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Diffuse alveolar hemorrhage in lupus nephritis complicated by microscopic polyangiitis
Motohide Isono1, Hisazumi Araki, Takao Haitani
1Department of Medicine, Otsu Municipal Hospital, Otsu, Shiga 520-0804, Japan. isonom@mx3.canvas.ne.jp
Diffuse alveolar hemorrhage (DAH), a rare complication of systemic lupus erythematosus (SLE), can present initially with severe organ involvement. This case highlights the association between myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) and severe manifestations in SLE patients.
Area of Science:
- Rheumatology
- Pulmonology
- Nephrology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
- Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening complication of SLE.
- Myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) are typically associated with small vessel vasculitis.
Observation:
- A 74-year-old woman presented with DAH as the initial manifestation of SLE.
- The patient also exhibited features of microscopic polyangiitis, including crescentic glomerulonephritis and purpura.
- Her serology was positive for MPO-ANCA.
Findings:
- Initial treatment with plasma exchange and methylprednisolone resulted in transient improvement.
- The patient experienced recurrent pulmonary hemorrhage.
- She subsequently developed cryptococcal pneumonia, leading to mortality.
Implications:
- This case underscores the potential for DAH to be the presenting feature of SLE.
- The presence of MPO-ANCA in this SLE patient correlated with severe organ involvement, specifically pulmonary hemorrhage and crescentic glomerulonephritis.
- This association suggests a complex interplay between SLE, vasculitis, and severe organ damage, necessitating careful monitoring and tailored treatment strategies.
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