Churg-Strauss syndrome: a new endotype of severe asthma? Results of 14 Turkish patients

YILMAZ İ., ÇELİK G. E., AYDIN Ö., Ozdemir S. K., SOYYİĞİT Ş., Sozener Z., ...Daha Fazla

CLINICAL RESPIRATORY JOURNAL, sa.3, ss.350-358, 2015 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Basım Tarihi: 2015
  • Doi Numarası: 10.1111/crj.12154
  • Dergi Adı: CLINICAL RESPIRATORY JOURNAL
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.350-358
  • Anahtar Kelimeler: asthma, Churg-Strauss syndrome, eosinophilia, eosinophilic granulomatosis with polyangiitis, TERM-FOLLOW-UP, SYSTEMIC VASCULITIS, MICROSCOPIC POLYANGIITIS, ALLERGIC GRANULOMATOSIS, CONSENSUS CONFERENCE, ANGIITIS, FEATURES, DISEASE, CLASSIFICATION, EPIDEMIOLOGY
  • Ankara Üniversitesi Adresli: Evet

Özet

IntroductionChurg-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. ObjectiveThe 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. MethodsThe 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. ResultsAll 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. ConclusionWe 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%.