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Systemic lupus erythematosus with pulmonary hypertension
S Koyama1, T Ichiyoshi, M Chino
1Department of Internal Medicine, Nagano Red Cross Hospital.
Internal Medicine (Tokyo, Japan)
|January 1, 1996
Summary
Pulmonary hypertension is a rare but serious complication of systemic lupus erythematosus (SLE). This case highlights that even with controlled SLE, pulmonary hypertension can progress, leading to a fatal outcome.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
- Pulmonary hypertension (PH) is a recognized, albeit uncommon, complication of SLE.
- Raynaud's phenomenon is frequently associated with SLE and can be an early indicator.
Observation:
- A 50-year-old Japanese female with a history of Raynaud's phenomenon presented with dyspnea due to pulmonary hypertension.
- Diagnosis of SLE was confirmed by proteinuria, lymphocytopenia, pleurisy, and neurological symptoms, despite a low-titer antinuclear antibody.
- The patient experienced progressive pulmonary hypertension despite improvement in SLE activity and steroid treatment.
Findings:
- The case illustrates an unusual presentation of pulmonary hypertension as a critical complication of SLE.
- Neurological manifestations, including seizures, were noted, consistent with SLE.
- Steroid therapy, while managing SLE activity, did not impede the progression of pulmonary hypertension.
Implications:
- This case underscores the importance of monitoring for pulmonary hypertension in SLE patients, even with seemingly controlled disease.
- Pulmonary hypertension in SLE may follow an aggressive and refractory course, independent of SLE activity.
- Further research into the pathogenesis and treatment of SLE-associated pulmonary hypertension is warranted.