Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review
| Autor: | Yener, Gülçin Otar, Tekin, Z.E., Demirkan, N.Ç., Yüksel, S. |
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| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
Lung Diseases
Male electromyography lung disease Biopsy thrombocytopenia rash Review Churg-Strauss Syndrome sensorimotor neuropathy differential diagnosis neurologic examination pain nuclear magnetic resonance imaging pathophysiology azathioprine necrotizing arteritis predictive value Remission Induction immunosuppressive treatment neurologic disease Magnetic Resonance Imaging eosinophilic granulomatosis with polyangiitis Treatment Outcome priority journal histopathology Drug Therapy Combination eosinophilia Immunosuppressive Agents Vasculitis combination drug therapy corticosteroid pediatrics Adolescent diagnostic imaging rare disease complication lung infiltrate respiratory tract disease Diagnosis Differential Polyneuropathies remission Predictive Value of Tests Polyneuropathy panniculitis x-ray computed tomography case report Humans human hypesthesia limb pain skin biopsy Glucocorticoids creatine kinase immunosuppressive agent asthma Churg Strauss syndrome Exanthema clinical feature cyclophosphamide glucocorticoid prognosis Tomography X-Ray Computed |
| Popis: | Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg–Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain. He had eosinophilia, and electroneuromyography demonstrated sensorimotor polyneuropathy. His skin biopsy revealed necrotizing eosinophilic vasculitis and eosinophilic panniculitis. Although he had no respiratory symptoms or history of asthma, prominent pulmonary involvement was evident on thoracic MRI. After treatment, his complaints of pain improved but mild neuropathy persisted. After 4 years of follow-up, he had minimal hypoesthesia in his right hand but had not experienced any relapses. This case highlights the fact that in cases suspected of EGPA, even without respiratory symptoms or asthma, detailed imaging should be performed for a definitive diagnosis. In addition, mild neurological findings may persist despite treatment in EGPA. The relevant literature on EGPA, with specific reference to pediatric cases, is reviewed. © 2017, Springer-Verlag GmbH Germany, part of Springer Nature. |
| Databáze: | OpenAIRE |
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