Open AccessC hurg– S trauss syndrome: a new endotype of severe asthma? Results of 14 T urkish patients
Author(s) -
Yılmaz İnsu,
Çelik Gülfem,
Aydın Ömür,
Özdemir Seçil Kepil,
Soyyiğit Şadan,
Sözener Zeynep,
Özgüçlü Selcan,
Atasoy Çetin,
Düzgün Nurşen,
Mungan Dilşad,
Sin Betül,
Demirel Yavuz Selim,
Mısırlıgil Zeynep
Publication year - 2015
Publication title -
the clinical respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.789
H-Index - 33
eISSN - 1752-699X
pISSN - 1752-6981
DOI - 10.1111/crj.12154
Subject(s) - medicine , etiology , asthma , azathioprine , cyclophosphamide , eosinophilia , endotype , prednisolone , dermatology , aspirin , gastroenterology , disease , chemotherapy
C hurg– S trauss syndrome ( CSS ) is a rare multisystem vasculitis. Considering the variation of autoimmune diseases in different races, it is of interest to determine whether any outstanding features exist for T urkish patients with CSS . Objective The aim of this study was to evaluate the clinical and serological features of the disease, the treatment, and long‐term follow‐up details, and to investigate possible etiological factors of T urkish CSS patients. Methods The study included 14 patients who were diagnosed with CSS , and followed by our department between 2004 and 2012. Possible etiological factors, initial symptoms, clinical presentations, treatment, as well as outcomes were documented. The study was approved by the local ethics. Results All patients fulfilled the A merican C ollege of R heumatology criteria. Initial symptoms were worsening asthma ( n = 14; 100%) and skin lesions ( n = 6; 43%). All patients had a diagnosis of asthma and nasal polyps, whereas 57.1% had aspirin hypersensitivity at the time of diagnosis. The lungs (100%) and skin (43%) were most commonly involved. Peripheral eosinophilia dominated on initial presentations of all patients. Initial treatments included oral methyl prednisolone in all cases, whereas cyclophosphamide and azathioprine were used in three cases. Relapses were detected in five cases. None of the cases were able to stop the oral corticosteroid treatment. No fatalities were observed. Conclusion We herein describe a new severe asthma endotype in connection with CSS . We suggest that physicians who deal with uncontrolled severe asthma cases should consider CSS in the presence of nasal polyps, aspirin hypersensitivity, and especially peripheral blood eosinophilia over 10%.