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Review series
Rare diseases • 7
Pleuropulmonary manifestations of systemic lupus erythematosus
Free
    1. Michael P Keane,
    2. Joseph P Lynch III
    1. Division of Pulmonary and Critical Care Medicine, University of Michigan Medical Center, Ann Arbor, MI 48109, USA
    1. Dr J P Lynch III, Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, 3916 Taubman Center, Box 0360, University of Michigan Medical Center, Ann Arbor, MI 48109-0360, USA

    https://doi.org/10.1136/thorax.55.2.159

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      Systemic lupus erythematosus (SLE), an autoimmune disorder which primarily affects women (10:1 female to male ratio), may affect virtually any organ.1 Predominant manifestations include non-deforming arthritis, serositis, photosensitivity, renal, haematological, and central nervous system involvement. Various laboratory abnormalities have been described in SLE, most commonly high titre antibodies directed against double stranded DNA,1nuclear ribonucleoprotein, Smith (Sm) antigen, Ro/SS-A, and La/SS-B/Ha.2 Pleuritis occurs in 17–60% of patients at some point in the course of the disease.1 ,3 Although severe parenchymal lung disease is uncommon, pulmonary complications of SLE are protean and include acute lupus pneumonitis, diaphragmatic dysfunction and shrinking lung syndrome, cavitating pulmonary nodules, pulmonary hypertension, pulmonary vasculitis, pulmonary embolism (often due to circulating anticardiolipin antibodies), alveolar haemorrhage (reflecting diffuse endothelial injury), chronic interstitial pneumonitis,3-7 bronchiolitis obliterans (with or without organising pneumonia),8 and opportunistic pulmonary infections or drug toxicity from immunosuppressive therapy.9

      Pleural disease

      The most common thoracic manifestation of SLE is pleuritis.3 ,10 Pleuritic pain is present in 45–60% of patients and may occur with or without a pleural effusion (fig1).3 Clinically apparent pleural effusions have been reported in up to 50% of patients with SLE3 and may be found in up to 93% of cases at necropsy.11 Effusions are usually bilateral but may be unilateral, equally distributed between the left and right hemithoraces. They are invariably exudative with higher glucose and lower lactate dehydrogenase levels than those found in rheumatoid arthritis.12 Antinuclear antibody (ANA), anti-DNA antibodies, and LE cells have been found in the pleural fluid.13 ,14 LE cells appear to be relatively specific.15 Pleural biopsies have rarely been performed in SLE and the findings are non-specific, revealing lymphocytic and plasma cell infiltration, fibrosis, and fibrinous pleuritis.16They are indicated …

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