INTERSTITIAL PULMONARY FIBROSIS ASSOCIATED CONNECTIVE TISSUE DISEASES – CLINICAL AND IMAGING ASPECTS

Madalina Gheorghe,Ruxandra Ionescu,Violeta Claudia Bojinca

doi:10.37897/rjr.2017.2.5

Abstract

The lung is a common site of complications in systemic connective tissue diseases (CTD), and its involvement can present in several ways. Although it is generally thought that interstitial lung disease develops later on in CTD, it is often the initial presentation (“lung dominant” CTD). Interstitial lung disease (ILD) can be present in most types of CTD, including rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD. Interstitial lung disease must be detected early in the course of collagen disorders by performing high-resolution computed tomography and pulmonary function tests. The pattern described on HRCT is predictive for treatment response and disease progression. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. The management of patients with CTD-associated ILD is optimized by multidisciplinary collaboration.

Highlights

The connective tissue diseases (CTDs) are a heterogeneous group of systemic disorders typically characterized by circulating autoantibodies and varying degrees of autoimmune mediated organ damage
Several histological subtypes are described in patients with rheumatic diseases, patterns identified in idiopathic interstitial pulmonary fibrosis, the most common ones being: usual interstitial pneumonia (UIP); nonspecific interstitial pneumonia (NSIP); organizing pneumonia (OP) and lymphocytic interstitial pneumonia (LIP) [4]
high-resolution computed tomography (HRCT) assessment is complex and involves the description of the typical modifications of the Interstitial lung disease (ILD) and the predominant histological type (UIP, NSPI, OP, LIP), the investigation should be performed by an experienced radiologist

Summary

INTRODUCTION

The connective tissue diseases (CTDs) are a heterogeneous group of systemic disorders typically characterized by circulating autoantibodies and varying degrees of autoimmune mediated organ damage. A mutidisciplinary approach between pneumologist and rheumatologist identified that approximately 50% of patients classified as idiopathic interstitial pulmonary fibrosis (IIP) met criteria for ILD associated CTD [2]. Several histological subtypes are described in patients with rheumatic diseases, patterns identified in idiopathic interstitial pulmonary fibrosis, the most common ones being: usual interstitial pneumonia (UIP); nonspecific interstitial pneumonia (NSIP); organizing pneumonia (OP) and lymphocytic interstitial pneumonia (LIP) [4]. HRCT assessment is complex and involves the description of the typical modifications of the ILD (ground glass, microreticulonodular infiltrates, distortion of the lung architecture and traction bronchiectasis, cystic pattern, called honeycombing) and the predominant histological type (UIP, NSPI, OP, LIP), the investigation should be performed by an experienced radiologist. In the following we will present the association of interstitial lung fibrosis in various connective tissue diseases with some example of clinical cases

Rheumatoid arthritis and interstitial pulmonary fibrosis

Findings

Systemic sclerosis and interstitial pulmonary fibrosis