Ear, nose and throat manifestations of Churg-Strauss syndrome.
CONCLUSIONS
Ear, nose and throat (ENT) involvement is common in Churg-Strauss syndrome (CSS), usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps. Otolaryngologists may play a pivotal role in making an early diagnosis of this disease.


OBJECTIVE
CSS is a systemic vasculitic disorder that affects small to medium-sized blood vessels. Although the cause of CSS remains unknown, tissue damage seems more likely to be mediated by activated eosinophils. Patients affected by CSS frequently have ENT manifestations, which are often present at the time of disease onset and may represent relevant clues for the diagnosis. Thus, our objective was to present the ENT manifestations at the onset, at the diagnosis and at some point during the course of the disease in a series of patients with CSS collected at a single center.


METHODS
Twenty-eight patients with CSS, as defined according to the 1990 American College of Rheumatology classification criteria, were identified. Twenty-one (75%) of these patients had ENT involvement. We evaluated the clinical course, laboratory data, histologic findings, treatment and outcomes.


RESULTS
Of the 21 patients, 13 (61.9%) had ENT involvement at asthma onset and 8 (38%) at diagnosis or during follow-up. The most common ENT manifestations were allergic rhinitis in 9 (42.8%) patients and nasal polyposis in 16 (76.1%). Three (14.2%) patients developed chronic rhinosinusitis without polyps, three (14.2%) had nasal crusting, one (4.7%) serous otitis media, one (4.7%) purulent otitis media, two (9.5%) progressive sensorineural hearing loss, and one (4.7%) unilateral facial palsy. Corticosteroid therapy associated with immunosuppressive drugs usually yielded improvement or stabilization.
Bacciu
Andrea
A
Department of Otolaryngology, University of Parma, Italy. andreabacciu@yahoo.it
Bacciu
Salvatore
S
Mercante
Giuseppe
G
Ingegnoli
Francesca
F
Grasselli
Chiara
C
Vaglio
Augusto
A
Pasanisi
Enrico
E
Vincenti
Vincenzo
V
Garini
Giovanni
G
Ronda
Nicoletta
N
Ferri
Teore
T
Corradi
Domenico
D
Buzio
Carlo
C
D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000368:Aged; D001249:Asthma / Q000175:diagnosis / Q000188:drug therapy; D002908:Chronic Disease; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D009298:Nasal Polyps / Q000175:diagnosis / Q000188:drug therapy; D010038:Otorhinolaryngologic Diseases / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000008:administration & dosage; D012189:Retrospective Studies; D012221:Rhinitis, Allergic, Perennial / Q000175:diagnosis / Q000188:drug therapy; D012852:Sinusitis / Q000175:diagnosis / Q000188:drug therapy
D003520:Cyclophosphamide; D011241:Prednisone; D008775:Methylprednisolone