Churg-Svauss Syndrome

Bailey 495 - 496

Churg-Sttauss syndrome ( CSS) is a rare small to medium
vessel neaotizing granulomatous vasculitis associated
with bronchial asthma and eosinophilia. It is thought to
be 1h2-mediated with activation by eosinophils. Several
rhinologic symptoms may appear early in the course of
the disease, creating an opportunity for the otolaryngologist
to make an early diagnosis. In fact. one series revealed
approximately one-third of patienta' reason for seeking care
was worsening nasal symptoms (39). Furthermore. head
and neck involvement was seen in approximately 75% of
patients who participated in one retrospective case series,
with allergic rhinitis (48%) and polyposis (53% to 76%)
being the most common presenting symptoms ( 41,42). It
is important for otolaryngologists to be familiar with these
symptoms, since some estimates suggest that they diagnose
up to 10% of all cases ofCSS (40).
Clinical presentation oftentimes starts with a prodromal
phase of asthma, allergic rhinitis, sinonasal polyposis,
and/ or rhinosinusitis. Initial presentation may also include
crusting and epistaxis, making distinction from WG difficult.
The next phases include eosinophilia and finally systemic
vasculitis and its resultant manifestations (Fig. 31.6).
Although involvement of many otgan systems has been
described, the sinonasal, respiratory, and peripheral nervous
systems are predominantly affected by CSS ( 41 ).
1he constellation of nasal polyps, asthma, constitutional
symptoms, and additional systemic complaints
should prompt evaluation with hematologic (CBC with
differential blood count to examine the proportion of
eosinophils) and metabolic serologies. It should be
noted that eosinophilia may be masked if systemic steroid
therapy is being utilized. Elevated ESR and CRP,
though nonspecific, will further support a systemic inflammatory
process. Immunoglobulin analysis may note hypergammaglobulinemia and elevation of IgE. Of note,
ANCA has not been shown to be a reliable assay for CSS
(40). The American College ofRheumatologyrequires four
of six criteria detailed in Table 31.5 for diagnosis of CSS.
Further biopsy findings include necrotizing vasculitis and
extravascular necrotizing granulomas.
Treatment of CSS generally consists of treatment and
subsequent maintenance therapy. Induction therapy
involves treatment with oral or intravenous steroids in
order to suppress the active disease process. Maintenance
therapy follows for approximately 1 year with low-dose
oral steroids and cyclophosphamide or methotrexate (41).
In a retrospective analysis that utilized a Lund-Mackay
grading scale.. seventeen out of 29 patients had endoscopic
evidence of polyposis with CSS. After initiation of medical
treatment in this series, none of these patients required
surgical intervention (42). We advise consultation with a
rheumatologist for long-term follow-up and multidisciplinary
coordination with otolaryngologists, medical pulmonologists,
cardiologists, and/or nephrologists.