Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study

Mediterranean Journal of Rheumatology

ARTICLE

Maria Karampeli¹  Konstantinos Thomas²  Sofia Flouda³  Aikaterina Chavatza³  Dionisios Nikolopoulos³  Antigone Pieta³  Dimitrios Tseronis³  Michail Aggelakos³  Dimitra Kassara³  Vasiliki Tzavara¹  Pelagia Katsimbri³  Dimitrios Boumpas³  Theofanis Karageorgas³ 

[1] Department of Internal Medicine, General Hospital Korgialenio-Benakio HRC;4th Department of Internal Medicine, “Attikon” University Hospital of Athens;Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, “Attikon” University Hospital of Athens

关键词: Interstitial lung diseases;    connective tissue diseases;    interstitial pneumonia with autoimmune features;    pulmonary fibrosis;   

DOI  :  10.31138/mjr.31.3.330

学科分类：社会科学、人文和艺术（综合）

来源: PCO Convin S.A.

【 摘 要 】

Objectives: Interstitial pneumonia with autoimmune features (IPAF) refers to patients with interstitial lung disease and autoimmune features not fulfilling the classification criteria for a specific connective tissue disease. We sought to study the characteristics, disease progression, response to treatment and complications of patients with IPAF in 1-year follow-up period. Methods: Clinical and laboratory findings, comorbidities, medications, pulmonary function tests (PFTs), chest HRCT and complications during the one-year follow-up period were documented for each of the 39 enrolled patients with IPAF. Results: The mean age at the time of IPAF diagnosis was 63.2 (±11) years, and 62% of patients were female. The most common clinical features were arthritis (82%) and rash (54%-not included in the IPAF criteria). Antinuclear antibodies (ANA) (59%) and non-specific interstitial pneumonia (NSIP-61.5%) were the most prevalent autoantibodies and radiological pattern respectively. PFTs at 12 months from baseline stabilized or improved in 79.5% of patients (p> 0.05). Infections were observed in 23.1% of patients during the first and in 12.8% during the second semester of follow-up. Two patients (5.1%) required hospitalization. All infections occurred in patients with non- usual interstitial pneumonia (UIP) pattern (p=0.02). Conclusions: Arthritis and rash are among the most common features in IPAF suggesting rash could be included into IPAF criteria. Almost 80% of patients had stable/improved PFTs at the end of follow-up. Infections occurred mainly in the first semester of treatment and in patients with non-UIP radiological pattern probably due to higher doses of corticosteroids used in these patients.

【 授权许可】

CC BY-NC