Churg-Strauss syndrome: a new endotype of severe asthma? Results of 14 Turkish patients.
BACKGROUND
Churg-Strauss 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 Turkish 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 Turkish 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 American College of Rheumatology 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.


CONCLUSIONS
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%.
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
İnsu
İ
Yılmaz
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Gülfem
G
Çelik
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Ömür
Ö
Aydın
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Seçil Kepil
SK
Özdemir
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Şadan
Ş
Soyyiğit
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Zeynep
Z
Sözener
Department of Internal Medicine, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Selcan
S
Özgüçlü
Department of Radiology, Ankara University School of Medicine, Ankara, Turkey.
Çetin
Ç
Atasoy
Department of Internal Medicine, Division of Rheumatology, Ankara University School of Medicine, Ankara, Turkey.
Nurşen
N
Düzgün
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Dilşad
D
Mungan
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Betül
B
Sin
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Yavuz Selim
YS
Demirel
Department of Chest Diseases, Division of Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
Zeynep
Z
Mısırlıgil
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001249:Asthma / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D015331:Cohort Studies; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014421:Turkey
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents
Churg-Strauss syndrome; asthma; eosinophilia; eosinophilic granulomatosis with polyangiitis