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Interstitial lung disease associated with Sjögren's syndrome: Management and prognosis

Carlos Roberto Ribeiro de Carvalho, MD, PhD
Daniel Deheinzelin, MD, PhD
Ronaldo A Kairalla, MD, PhD
Section Editor
Talmadge E King, Jr, MD
Deputy Editor
Helen Hollingsworth, MD


Sjögren's syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function and associated with lymphocytic infiltration of exocrine glands, especially the lacrimal and salivary glands. In addition to causing dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), SS can affect extraglandular organ systems including the skin, lung, heart, kidney, neural, and hematopoietic systems.

Respiratory complications of SS include airway mucosal dryness (also known as xerotrachea), a variety of interstitial lung diseases (ILDs), non-Hodgkin lymphomas, pleural thickening or effusion, and, rarely, thromboembolic disease or pulmonary hypertension.

The management and prognosis of interstitial lung disease in SS will be reviewed here. The clinical manifestations, classification, evaluation, and diagnosis of Sjögren's syndrome and its associated interstitial lung diseases are presented separately. (See "Clinical manifestations of Sjögren's syndrome: Exocrine gland disease" and "Clinical manifestations of Sjögren's syndrome: Extraglandular disease" and "Diagnosis and classification of Sjögren's syndrome" and "Pathogenesis of Sjögren's syndrome" and "Interstitial lung disease associated with Sjögren's syndrome: Clinical manifestations, evaluation, and diagnosis".)


Management strategies for Sjögren-associated lung diseases are empiric, since no controlled studies have been performed. We base the treatment approach on the identified lung pathology, and the severity of symptoms, physiologic impairment, and extent of radiographic disease. (See "Interstitial lung disease associated with Sjögren's syndrome: Clinical manifestations, evaluation, and diagnosis", section on 'Histopathology'.)

Sjögren-associated ILD without biopsy confirmation — Asymptomatic patients with mild interstitial lung disease (ILD) based on high resolution computed tomography (HRCT) and/or pulmonary function testing (PFTs) may prefer not to pursue a lung biopsy for histopathologic diagnosis. For these patients, we monitor symptoms, HRCT, and PFTs at 6 to 12 month intervals. If progressive disease is identified, we typically advise further evaluation to identify the specific histopathology. (See "Interstitial lung disease associated with Sjögren's syndrome: Clinical manifestations, evaluation, and diagnosis", section on 'Evaluation and diagnosis'.)

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Literature review current through: Sep 2017. | This topic last updated: May 26, 2016.
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