years for his asthma, which he self-discontinued 4 months prior to presentation.
This prompted an extensive workup with multi-specialty involvement. Labs were remarkable for normocytic anemia, elevated inflammatory markers, and progressively increasing peripheral eosinophilia associated with elevated IgE levels 486 UI/ml; ANA, ANCA and rheumatoid serologies were negative. Serial CT chests revealed progressive bilateral micronodular infiltrates suspicious for inflammatory/infectious respiratory bronchiolitis. Bronchoscopy with BAL and subcarinal lymph node biopsies demonstrated 11% eosinophils and non-caseating granulomas respectively. Chronic rhinosinusitis was confirmed on maxillofacial CT.
In light of the history of chronic rhinosinusitis, adult-onset asthma, increasing eosinophilia with elevated IgE levels, and suggestive lymph node biopsies, a clinical diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome [CSS]) was made, on the grounds that ANCA serologies may be negative in up to 40-50% patients. It was then discovered that his “Mexican medication” contained oral triamcinolone, a corticosteroid.
He was initiated on high dose methylprednisolone and methotrexate, with resolution of his constitutional symptoms.
This case demonstrates the elusive, misleading manifestations of CCS and how administration of chronic corticosteroids can further mask its presentation – a phenomenon known as “forme fruste”. In medicine, a forme fruste (French, “crude, or unfinished, form”) is an atypical or attenuated manifestation of a disease or syndrome, suggesting incompleteness, partial presence, or an aborted state. This case also highlights the importance of obtaining a comprehensive history of a patient’s entire medication regimen, including complementary therapies, herbs, and remedies from “over the border.”
CSS is a rare disease belonging to the ANCA vasculitis class. It is differentiated by its initially deceptive allergic presentation and hypereosinophilia. Being a vasculitic disorder, this disease eventually leads to multisystem organ dysfunction. However, at onset, it frequently poses as an innocent atopic syndrome that is often misdiagnosed and disregarded for decades before its more sinister features arise. Although uncommon, physicians should remain aware of this phenomenon, especially when altering the medication regimen of an asthmatic patient on chronic steroid therapy.
To cite this abstract:Zhou, JX; Ram, A; Tourtellotte, L . POOR FORME: “ADULT ONSET ASTHMA” WITH AN UNEXPECTED VASCULITIC TWIST. Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev. Abstract 808. Journal of Hospital Medicine. 2017; 12 (suppl 2). https://www.shmabstracts.com/abstract/poor-forme-adult-onset-asthma-with-an-unexpected-vasculitic-twist/. Accessed February 25, 2020.