Interstitial lung disease in systemic autoimmune rheumatic diseases: a comprehensive review

Abstract

Interstitial lung diseases (ILDs) are among the most serious complications associated with systemic rheumatic diseases, and lead to significant morbidity and mortality; they may also be the first manifestation of connective tissue diseases (CTDs). The aim of this narrative review is to summarise the data concerning the pathogenesis of CTD/ILD and its distinguishing features in different rheumatic diseseas. Areas covered: The pathogenesis, clinical aspects and treatment of ILD associated with rheumatic systemic diseases and CTDs were reviewed by searching the PubMed, Medline, and Cochrane Library databases for papers published between 1995 and February 2017 using combinations of words or terms. Articles not written in English were excluded. Expert commentary: The management of CTD-ILD is challenging because of the lack of robust data regarding the treatments used, the heterogeneity of the diseases themselves, and the scarcity of well-defined outcome measures. Treatment decisions are often made clinically on the basis of functional, radiographic progression, and exacerbating factors such as age and the burden of comorbidities. Given the complexities of diagnosis and the paucity of treatment trials, the management of CTD patients with ILD requires multidisciplinary collaboration between rheumatologists and pulmonologists in CTD-ILD clinics.

Rituximab in the Treatment of Interstitial Lung Disease Associated with Autoimmune Diseases: Experience from a Single Referral Center and Literature Review

“…Nowadays, there is a growing awareness of the significant morbidity and mortality that the presence of an AD-ILD entails [ 5 , 6 , 7 ]. In this sense, several reports have been published on the diagnostic and therapeutic management of this group of diseases [ 12 , 14 , 30 ].…”

“…The prevalence and mortality of patients with AD-associated ILD (AD-ILD) varies depending on the specific AD and the ILD pattern. In this regard, systemic sclerosis (SSc) and idiopathic inflammatory myositis (IIM), especially anti-synthetase syndrome, are more frequently associated with ILD development, followed by rheumatoid arthritis (RA) [ 5 , 6 , 7 ]. While non-specific interstitial pneumonia (NSIP) is the histological and radiological ILD pattern more commonly associated to the majority of ADs, RA is usually related to a usual interstitial pneumonia (UIP) pattern, which implies a poorer prognosis [ 7 , 8 , 9 , 10 ].…”

Pulmonary Nodular Lymphoid Hyperplasia with Mass-Formation: Clinicopathologic Characteristics of Nine Cases and Review of the Literature

“…Interstitial lung disease (ILD) may be the first presentation of connective tissue diseases (CTDs); up to 15% of patients initially diagnosed with idiopathic nonspecific interstitial pneumonia (NSIP) have an underlying systemic autoimmune rheumatic disease upon further evaluation [ 14 , 15 ]. The most common histological pattern in patients with CTD is NSIP [ 14 ]. However, no evidence of ILD except PNLH was found in the systemic lupus erythematosus patient included in this study.…”

Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome

“…Clinically significant ILD develops in 8 to 27% of subjects with pSS. 7,8,45,50,164,[198][199][200][201][202][203] Among patients with pSS, 10% had developed ILD by 1 year postdiagnosis and 20% by 5 years. 45 Compared with pSS patients without lung disease, pSS with lung involvement have a higher mortality rate 45,200 and poorer quality of life.…”

“…298 The role of CS, IA, DMARD, or biologics for ocular symptoms has been disappointing. 199,299 However, anecdotal responses to these agents have been noted in patients with systemic or serious complications of SS; these data are gleaned from retrospective studies and small case series. 121 In CTD-associated ILD, data regarding CS or other IA are limited.…”