Sistemik sklerozda interstisyel akciğer hastalığı ve pulmoner arteriyel hipertansiyon için risk faktörleri ve biyobelirteçler: Türkiye'de iki tersiyer merkezin deneyimi

Öz

Amaç: Sistemik skleroz (SSk) hastalarının klinik ve laboratuvar özelliklerini tanımlamak, önemli morbidite ve mortalite nedenleri olan interstisyel akciğer hastalığı (İAH) ve pulmoner arteriyel hipertansiyon (PAH) prevalansını etkileyen risk faktörlerini araştırmak. Hastalar ve yöntemler: Sistemik skleroz tanısı olan 88 hasta İAH ve PAH varlığına göre karşılaştırıldı. Akciğerin yüksek rezolüsyonlu bilgisayarlı tomografisi ile İAH doğrulandı ve istirahatte uygulanan ekokardiyografiye göre sağ ventriküler sistolik basınç >40 mmHg olduğunda olası PAH olarak değerlendirildi ve kaydedildi. Bulgular: 88 hastanın %44,3'ünde yaygın tipte ve %55,7'sinde sınırlı tipte SSk vardı. Diffüz tip, pozitif antiskleroderma-70 (anti-Scl70) antikoru ve anti-sentromer antikor yüzdeleri, beyaz kan hücresi, trombosit sayısı, eritrosit sedimantasyon hızı (ESH), sigara içme ve sklerodaktili ve telanjiektazi varlığı İAH olan grupta anlamlı farklı saptandı. Anti-Scl70 antikorunun pozitif titresi (odds oranı (OR)=6,124, p=0,004), trombosit sayısı (OR=0,138, p=0,002), ESH (OR=1,042, p=0,035) ve talenjiektazi varlığı (OR=10,571, p=0,001), SSk'li hastalarda İAH ile ilişkili bulundu. Ayrıca diffüz tip (OR=0,223, p=0,010), sklerodaktili (OR=11,112, p=0,028) ve talenjiektazi (OR=3,861, p=0,020) varlığı nonspesifik interstisyel pnömoni paterninde İAH gelişimi için risk faktörleri iken, anti Scl-70 antikor pozitifliği (OR=12,921, p=0,019) ve yüksek ESH'nın (OR=1,034, p=0,030) usual interstisyel pnömoni paterni gelişimi için risk faktörleri olduğu saptandı. PAH açısından değerlendirildiğinde ise tek risk faktörünün ileri yaş olduğu görüldü (OR=1,073, %95 GA:1,012-1,139, p=0,019). Sonuç: Anti Scl-70 antikorunun pozitif titresi, yaygın tip, talenjiektazi varlığı ve yüksek ESH, SSk hastalarında İAH'nın varlığı ile bağımsız olarak ilişkilendirildi.

Anahtar Kelimeler

• İnterstisyel akciğer hastalığı
• NSIP
• pulmoner arteriyel hipertansiyon
• sistemik skleroz
• UIP

Risk factors and biomarkers for ınterstitial lung disease and pulmonary arterial hypertension in systemic sclerosis: experience of two tertiary centers in Türkiye

Abstract

Purpose: To define the clinical and laboratory characteristics of patients with systemic sclerosis (SSc), and to investigate the risk factors affecting the prevalence of interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), which are important causes of morbidity and mortality. Patients and methods: 88 patients with SSc were compared according to the presence of ILD and PAH. ILD was confirmed by chest high-resolution computed tomography, and PAH was suspected and considered probable PAH when right ventricular systolic pressure was >40 mmHg according to echocardiography during rest. Results: Of the 88 patients, 44.3% had diffuse-type and 55.7% had limited-type SSc. Diffuse type, percentages of positive anti-scleroderma-70 (anti-Scl70) antibody and anti-centromere antibody, white blood cell (WBC), platelet, erythrocyte sedimentation rate (ESR), smoking, and presence of the sclerodactyly and telangiectasia differed significantly in SSc with ILD group. The positive titer of anti-Scl70 antibody (odds ratio (OR)=6.124, p=0.004), platelet count (OR=0.138, p=0.002), ESR (OR=1.042, p=0.035) and presence of telangiectasia (OR=10.571, p=0.001) were associated with ILD in patients with SSc. Also, while diffuse-type (OR=0.223, p=0.010), the presence of sclerodactyly (OR=11.112, p=0.028) and telangiectasia (OR=3.861, p=0.020) were risk factors for the development of ILD in nonspecific interstitial pneumonia pattern, anti-Scl-70 antibody positivity (OR=12.921, p=0.019) and high ESR (OR=1.034, p=0.030) were found to be risk factors for the development of usual interstitial pneumonia pattern. When evaluated in terms of PAH, the only risk factor was found to be advanced age (OR=1.073, 95% CI:1.012-1.139, p=0.019). Conclusion: Positive titer of the anti-Scl70 antibody, diffuse type, presence of telangiectasia, and high ESR were independently associated with ILD in SSc patients.

Keywords

• Interstitial lung disease
• NSIP
• pulmonary arterial hypertension
• systemic sclerosis
• UIP

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