Elsevier

Clinics in Chest Medicine

Volume 31, Issue 3, September 2010, Pages 489-500
Clinics in Chest Medicine

Pulmonary Involvement in Sjögren Syndrome

https://doi.org/10.1016/j.ccm.2010.05.007Get rights and content

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Diagnostic criteria

The diagnosis of SS is based on 6 criteria proposed by the American-European consensus group11: (1) ocular symptoms of dryness, (2) oral symptoms of dryness or persistently swollen salivary glands, (3) objective evidence of ocular involvement (positive results on Schirmer test or Rose Bengal), (4) lymphocytic sialoadenitis on minor salivary gland biopsy, (5) objective evidence of salivary gland involvement (abnormal salivary flow, parotid salography, or salivary scintigraphy), and (6)

Prevalence of pulmonary involvement

A plethora of pulmonary manifestations is found in pSS (Box 1). The most frequent are interstitial lung disease (ILD), airway disease (xerotrachea and small airway obstruction), and lymphoproliferative disorders. The diagnosis of lung disease in SS is based on clinical evaluation, radiographic findings, pulmonary functional testing, bronchoalveolar lavage (BAL), and histopathology. The prevalence of pulmonary involvement in pSS has been reported to be between 9% and 75%.12, 13 Lung

Clinical manifestations

Although many patients remain asymptomatic, the main clinical manifestations of pulmonary involvement in pSS are cough and exertional dyspnea. These symptoms are associated with ILD and airway involvement, and their frequency depends on the dominating feature of lung disease. Dry cough is associated with xerotrachea and airway disease, which may remain undiagnosed when cough is the only sign or symptom of lung disease. Constantopoulos and colleagues13 reported dry cough as the primary symptom

Pulmonary function

In patients with pSS and ILD, PFT commonly reveals a restrictive pattern with a low diffusing capacity.19, 20 In one study of 100 patients with pSS, significant reductions in FEV1, FVC, and diffusing capacity of lung for carbon monoxide (Dlco) were present in 14, 12, and 10 patients, respectively.20 The presence of small airway dysfunction is common in nonsmoking patients with pSS and is often asymptomatic.16, 17 The correlations between radiological findings and PFT vary among different case

Bronchoalveolar lavage

The role of BAL in the evaluation of SS-related lung disease is not clear. Dalavanga and colleagues22 studied BAL in patients with pSS and found alveolitis in 52% of patients, characterized by an increase in the total cell count and a predominance of lymphocytes on the differential count. The CD4/CD8 ratio was reduced in the patients with pSS and a lymphocytic alveolitis compared with patients with pSS without alveolitis and healthy nonsmoking controls. The patients with a lymphocytic

Radiographic findings

The chest radiograph may be normal or show bilateral alveolar, reticular, or nodular markings or a combination thereof, mainly localized in the middle and lower lung zones. The chest radiograph, however, has a low diagnostic value in the detection of pulmonary involvement in SS, especially in the early stages of lung disease. In a series of 37 asymptomatic patients with pSS, all chest radiographs were normal, whereas abnormal computed tomographic (CT) findings were detected in 24.16 These

Airway manifestations

Upper airway involvement is a common phenomenon in SS. Dryness of nasal and oral mucosa due to diminished glandular function can be the cause of smell and taste disorders, bleeding, sinusitis, and septal perforation. Freeman and colleagues27 reported that in 111 patients with pSS, 50% complained of nasal symptoms, although only 20% had abnormal rhinoscopy. Similarly, 60% complained of throat symptoms, but only 20% had pathologic findings on indirect laryngoscopy.

Involvement of the trachea and

Follicular bronchiolitis

Follicular bronchiolitis is a type of lymphoproliferative disorder of the lung and usually coexists with lymphocytic bronchitis, bronchiolitis, or LIP in patients with pSS. Histologically, follicular bronchiolitis is characterized by nodules of lymphocytic infiltrates with reactive germinal centers surrounding the terminal and respiratory bronchioles (bronchi-associated lymphoid tissue hyperplasia). Clinically it presents with cough and dyspnea. A reticular or reticulonodular pattern is seen

Interstitial lung disease

Diffuse ILD is the most common form of lung involvement in pSS and includes LIP, NSIP, usual interstitial pneumonia (UIP), and OP.

Lymphoma

The evolution from the benign lymphocytic infiltration characteristic of SS to lymphoma is a multistep process. Epithelial infiltration consists of polyclonal B and T lymphocytes, whereas lymphoma represents monoclonal B-cell proliferation. The risk of lymphoma in patients with SS is 44 times higher than the incidence in a healthy population.45 It has been estimated by several studies that 4% to 8% of patients with SS will develop lymphoma in the course of their disease.46, 47 The most common

Pseudolymphoma

Pseudolymphoma or pulmonary nodular lymphoid hyperplasia is considered a benign lesion characterized pathologically by infiltration of mature polyclonal lymphocytes and plasma cells. Strimlan and colleagues12 reported 1 case of pseudolymphoma in a large series of patients with pSS and sSS. There are some controversial data on whether pseudolymphoma is different from extranodal marginal zone B-cell lymphoma. Differentiation of pseudolymphoma from other lymphoproliferative disorders can be based

Amyloidosis

Amyloidosis is caused by the extracellular deposition of an abnormal fibrillar protein. The secondary form of the disease has been occasionally described in SS and is often associated with lymphoproliferative disorders. In the study of 343 patients with pSS and sSS by Strimlan and colleagues,12 2 patients were diagnosed with pulmonary amyloidosis. Jeong and colleagues52 studied CT scans in 5 women with pSS with pulmonary amyloidosis and lymphoproliferative disorders and described multiple

Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is associated with several connective tissue disorders, most commonly with scleroderma. Although pulmonary involvement in SS is relatively frequent, the prevalence of pulmonary hypertension is rare.54, 55 Pulmonary hypertension is one of the more severe pulmonary complications of SS, with survival rates of 73% at 1 year and 66% at 3 years.56

The pathogenesis of pulmonary hypertension in pSS is not clear, but it is believed that it results from vasculitis

Other manifestations

Pleural effusions in SS are more often associated with sSS in rheumatoid arthritis or systemic lupus erythematosus. Pleural effusions in pSS are rare. So far, 9 cases of pSS complicated by pleural effusions have been reported in the literature. Pleural effusions are more often bilateral but can be unilateral. The pleural fluid is exudative with predominant lymphocytic cell counts, normal glucose levels and pH, and low adenosine deaminase levels. In 2 of the reported cases, pleural effusions

Treatment and prognosis

pSS usually is a slowly progressing disease; if extraglandular involvement or lymphoma is present, the progression is faster. The most common pSS symptoms (sicca) are not treated with corticosteroids or other immunosuppressive drugs because of lack of efficacy. Topical treatment is recommended for symptom amelioration, such as lubricants for the mouth and eyes, nonsteroid antiinflammatory for joint pains, and normal saline nebulizers for xerotrachea. Systemic therapies are reserved for severe

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