[Fatal acute pneumonopathies in disseminated lupus erythematosus]. 1988

J F Kerbourc'h, and J Cledes, and M Garre, and C Zabbe, and S Gouva, and J Clavier
Service de Pneumologie et Oncologie broncho-pulmonaire, CHRU Augustin Morvan, Brest.

Two cases of systemic lupus erythematosus (SLE) complicated by pneumonia which resulted in death are reported. The first patient, a 21-year old woman, died of acute diffuse lupus pneumonia; the initial and unusual radiological image of "multiple balloons" progressed within 2 months to terminal interstitial fibrosis. The second patient, a 60-year old woman, died of infection on an interstitial pneumonia which turned into severe fibrosis within 16 months. Acute or chronic lupus pneumonia is uncommon, but it may follow a very serious course. Clinically, true lupus pneumonia must be distinguished from all other types of lung involvement in SLE, such as infection, pulmonary haemorrhage or oedema, iatrogenic pathology, thromboembolic disease, etc. The pathogenetic mechanism of pulmonary lesions directly related to SLE is obscure, although some lung biopsy specimens have shown positive immunofluorescence. Concerning treatment, the initial response to corticosteroid therapy is usually very good, especially in the acute forms of the disease. However, in severe cases immunosuppressive drugs or even plasma exchanges must be added to steroids. For treatment to be rapidly initiated the diagnostic procedures must be completed in the early stages of the disease, involving, when necessary, surgical lung biopsy.

UI MeSH Term Description Entries
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011014 Pneumonia Infection of the lung often accompanied by inflammation. Experimental Lung Inflammation,Lobar Pneumonia,Lung Inflammation,Pneumonia, Lobar,Pneumonitis,Pulmonary Inflammation,Experimental Lung Inflammations,Inflammation, Experimental Lung,Inflammation, Lung,Inflammation, Pulmonary,Inflammations, Lung,Inflammations, Pulmonary,Lobar Pneumonias,Lung Inflammation, Experimental,Lung Inflammations,Lung Inflammations, Experimental,Pneumonias,Pneumonias, Lobar,Pneumonitides,Pulmonary Inflammations
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D011658 Pulmonary Fibrosis A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death. Alveolitis, Fibrosing,Idiopathic Diffuse Interstitial Pulmonary Fibrosis,Fibroses, Pulmonary,Fibrosis, Pulmonary,Pulmonary Fibroses,Alveolitides, Fibrosing,Fibrosing Alveolitides,Fibrosing Alveolitis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000208 Acute Disease Disease having a short and relatively severe course. Acute Diseases,Disease, Acute,Diseases, Acute
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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