Life threatening pneumonia in a lupus patient: a case report
© Kupczyk et al; licensee BioMed Central Ltd. 2008
Received: 28 May 2008
Accepted: 31 July 2008
Published: 31 July 2008
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.
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]. 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. Only few cases of non-infectious fulminant lupus pneumonitis mimicking, by its interstitial pattern, atypical pneumonia has been presented in literature to date. Differential diagnosis and treatment of this condition represent a real challenge but only early introduction of intensive immunosuppressive treatment and close cooperation between ICU, pulmonology and rheumatology departments reduce the risk of fatal outcome.
The above described case presents fulminant lupus pneumonitis a rare but life threatening complication of SLE. Pulmonary manifestations of SLE can include a wide spectrum of diseases such as pleuritis, pneumonia, pulmonary embolism, pneumothorax and pulmonary haemorrhage [1, 2]. Hsu et al in the group of 51 critically ill patients with SLE treated in the ICU found the mortality rate about 47% with pneumonia and acute respiratory distress syndrome (ARDS) followed by sepsis as the most common cause of admission . The pathogens cultured in studied cases included Pseudomonas aeruginosa, Salmonella sp, Staphylococcus aureus and epidermidis, Streptococcus pneumoniae, E. coli and Acinetobacter baumannii. In two patients disseminated tuberculosis was diagnosed. These findings are not surprising if we remember that glucocorticosteroids and other drugs used in the treatment of SLE induce significant immunosupression thus increasing the risk of all kinds of infections. In line with the paper of Hsu et al septic shock is associated with higher risk of fatal outcome in SLE patient treated in ICU, that is why identification of pathogen and immediate antimicrobial therapy is of great importance . Only one patient (1,6%) from the study group  has been diagnosed with noninfectious pneumonitis. Comer et al reported another case of a patient with SLE, whose pregnancy was complicated by fulminant pneumonitis and pericarditis . Single cases has been also presented by other authors [5, 6]. Isbister et al described a 14 year old girl with SLE, complicated with lupus pneumonitis, acute renal failure and aplasia . Plasmapheresis, dialysis and immunosuppressive therapy were useful in the treatment. Mok CC et al described two clinically very similar cases . One patient was confirmed to have coronavirus pneumonia while the other had fulminant lupus pneumonitis.
Diagnosis of fulminant lupus pneumonitis is a real challenge. As presented above several patogens should be taken into consideration in a case of interstitial pneumonitis including but not limited to viruses , Pneumocystis carinii  and Mycobacterium sp . In the case we present diagnosis has been made basing on a data from several negative cultures and striking history of sudden reduction, not increase in the dosis of the immunosuppressive agents. Intensive immunosuppressive treatment including glucocorticosteroids, cyclophosphamide, methotrexate, cyclosporin and in selected cases plasmapheresis should be introduced. A close cooperation between ICU, pulmonology and rheumatology departments is required in such a case to minimalize the risk of fatal outcome.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
List of abbreviations
Acute respiratory distress syndrome
Bone marrow biopsy
High resolution computed tomography
Intensive care unit
Systemic lupus erythematosus
white blood count.
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