Definition, evaluation and therapy of progressive fibrosing interstitial lung disease associated with rheumatic diseases - a consensus paper of the Romanian Society of Rheumatology and Romanian Society of Pneumology

Abstract

Interstitial lung diseases (ILD), of which the most well-known is idiopathic pulmonary fibrosis (IPF), are a heterogeneous group of diseases, with similar, inflammatory and/or fibrosing mechanisms, which lead to the rapid decline of lung function and implicitly to a high degree of morbidity and mortality. Besides IPF, other interstitial lung diseases, such as those associated with connective tissue diseases, the most common being rheumatoid arthritis and systemic sclerosis, can develop a progressive fibrosing phenotype. Thus, because they have similar pathogenic mechanisms and clinical manifestations, all these diseases are described under the term of progressive fibrosing interstitial lung disease (PF-ILD). It is recommended that the diagnosis, clinical and paraclinical evaluation of ILD be made through a standardized management, within a multidisciplinary team that must include a pulmonologist, radiologist and rheumatologist, evaluation after which the subsequent treatment will be decided. Early diagnosis leads to an effective therapeutic intervention and decreased mortality. The progressive character has been defined if the progression occurs despite the current optimal management and treatment, which includes glucocorticoids and immunosuppressive therapy, at which point the indication of the antifibrotic treatment appears. The complete evaluation of ILD involves a rigorous anamnesis and clinical examination to identify environmental and professional risk factors, the patient’s chronic medication, family and personal history, the onset of the disease, as well as the identification by auscultation of bilateral basal crackles. From the paraclinical examination, the most important is the imaging studies (standard chest X-rays and mandatory HR-CT) which provide information about the anatomy, pattern, evolution in time or clues related to the underlying disease. The progressive character has been defined if the progression occurs despite the current optimal management and treatment, which includes glucocorticoids and immunosuppressive therapy, at which point the indication of the antifibrotic treatment appears. Evaluation of lung function is very important to complete the patient picture with PF-ILD, and the gold standard is the combination of spirometry, body plethysmography with diffusing capacity for carbon monoxide (DLCO), gasometry and an exercise test. Using this information, the optimal treatment will be led by the same multidisciplinary team that established the diagnosis, taking into account many aspects related to the characteristics of the disease, the cause, the safety profile and it will be monitored according to the existing protocols.

Keywords

• interstitial lung diseases
• connective tissue diseases
• early diagnosis
• progressive character
• optimal treatment