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Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg Strauss syndrome, is a rare small vessel vasculitis characterized by asthma, eosinophilia, and systemic involvement. This case report presents a 51-year-old man with a history of asthma, who presented with neurological symptoms including blurring of vision, bilateral ptosis, and altered sensorium. The patient had a previous history of cervical and lumbar spine surgeries for symptoms attributed to spondylosis. Further examination revealed hyperpigmented lesions on the limbs, wasting of small muscles, and foot drop.
Investigations showed eosinophilia and thrombocytosis on blood analysis, with bone marrow aspiration revealing eosinophilic and megakaryocytic hyperplasia. The cerebrospinal fluid analysis indicated eosinophilia and lymphocytic predominance. Imaging studies revealed hypodense areas in the midbrain and pons, as well as age-related cerebral atrophy.
Based on the diagnostic criteria for EGPA, the patient fulfilled the requirements of asthma, blood eosinophilia, peripheral neuropathy, transient pulmonary infiltrates, paranasal sinus abnormalities, and the presence of extravascular eosinophils on biopsy. The diagnosis was further supported by the presence of positive antineutrophilic cytoplasmic autoantibodies (ANCA-MPO). Treatment was initiated with intravenous corticosteroids and azathioprine due to the severity of the disease.
EGPA is a heterogeneous disease with varying clinical presentations and organ involvement. Early and accurate diagnosis is crucial for appropriate management and improved outcomes. Glucocorticoids, such as prednisolone, are the mainstay of treatment. While the overall mortality rate is relatively low compared to other systemic vasculitides, cardiac involvement can contribute to significant morbidity and mortality.
In conclusion, this case report highlights the importance of considering EGPA in patients with asthma, eosinophilia, and multi-system involvement. Prompt recognition and initiation of appropriate therapy can help mitigate complications and improve patient outcomes. Further research is needed to better understand the pathogenesis and optimal management strategies for this rare vasculitic disorder.
JSS Academy of Higher Education & Research
Conflict of Interest
Churg Strauss, Hypereosinophilic syndromes, Vasculitis, ANCA-MPO
Mukherjee R, M B.
Eosinophilic granulomatosis with polyangiitis (Churg Strauss Syndrome) – a case report.
Digital Journal of Clinical Medicine.
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