CT Findings of Thoracic Manifestations of Primary Sjögren Syndrome: Radiologic-Pathologic Correlation

Abstract

Primary Sjögren syndrome is an immune-mediated exocrinopathy characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands. Various systemic extraglandular disorders are associated with primary Sjögren syndrome, and the thorax is commonly affected. The pulmonary manifestations of primary Sjögren syndrome may be categorized as airway abnormalities, interstitial pneumonias, and lymphoproliferative disorders; in each category, bronchiectasis or centrilobular nodules, nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia are common. These manifestations do not usually occur in isolation; they are concomitantly seen with other types of lesions. Mucosa-associated lymphoid tissue (MALT) lymphoma and amyloidosis are key components of lymphoproliferative disorders, and MALT lymphoma should always be considered because its morphologic characteristics are similar to those of benign lymphoproliferative disorders. Amyloidosis is rare but important because it carries a risk for underlying MALT lymphoma or plasmacytoma, and it may lead to hemoptysis during biopsy. In addition, thin-walled air cysts are characteristic of primary Sjögren syndrome, irrespective of the main pulmonary manifestations. Lymphadenopathy and multilocular thymic cysts may be seen in the mediastinum. During the follow-up period, there is a risk for acute exacerbation of interstitial pneumonia and development of malignant lymphoma. Often, primary Sjögren syndrome is subclinical, but there are various underlying risks. Thus, imaging findings are important. In addition to the various types of interstitial pneumonia and airway abnormalities, air cysts and mediastinal manifestations may help diagnose primary Sjögren syndrome.

Chest HRCT findings in patients with primary Sjögren’s syndrome

“…Respiratory manifestations in pSS are heterogeneous and may include: dry cough, diffuse panbronchiolitis (DPB), bronchiectasis, nonspecific interstitial pneumonia (NSIP), idiopathic pulmonary fibrosis (IPF), cryptogenic organizing pneumonia (COP), lung cysts, nodular opacities, follicular bronchiolitis, lymphoid interstitial pneumonia (LIP), pseudolymphoma, lymphomatoid granulomatosis, lymphoma (usually of mucosaassociated lymphoid tissue type-MALT), pulmonary amyloidosis, pulmonary hypertension and pleural involvement. 11,12 The mediastinal manifestations of pSS include lymphadenopathy, thymic lymphoid hyperplasia, multilocular thymic cysts and, rarely, MALT lymphoma. 11,12 Such a wide range of manifestations may lead to significant diagnostic and therapeutic difficulties.…”

“…11,12 The mediastinal manifestations of pSS include lymphadenopathy, thymic lymphoid hyperplasia, multilocular thymic cysts and, rarely, MALT lymphoma. 11,12 Such a wide range of manifestations may lead to significant diagnostic and therapeutic difficulties. Occasionally, a histopathological assessment of lung biopsy is needed.…”

“…In HRCT scans, bronchial wall thickening (8-68%), nodules (6-29%), bronchiectasis (5-46%), air trapping (32%) and ground-glass attenuation were most frequently observed. [11][12][13] According to available publications, the prevalence of respiratory manifestations in pSS is estimated to be about 9-12% and clinical features are present in about 43-75% of patients when radiology imaging such as chest X-ray, HRCT or MRI are performed.…”

Pulmonale Beteiligung bei Kollagenosen

“…Beim primären Sjögren-Syndrom stehen drei meist koexistente pulmonale Manifestationsformen im Vordergrund [29], die im Folgenden dargestellt werden. …”

“…Darüber hinaus können sich hyperplastische Veränderungen in den hilären und mediastinalen Lymphknotenstationen und im lymphoiden Gewebe des Mediastinums mit lymphoider Hyperplasie des Thymus und Thymuszysten manifestieren [29].…”