Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

Abstract

Interstitial pneumonia (IP) refers to involvement of the lung parenchyma by varying degrees of inflammation and fibrosis, in contrast to airspace disease typically seen in bacterial pneumonia. IP lies in the center of a heterogenous group of diffuse interstitial lung diseases (ILDs), either idiopathic or linked to underlying disorders. One of the major categories of disorders frequently associated with IP is a connective tissue disease (CTD), in which autoimmunemediated tissue injury leads to multiple organ impairment. Today, IP represents the most critical pulmonary complication in CTD, resulting in significant morbidity and mortality.Despite growing understanding of the pathology of IPs, as well as the accumulating knowledge from both basic and clinical studies of CTDs, the pathogenesis of CTD-associated IP remains unclear. This chapter will provide an overview of the general understanding of ILD and illustrate the current state of knowledge on IP associated with CTD, in order to fully comprehend the entirety of its complex pictures. Moreover, we will propose a new insight into the immune pathogenesis of CTD-IP by presenting evidence which robustly indicates that T cells trigger initial development of IP in polymyositis/dermatomyositis, suggesting potential approaches for controlling such particular T cells in therapeutic interventions for IP.

Clinical Profile of Connective Tissue Disease- related Interstitial Lung Diseases at a Tertiary Care Center in Western India

“…16,17 Cough and dyspnea on exertion were the cardinal symptoms in 46 patients accounting for 90% of cases, which is in concordance with various studies on ILD. [18][19][20] The most common clinical signs that were seen in ILDs were clubbing, post-exercise desaturation, and bilateral Velcro crackles. In our study, clubbing was observed in 73% of cases, and post-exercise desaturation and crackles were seen in all patients.…”

“…It is similar to various studies, where UIP is the most common pattern associated with RA-ILD and NSIP is the most common to be associated with scleroderma-ILD. 18,27 Among 10 patients of MCTD, 6 (60%) patients had NSIP, 3 (30%) patients had UIP, and 1 (10%) patient had LIP pattern. Nonspecific interstitial pneumonia pattern was seen in 1 DM patient and 2 patients of SLE.…”