Pulmonary manifestations of Sjögren's syndrome

Abstract

In 9-20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

FIGURE 1

a) Axial computed tomography (CT) showing mild thickening of bronchial walls (arrows) in a woman with Sjögren's syndrome. b) Chronic cough and recurrent pulmonary infections associated with bronchiolitis in a 60-year-old woman with primary Sjögren's syndrome. High-resolution CT showing multiple ill-defined centrilobular nodules (arrowheads) with sparing of the subpleural region. c, d) Follicular bronchiolitis in a 54-year-old woman with primary Sjögren's syndrome. c) High-resolution CT showing an area of heterogeneous attenuation in the left lower lobe of the lung (a finding referred to as mosaic attenuation) and the presence of well-defined, round, thin-walled air cysts (arrows). d) Photomicrograph (haematoxylin–eosin stain) showing lymphoplasmacytic infiltration and lymphoid follicles in the bronchiolar wall (arrowheads) and mild inflammation in the adjacent alveolar area. These findings are indicative of follicular bronchiolitis. Original magnification ×40.

FIGURE 2

a) Usual interstitial pneumonia in a 69-year-old woman with primary Sjögren's syndrome. High-resolution computed tomography (CT) showing bilateral reticular areas and honeycombing with peripheral and basal predominance (arrows). b) Combined pulmonary fibrosis and emphysema syndrome in a 68-year-old smoker with primary Sjögren's syndrome complicated by pulmonary hypertension. High-resolution CT shows bilateral reticular areas and honeycombing with posterior and basal predominance. Emphysema is predominant in apical areas. c, d) Lymphocytic interstitial pneumonia in a 59-year-old woman with primary Sjögren's syndrome and lymphocytic alveolitis. c) High-resolution CT shows thickening of interlobular septa with superimposition of intralobular reticulation. d) Photomicrograph (haematoxylin–eosin stain) shows diffuse thickening of alveolar septa and peribronchiolar infiltration with lymphocytes and plasma cells (arrowheads). Original magnification ×100.

FIGURE 3

Nonspecific interstitial pneumonia in a woman a, c) at the time of Sjögren's syndrome diagnosis and b) after 3 years. a, b) High-resolution computed tomography (CT) show bilateral areas of ground-glass attenuation and traction bronchiectasis (arrowheads) with peripheral intralobular reticulation. c) Photomicrograph (haematoxylin–eosin stain) shows diffuse and homogenous collagenous fibrosis in the alveolar area. d) Organising pneumonia and primary Sjögren's syndrome revealed by acute onset of pleuropneumonia in a 64-year-old man. High-resolution CT images show bilateral patchy areas of consolidation (#) and areas of ground-glass opacity (*). The patient was diagnosed with nonspecific interstitial pneumonia 2 years later.

FIGURE 4

a) Mucosa-associated lymphoid tissue lymphoma in a 45-year-old woman with primary Sjögren's syndrome. High-resolution computed tomography (CT) shows air space consolidation (*), mass (#) and interlobular septal thickening (arrowheads) in the right lower lobe. b) High-resolution CT obtained in a 64-year-old woman with lymphocytic interstitial pneumonitis and primary Sjögren's syndrome shows well-defined, round, thin-walled air cysts in the peribronchovascular regions (arrows), and areas of ground-glass and reticular attenuation.