Pulmonary manifestations of rheumatologic diseases

Abstract

Purpose of review The present review intends to provide an overview of the diversity and complexity of pulmonary manifestations of rheumatologic diseases and gaps in knowledge to effectively manage them. Recent findings Diffuse lung disease in children with rheumatologic diseases represents a heterogeneous group of autoimmune disorders. Despite their significant morbidity and mortality, we have limited understanding about their pathogenesis. Here, we provide an overview of the pathophysiology and current management approach of these disorders, highlighting tools which assist with diagnosis, risk stratification and therapy. In this context, we address the need to develop a standardized approach to diagnose at-risk patients with rheumatologic disease and to predict their progression and the need to develop robust studies which evaluate the factors and interventions that influence pulmonary disease outcome. Summary Diffuse lung disease in children with rheumatologic diseases represents a heterogeneous group of severe autoimmune disorders. By adopting a collaborative research approach among multicenters to help diagnose, risk stratify, and understand disease progression, effective management decisions can be optimized to improve clinical outcome.

Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?

“…Macrophages act as antigen presenting cells, ingesting the inhaled antigenic stimuli and causing an inappropriate activation of autoimmune CD4 T-lymphocytes and B-lymphocytes. Several distinct immune cell populations contribute to ILD [6,7].…”

“…In addition to these immunological and fibrotic processes, genetic factors and microbiological triggers are also believed to contribute to the pathogenesis of lung disease in CTD, particularly ILD. These changes essentially impair lung function and potentially can result in abnormal gas exchange caused by the restructuring of the interstitial spaces of the lungs [7].…”

“…A few specific autoantibodies (e.g. anti-RNP, anti-Jo-1) are associated with pulmonary disease in CTD, but their titres may not systematically correlate with disease activity [7].…”

Histology of Pulmonary and Bronchiolar Disorders in Connective Tissue Diseases

“…Pulmonary disease is associated with anti-U1 ribonucleoprotein seropositivity. [52][53][54][55] In patients with juvenile DM a small vessel vasculitis is the commonest manifestation but OP, UIP, and ARDS are described. The development of pulmonary complications appears more common in those patients with anti-Jo-1antibody, and rapid disease progression is associated with the presence of melanoma differentiation associated protein 5 (MDA-5) antibody.…”

“…In contrast to adults, children with SS and juvenile ankylosing spondylitis rarely develop lung disease. [51][52][53][54][55] As in adults the pulmonary manifestations of systemic rheumatologic disease may be hard to differentiate from the pulmonary effects of drugs, immunosuppression, and infection, which must be considered in the differential diagnosis.…”