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Fulminant Pneumocystis carinii pneumonia in 4 patients with dermatomyositis
H Bachelez1, B Schremmer, J Cadranel
1Institut de Recherche sur la Peau, Hôpital Saint-Louis, Paris, France.
Abstract:
Between 1989 and 1996, 4 cases of Pneumocystis carinii pneumonia (PCP) were observed in patients seronegative for the human immunodeficiency virus who were receiving corticosteroid therapy for dermatomyositis in our institution. These cases were considered unusual in light of the short delay of their onset after initiation of immunosuppressive therapy and their fulminant course: 3 of these patients died of PCP occurring during the first month of treatment with prednisone. In all 4 patients lymphopenia was observed before the initiation of corticosteroid treatment and low CD4 and CD8 cell counts were evident at the time of PCP. These observations support the view of an increase in both the severity and incidence of PCP in patients without human immunodeficiency virus infection and question the need for a primary prophylaxis in patients with connective tissue diseases receiving high-dose corticosteroid therapy.
Insights
Four patients without HIV developed severe Pneumocystis pneumonia (PCP) while on corticosteroid therapy for dermatomyositis. Early onset and rapid progression suggest increased PCP risk in these patients, questioning the need for primary prophylaxis.
Area of Science:
- Immunology
- Infectious Diseases
- Rheumatology
Background:
- Corticosteroid therapy is commonly used for autoimmune conditions like dermatomyositis.
- Pneumocystis pneumonia (PCP) is typically associated with human immunodeficiency virus (HIV) infection.
- The risk of opportunistic infections can increase with immunosuppressive treatments.
Observation:
- Four cases of PCP occurred in HIV-seronegative patients receiving corticosteroids for dermatomyositis between 1989 and 1996.
- PCP onset was rapid after initiating immunosuppressive therapy, with a fulminant course in three patients.
- Lymphopenia and low CD4/CD8 cell counts were noted before and during PCP in all affected patients.
Findings:
- Patients without HIV receiving high-dose corticosteroids for dermatomyositis experienced severe PCP.
- Early lymphopenia and reduced CD4/CD8 counts may indicate susceptibility to PCP in this population.
- The incidence and severity of PCP appear elevated in non-HIV patients undergoing immunosuppressive therapy.
Implications:
- These findings challenge the traditional understanding of PCP risk factors.
- The study questions the necessity of primary PCP prophylaxis in connective tissue disease patients on high-dose corticosteroids.
- Further research is warranted to evaluate prophylactic strategies for non-HIV immunocompromised individuals.