Teaching Case

Patient with xerostomia, keratoconjunctivitis sicca, fatigue and Raynaud's phenomenon; there were no respiratory symptoms.

The patient was diagnosed with xerostomia and keratoconjunctivitis sicca. During follow-up she presented with non-specific manifestations of fatigue and Raynaud's phenomenon; there were no respiratory symptoms. On chest auscultation bibasilar inspiratory crackles were noted. Pulmonary function tests showed reduced diffusion capacity. Bronchoalveolar lavage findings showed a reversal of the CD4:CD8 ratio. Her serum contained autoantibodies directed against rheumatoid factor, extractable nuclear antigen and double-stranded DNA; the Waaler-Rose test was also positive. A chest radiograph and HRCT examination of the chest were performed.

Sjögren's syndrome (1) is a chronic, slowly progressive, autoimmune disease characterised by lymphocytic infiltration of the exocrine glands. The salivary and lacrimal glands are most frequently involved, but extraglandular manifestations occur in 5-10% of cases. The disease can be seen alone (primary Sjögren's syndrome) or in association with another autoimmune disease (RA, SLE or systemic sclerosis). It usually affects women (F:M, 9:1)(2) in the fourth and fifth decades of life.

Pulmonary involvement in collagen vascular diseases is common. Pleuropulmonary manifestations (1) of Sjögren's syndrome include airways disease, interstitial pulmonary disease, pleural disease and lymphoproliferative disorders. Airways disease results from tracheobronchial desiccation and recurrent infection, and from lymphocytic infiltration of the airways, which causes follicular lymphocytic bronchiolitis. Interstitial lung disease may be due to fibrosing alveolitis or lymphocytic interstitial pneumonitis. Pleural disease consists of pleuritis and pleural thickening or effusion and is more common in secondary Sjögren's. In fact lung abnormalities in secondary Sjögren's syndrome are often dominated by the associated autoimmune disease, and abnormalities that reflect the associated disease may be difficult to distinguish from those that characterise the Sjögren's syndrome itself. Patients also have an increased risk of lymphoma, which should be suspected in cases of mediastinal lymphadenopathy or/and in cases of single or multiple areas of consolidation with prominent air-bronchogram, which show little or no change over a prolonged period.

The advanced disease is easily detectable on chest radiographs. Plain films show primarily a reticular or reticulonodular interstitial pattern in the lower parts of the lungs. Associated airspace shadowing is suggestive of lymphocytic interstitial pneumonitis (LIP). Other radiological features that have been reported are bronchiectasis, pleural effusions or enlarging mediastinal lymph nodes and multiple nodular opacities in cases of pseudolymphoma or lymphoma. (2)

Like other systemic disorders the most common HRCT changes are signs of chronic bronchiolar inflammation alone or in association with the presence of varying degrees of interstitial disease. The spectrum of HRCT findings (3,4,5)in small airways disease includes air trapping, bronchiectasis/bronchiolectasis, bronchial wall thickening and tree-in-bud appearance. An unusual manifestation of airways disease in Sjögren's syndrome consists of bronchiolitis associated with cystic lung disease, resulting from severe peribronchial infiltrate with air trapping. Signs of fibrosing alveolitis include fibrosis (intralobular interstitial thickening, irregular interfaces, visible intralobular bronchioles, honeycombing and traction bronchiectasis), irregular interlobular thickening, ground-glass opacity, with a peripheral, subpleural, lower lung zone and posterior predominance. Ground-glass opacity can be due to present active inflammation or due to thickening of the intralobular septa. The disease can cause dry eyes, dry mouth, vasculitis, and neurologic disease, and each symptom may be at times correctly attributed to Sjogren's or incorrectly attributed to another disease. The treatment of these symptoms is still mainly symptomatic. The outcome of the patient depends on the evolution of the disease but a standard basic treatment is not available for the moment.