Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formally known as Churg-Strauss, is a rare multisystem vasculitis characterized by asthma, rhinosinusitis and eosinophilia. The lungs are the most common organ system affected, followed by the skin. Extrapulmonary organ involvement, specifically cardiac involvement, is the leading cause of morbidity and mortality associated with the disease [1]. Disease remission is usually achieved with high dose corticosteroids or immunosuppressive therapy. The clinical features of EGPA typically evolve over several stages, which include a prevasculitic or prodromal stage, an eosinophilic stage, and finally, a life-threatening vasculitic stage. Historically, EGPA was a pathologic diagnosis, meaning that overt vasculitis had to be present in order to establish a diagnosis; however, nearly forty-percent of patients with EGPA present with asthma, pulmonary infiltrates and eosinophilia prior to the development of vasculitis [1-3]. The American College of Rheumatology (ACR) implemented a diagnostic criterion that encompasses a clinical approach, which allows for the diagnosis of EGPA to be made, even when there is no pathologic evidence of vasculitis. This allows for early recognition of disease and prompt initiation of treatment, which may lead to improved outcomes. We introduce a patient who was misdiagnosed with bacterial pneumonia on three separate occasions, but was later found to have EGPA despite lacking pathologic evidence of vasculitis.

Keywords: Churg-Strauss; Eosinophilic Granulomatosis with Polyangiitis; Eosinophilia; Vasculitis; Asthma

References

1. Churg Andrew. “Recent Advances in the Diagnosis of Churg-Strauss Syndrome”. Modern Pathology 14.12-28 (2001): 1284-1293.
2. King Talmadge. “Clinical Features and Diagnosis of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)” (2020).
3. King Talmadge E. “Epidemiology, Pathogenesis, and Pathology of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) (2020).
4. Nguyen Yann and Loïc Guillevin. “Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)”. Seminars in Respiratory and Critical Care Medicine 39.04 (2018): 471-481.
5. Noth Strek and Alan Leff. “Churg-strauss syndrome”. The Lancet 361 (2019): 587-594.

Citation

Citation: Kathleen McCabe and Imran Naqvi. “A Curious Case of Churg-Strauss”. Acta Scientific Clinical Case Reports 1.9 (2020): 19-21.

Copyright

Copyright: © 2020 Kathleen McCabe and Imran Naqvi. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.