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Clinical and laboratory characteristics of 19 patients with C hurg– S trauss syndrome from a single S outh A ustralian centre
Author(s) -
Whyte A. F.,
Smith W. B.,
Sinkar S. N.,
Kette F. E.,
Hissaria P.
Publication year - 2013
Publication title -
internal medicine journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 1444-0903
DOI - 10.1111/imj.12173
Subject(s) - medicine , myalgia , thrombosis , vasculitis , odds ratio , retrospective cohort study , complication , anti neutrophil cytoplasmic antibody , gastroenterology , surgery , disease
Background C hurg– S trauss syndrome ( CSS ) is a rare, idiopathic systemic vasculitis. There is emerging evidence of an association between the presence or absence of antineutrophil cytoplasmic antibodies ( ANCA ) and clinical phenotype. Thromboembolism is an increasingly recognised complication of the disease. Aims Given the paucity of A ustralian data, the aim of this study was to examine the clinical and laboratory features of CSS in a single A ustralian centre. Methods We performed a retrospective review of all patients who fulfilled the A merican C ollege of R heumatology classification criteria for CSS managed at the D epartment of I mmunology, R oyal A delaide H ospital between 2002 and 2008. Results Nineteen patients were included. All patients had asthma and most had upper airway involvement. Peripheral nerve, musculoskeletal, gastrointestinal and cutaneous involvement was common. Renal and cardiac involvement was uncommon in this series. Histological confirmation was obtained in 15 patients (78.9%). Ten patients (52.6%) were ANCA +, and these were more likely to have musculoskeletal involvement, such as arthralgia or myalgia (odds ratio 57, P = 0.005). Thrombosis was a feature at diagnosis in six patients (31.6%); two of these recurred with relapse. Sixteen patients (84.2%) were followed up; five died, and mean survival was 8.9 years. Conclusions This is the first A ustralian study to focus on CSS . Our results demonstrate similar presentation and prognosis of CSS to previous descriptions; however, we noted that musculoskeletal involvement was more common in ANCA + patients. In our series, thrombosis was a significant complication and we suggest that thromboprophylaxis may be warranted.