Bilateral exophthalmos requiring rheumatologic consultation. A case of the Churg-Strauss syndrome
Morgiel, E.; Krecipro-Nizińska, E.; Samotij, D.; Wojtala, R.; Wiland, P.
Annales Academiae Medicae Stetinensis 58(2): 31-35
2012
ISSN/ISBN: 1427-440X PMID: 23767179 Document Number: 662931
The Churg-Strauss syndrome is a necrotic eosinophilic vasculitis affecting small and medium vessels. The etiology of this disease known to have an autoimmune background remains unclear. The Churg-Strauss syndrome is progressive and may involve multiple organs. Clinical manifestations are variegate, impeding the diagnosis. Typical symptoms include asthma, eosinophilia, mono- and polyneuropathy, sinusitis, pulmonary lesions and heart involvement. We present a case of a patient with bronchial asthma who developed chronic sinusitis of the ethmoid, maxillary, and paranasal sinuses at the age of 37 years and was operated four times for this reason. At this age the patient also developed bilateral exophthalmos; normal thyroid function stood against the diagnosis of thyroid orbitopathy. The patient was treated by an ophthalmologist with glucocorticosteroids for three years. Glucocorticosteroids were withdrawn at the age of 40 years because of chronic pancreatitis. The patient suffered myocardial infarction at the age of 41 years and was diagnosed with the Churg-Strauss syndrome at the age of 44 years. The Churg-Strauss syndrome is a rare systemic disease with a variable course and variegate clinical manifestations which may be the reason for diagnostic difficulties.