Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists.
OBJECTIVE
To determine the association of antineutrophil cytoplasmic antibodies (ANCA) and leukotriene receptor antagonists with disease activity in a large series of patients with Churg-Strauss syndrome.


METHODS
Potential subjects were identified by a computerized search of the Mayo Clinic Rochester database for the years 1990 to 2000. Patients meeting one of three classification schemes for Churg-Strauss syndrome were included.


RESULTS
Ninety-one patients met the inclusion criteria. Clinical manifestations were similar to those in previous reports. Mortality was similar to that in the general population. ANCA testing was performed in 74 patients. Seventy-three percent (n = 22) of the 30 patients tested before therapy were ANCA positive, as were 75% (n = 12) of the 16 patients tested during a disease flare. In comparison, 16% (n = 8) of the 49 tested during remission were ANCA positive. Serial measurements indicated a correlation of ANCA levels with disease activity. Central nervous system involvement was the only clinical manifestation that correlated with ANCA status (P = 0.05). Twenty-three patients received leukotriene receptor antagonists, of whom 16 (70%) began treatment before diagnosis and 6 (27%) began during remission. Two of those treated after diagnosis relapsed. In 1 patient the relation between disease and leukotriene receptor antagonist use could not be determined. Use of leukotriene receptor antagonists did not affect the time between onset of asthma and manifestations of vasculitis, and was not correlated with organ manifestations, except sinus disease.


CONCLUSIONS
No one classification scheme identified all patients. Churg-Strauss syndrome has a better prognosis than other ANCA-associated vasculitides. ANCA status correlates with disease activity, whereas a pathogenic role for leukotriene receptor antagonists in the development of Churg-Strauss syndrome was not noted.
Keogh
Karina A
KA
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Specks
Ulrich
U
D000085:Acetates / Q000627:therapeutic use; D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism; D001249:Asthma / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D001706:Biopsy; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000378:metabolism; D002648:Child; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D003521:Cyclopropanes; D004576:Electromyography; D004803:Eosinophilic Granuloma / Q000188:drug therapy / Q000401:mortality / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007211:Indoles; D020024:Leukotriene Antagonists / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D008910:Minnesota; D009195:Peroxidase / Q000378:metabolism; D048448:Phenylcarbamates; D011237:Predictive Value of Tests; D011804:Quinolines / Q000627:therapeutic use; D013223:Statistics as Topic; D013440:Sulfides; D013449:Sulfonamides; D016019:Survival Analysis; D014105:Tosyl Compounds / Q000627:therapeutic use; D016896:Treatment Outcome
D000085:Acetates; D019268:Antibodies, Antineutrophil Cytoplasmic; D003521:Cyclopropanes; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D007211:Indoles; D020024:Leukotriene Antagonists; D048448:Phenylcarbamates; D011804:Quinolines; D013440:Sulfides; D013449:Sulfonamides; D014105:Tosyl Compounds; D009195:Peroxidase; C093875:montelukast; C062735:zafirlukast