Abstract
We report a case of systemic lupus erythematosus (SLE) in a 44-year old Caucasian woman complicated with pneumonia and severe respiratory failure requiring ICU treatment and mechanical ventilation. Symptoms developed in a generally well controlled SLE course after sudden stop in immunosupresant therapy (methotrexate, cyclosporin and methylprednisolone). A fulminant course of the disease, an interstitial pattern in a high resolution computed tomography (HRCT) and negative repeated sputum, blood and bronchoaspirate cultures enabled diagnosis of fulminant lupus pneumonitis. The response to pulses of cyclophosphamide and methylprednisolone was good but complicated with a significant leukopenia. HRCT confirmed significant remission of pulmonary changes. Fulminant lupus pneumonitis is a rare but potentially life threatening complication of SLE. Differential diagnosis requires exclusion of pneumonia induced by pathogens such as Pneumocystis jirovevecii (carinii) and Mycobacterium sp. Intensive immunosuppressive therapy and close cooperation between ICU, pulmonology and rheumatology departments is necessary in such a case to minimalize the risk of fatal outcome.
Highlights
Systemic lupus erythematosus (SLE) is an autoimmune chronic systemic disease which can involve several organs such as skin, lungs, brain and heart
Pulmonary manifestations of SLE can include a wide spectrum of diseases such as pleuritis, pneumonia, pulmonary embolism, pneumothorax and pulmonary haemorrhage [1,2]
Pulmonary manifestations of SLE can include a wide spectrum of diseases such as pleuritis, pneumonia, pulmonary embolism, pneumothorax and pulmonary haemorrhage cyclophosphamide boluses
Summary
Systemic lupus erythematosus (SLE) is an autoimmune chronic systemic disease which can involve several organs such as skin, lungs, brain and heart. As the basic treatment of SLE include several drugs inducing immunosuppression pneumonia and acute respiratory distress syndrome (ARDS) followed by sepsis are the most common causes of admission to the ICU and fatal outcome in these patients. Few cases of non-infectious fulminant lupus pneumonitis mimicking, by its interstitial pattern, atypical pneumonia has been presented in literature to date.
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