Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

Abstract

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.

Keywords: Asian Continental Ancestry Group; Connective Tissue Disease; Diagnosis; Disease Management; Guidelines as Topic; Lung; Lung Disease, Interstitial.

Figure 1. (A) Mechanic's hand, cracking and fissuring along the sides of the digits and palm. (B) Gottron's papules, red and scaly papules that erupt on the metacarpophalangeal joints. (C) Sclerodactyly, fixed fingers in a semi-flexed position with tightened and wax like skin. (D) Digital ulceration, an ulceration on the tip of index finger. (E) Telangiectasias, multiple dilated facial small vessels. (F) Heliotrope rash, violaceous erythema on the upper eyelids (The patient provided verbal consent for the picture).

Figure 2. (A) Radiologic pattern of non-specific interstitial pneumonia in a patient with systemic sclerosis. High resolution computed tomography (HRCT) image shows bilateral subpleural and basal predominant fine reticular pattern and ground-glass opacity. (B) Radiologic pattern of organizing pneumonia in a patient with dermatomyositis. HRCT image shows multiple peripheral patch consolidations. (C) Radiologic pattern of lymphocytic interstitial pneumonia in a patient with Sjögren's syndrome. HRCT image shows multifocal variable-sized, thin-walled, cystic lesions on both lungs.

Figure 3. Radiologic pattern of usual interstitial pneumonia in a patient with rheumatoid-arthritis. High resolution computed tomography image shows bilateral subpleural honeycombing and reticular opacity with traction bronchiectasis.

Figure 4. Histopathology of the lung in a patient with interstitial pneumonia with autoimmune features. (A) Usual interstitial with lymphoid follicles (×10). (B) Lymphoid bronchiolitis (×100). (C) lymphoplasmacytoid cell infiltrates (×200). Hematoxylin eosin saffron. Courtesy of Prof. Shim HS, Yonsei University.