Clinical Characteristics of Interstitial pneumonia with autoimmune features: A Single-Center Experience

Öz

Objective: Interstitial pneumonia with autoimmune features (IPAF) is a recently determined condition characterized by clinical or serological symptoms indicative of an underlying autoimmune process but without fulfilling connective tissue disease classification criteria. This study aims to identify the clinical, serological, and morphological features of AFIP patients. Materials and Methods: This is a retrospective cohort study. A total of 38 patients diagnosed with AFIP in the Rheumatology outpatient clinic of XXX University between 2015 and 2022 were included in the study. Results: The patients' mean age was 58.26 ±12.31. The majority of patients (n=27, 71% female) were female. The most frequently described clinical features among patients were inflammatory arthritis or polyarticular morning joint stiffness lasting more than 60 minutes (n=9, 23.6%) and Raynaud's phenomenon (n=9, 23.6%). The most commonly identified serological findings were antinuclear antibody (ANA) (n=27, 71.1%), Anti-Ro/SSA antibody (n=6, 15.8%), rheumatoid factor (n=5, 13.2%), and Anti-cyclic citrullinated peptide (Anti-CCP) (n=5, 13.2%) positivity. The mean follow-up was 43.21 ± 23.30 months, and only one out of 10 patients who died during the follow-up was associated with IPAF. Thirty-one (81.5%) patients were treated with immunosuppressants, and 29 (76.3%) were given steroid treatment. The most commonly used immunosuppressive drug with steroids was cyclophosphamide (n=9, 23.6%). Anti-fibrotic therapy was administered to 4 patients (10.5%) who did not respond adequately to immunosuppressive drugs. Morphologically, the most frequent radiological pattern was the non-specific interstitial pneumonia (NSIP) pattern (n=12, 26.3%). Conclusion: This single-center study demonstrated that IPAF primarily affects females, often presents with joint manifestations and Raynaud's phenomenon, and more frequently exhibits the NSIP pattern on imaging. Due to the heterogeneity of IPAF, defining its clinical and morphological characteristics is essential for early diagnosis and treatment.

Anahtar Kelimeler

• Rheumatic disease
• interstitial lung disease
• interstitial pneumonia with autoimmune features
• idiopathic pulmonary fibrosis

Otoimmün Özellikler Gösteren İnterstisyel Pnömoninin Klinik Özellikleri: Tek Merkez Deneyimi

Öz

Giriş ve Amaç: Otoimmün özellikli interstisyel pnömoni (OÖİP), altta yatan otoimmün bir süreci düşündüren klinik veya serolojik özelliklerin mevcut olduğu; ancak bağ doku hastalıkları için tanımlanmış sınıflandırma kriterlerinin karşılanmadığı, yakın zamanda tanımlanmış bir antitedir. Bu çalışmanın amacı, OÖİP hastalarının klinik, serolojik ve morfolojik özelliklerini belirlemektir. Gereç ve Yöntemler: Bu çalışma bir retrospektif kohort çalışmasıdır. Çalışmaya 2015-2022 tarihleri arasında XXX üniversitesi Romatoloji bölümünde OÖİP tanısı ile takip edilen 38 hasta dahil edilmiştir. Bulgular: Çalışmadaki hastaların yaş ortalamaları 58,26 ± 12,31 idi. Çoğunluğu (n=27, %71) kadın hastalar oluşturmaktaydı. Hastalarda en sık tanımlanan klinik özellikler inflamatuar artrit veya 60 dakikadan uzun süren poliartiküler sabah sertliği (n=9, %23,6) ve Raynaud fenomeniydi (n=9, %23,6). En sık tanımlanan serolojik bulgular antinükleer antikor (ANA) pozitifliği (n=27, %71,1), Anti-Ro/SSA antikor pozitifliği (n=6, %15,8), romatoid faktör (RF) (n=5, %13,2) ve Anti-Siklik sitrüline peptid pozitifliği (Anti-CCP) (n=5, %13,2) idi. Ortalama takip süresi 43,21 ± 23,30 ay olup, takip süresince ex olan 10 hastadan yalnızca biri OÖIP ile ilişkilendirilmiştir. Hastaların 31’i (%81,5) immünsüpresiflerle tedavi edilmiş olup, 29’una (%76,3) steroid tedavisi verilmiştir. Steroidlerle birlikte en çok kullanılan immünsüpresif ilaç siklofosfamid (n=9, %23,6) idi. İmmünsupresif ilaçlara yeterli yanıt alınamayan 4 hastaya (%10,5) ise anti-fibrotik verilmiştir. Morfolojik alanda radyolojik paternler içerisinde en sık nonspesifik interstisyel pnömoni (NSİP) paterni gözlenmiştir (n= 15, %39,5). Sonuç: Tek merkez deneyimini sunan bu çalışmada OÖİP’nin çoğunlukla kadın hastalardan oluştuğu, sıklıkla eklem bulguları ve Raynaud ile prezente olduğu ve görüntülemede en sık NSİP paterni özelliklerini gösterdikleri belirlenmiştir. Oldukça heterojen özellikler gösteren OÖİP’nin klinik ve morfolojik özelliklerinin iyi belirlenmesi, hastalığın erken tanısı ve tedavisi için önemlidir.

Anahtar Kelimeler

• otoimmün özellikli interstisyel pnömoni
• Romatolojik hastalık
• interstisyel akciğer hastalığı
• idiopatik pulmoner fibrozis

Kaynakça

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