R. Behrens et al., CHURG-STRAUSS-SYNDROME WITH EOSINOPHIL CA TIONIC PROTEIN IN A 39-YEAROLD WOMAN, Deutsche Medizinische Wochenschrift, 123(1-2), 1998, pp. 6-11
History and clinical findings: A 39-year-old woman in good health sudd
enly developed painful paraesthesia and incomplete paresis of all four
limbs. Furthermore, arterial hypertension and tachycardia were found.
The patient reported a history of chronic allergic rhinitis for 10 ye
ars and asthma one year. Investigations: Laboratory data showed an inc
reased leucocyte count with a substantial increase in eosinophils in p
eripheral blood as well as in bone marrow. Nonspecific inflammatory ma
rkers such as erythrocyte sedimentation rate, C-reactive protein, fibr
inogen as well as eosinophil cationic protein (ECP) were also increase
d. Histological investigations of a skin-muscle biopsy of the quadrice
ps revealed necrotizing vasculitis with extravascular granulomata and
histocytic giant cells, Diagnosis, treatment and course: Based on the
diagnosis of Churg-Strauss syndrome treatment with methylprednisolone
(500 mg every second day) was started, but was found to be ineffective
after 10 days. Symptoms responded well to a subsequent course of 150
mg cyclophosphamide combined with 50 mg prednisolone per day. Pareses
and pain were significantly reduced and all qualitative nerve function
s returned to normal within two weeks of treatment. Laboratory paramet
ers, especially the ECP, were similarly normalized. Conclusion: Churg-
Strauss syndrome should be considered in the differential diagnosis of
unexplained polyneuropathy. Determination of ECP may not only help in
the diagnosis but does also facilitate monitoring of treatment and of
further course of the disease.