Lung complications of Sjogren syndrome

Abstract

Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.

A Rare Case of Sjogren’s Syndrome-Related Recurrent Pleural Effusion

“…The cause is unknown, and the etiology could be complicated. In roughly 30%-40% of patients with pSS, further systemic symptoms arise [ 8 ].…”

“…Pleural effusion in SS should always raise the potential of an overlap syndrome with SLE or RA (secondary SS) because pleural effusion is more common in the latter connective tissue disorders (CTDs) than in SS. Pleural infection or lymphoma is the most common differential diagnosis [ 8 ]. Bilateral pleural effusions are more common.…”

“…Complement levels in the pleural and/or serum may also be reduced. Pleural thickness has been found to be more frequently related to recurrent pneumonia and atelectasis in patients with SS [ 8 ].…”

A Case of Progressive Dyspnea: Lymphocytic Interstitial Pneumonia in Collagen Vascular Disease

“…Extra-glandular symptoms of Sjogren’s disease can involve almost every organ system and include polyarthralgia, erosive arthritis, peripheral neuropathies, oligoclonal and monoclonal gammopathies, cryoglobulinemia, and vasculitis [ 2 ]. Pulmonary involvement can be seen in 10-20% of patients and disease activity is seen primarily in the airways and interstitium - pleural disease and pulmonary hypertension are extremely uncommon [ 3 ]. Symptoms are variable in patients and may range from mild dyspnea to severe disability.…”

“…Pulmonary function testing commonly shows restrictive lung disease. Airway involvement in these patients may manifest as bronchial hyperresponsiveness (42-60%), bronchiolitis (12-14%) - predominantly follicular, and cylindrical bronchiectasis (7-54%) [ 3 ]. We describe a case of lymphocytic interstitial pneumonia with Sjogren predominant mixed connective tissue disorder (MCTD) and complicated by anti-Ku positive inflammatory myopathy.…”