Diagnosis and Management of Interstitial Lung Disease in Patients with Connective Tissue Diseases

Abstract

Interstitial lung disease (ILD) associated with connective tissue diseases (CTDs) is highly heterogeneous in its clinical presentation and course. The diagnosis and management of CTD-ILD require a multidisciplinary approach involving, at minimum, a rheumatologist, a pulmonologist, and a radiologist. Close monitoring of patients with CTD-ILD is important to enable early detection of disease progression and inform decisions regarding the initiation or escalation of pharmacotherapy. In the absence of guidelines regarding how CTD-ILDs should be treated, clinicians face difficult decisions on when to use immunosuppressant and anti-fibrotic therapies. The importance of a multidisciplinary and individualized approach to the diagnosis and management of CTD-ILD is highlighted in the three case studies that we describe in this article.

Figure 1

High-resolution computed tomography of the chest (Case 1). (a) Confluent ground glass opacity and consolidation, mainly in the mid and lower lungs, associated with moderate traction bronchiectasis. The distribution is partially subpleural with extension along the bronchial vascular bundles. Lung volumes are markedly reduced. (b) Coronal view demonstrating peri-diaphragmatic consolidation with an abrupt transition to normal parenchyma.

Figure 2

Surgical biopsy of the right lung (Case 1) demonstrates features of mixed cellular-fibrotic non-specific interstitial pneumonia (a) with scattered foci of organizing pneumonia (arrow) (b).

Figure 3

High-resolution computed tomography of the chest (Case 2). The scan 10 months prior to our evaluation (a) demonstrated a typical appearance of non-specific interstitial pneumonia (NSIP) with increased reticulation, traction bronchiectasis, lobar volume loss, and ground glass opacification in the lower lung zones. Bibasilar ground glass opacities that spare the subpleural region are characteristic for NSIP. The scan taken at our center 10 months later (b) demonstrated significant progression in fibrotic abnormalities.

Figure 4

High-resolution computed tomography of the chest (Case 3). The scan prior to our evaluation (a) demonstrated pulmonary nodules, thin-walled cysts, basilar predominant ground glass attenuation, and subpleural consolidation consistent with lymphocytic interstitial pneumonia (LIP) in the setting of Sjögren's syndrome. A repeat scan (b) demonstrated increased ground glass attenuation and stable thick-walled cysts, and a few of the bilateral solid pulmonary nodules had slightly increased in size.

Figure 5

Computed tomography- (CT-) guided lung biopsy (Case 3). Hematoxylin and eosin stain (a) demonstrates amorphic eosinophilic material reminiscent of an amyloid process, whereas both kappa staining (b) and lambda staining (c) demonstrate mixed light chain deposition.