Table 1

The features of the acute and chronic pulmonary complications of juvenile systemic lupus erythematosus1 2

ComplicationPrevalence (%)Symptoms and signsInvestigationsTreatmentPrognosis
 Pleuritis50–80Dyspnoea, pleuritic pain, orthopnoea, pleural rubCXR: may have pleural effusionNSAIDs if mild, corticosteroids if severeGood
 Acute lupus pneumonitis<10Dyspnoea, cough, fever, pleuritic pain, haemoptysis, hypoxiaCXR: patchy acinar infiltrates at basesCorticosteroids, plasmaphoresisMortality >50%
 Alveolar haemorrhage<2Dyspnoea, cough, pleuritic pain, hypoxiaCXR: patchy acinar infiltrates at bases, fall in haemoglobinMethylprednisolone, then corticosteroids
Cyclophosphamide, plasmaphoresis
Mortality >50%
 Thromboembolic diseaseUnknownDyspnoea, pleuritic pain, fever, pleural rubCXR: may have pleural effusionAnticoagulantVariable. Can lead to pulmonary hypertension
 Infective  pneumoniaUp to 90Dyspnoea, fever, chest pain, coughCXR: consolidation
bronchoalveolar lavage may define organism
 Pulmonary hypertension5–14Dyspnoea, chest pain, signs of right heart failureECHO: pericardial effusion. ↑ Right ventricular pressures
↓DLCO, stable FVC
Pulmonary vasodilators
Consider anticoagulation
Mortality up to 50%
 Chronic interstitial lung disease3Exertional dyspnoea, chronic cough, pleuritic pain↓DLCO, ↓FVC
CXR/HRCT: ↓lung volume interstitial infiltrates, ground glass, honeycombing
CorticosteroidsVariable. Can be slowly progressive
 Shrinking lung syndrome<1Progressive dyspnoea, pleuritic pain, tachypnoeaCXR: ↓ lung volume, raised hemi diaphragm, basal atelectasis.
Immunosuppressive therapyGood
  • CXR, chest X-ray; DLCO, diffusing capacity for carbon monoxide; HRCT, high-resolution CT; NSAID, non-steroidal anti-inflammatory drugs.