Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Diffuse alveolar hemorrhage in patient with SLE
- Radiologic Findings
- Figs 1. Chest PA shows diffuse bilateral airspace opacification, especially Left lung.
Fig 2-3. Lung window setting of the high resolution chest CT scan shows diffuse ground-glass opacity and consolidation with mild interlobular septal thickening in both lungs, especially left lung.
Clinical Presentation
The patient visited our emergency department because of cough, dyspnea and hemoptysis, which have been aggravated for last 5 days. She was diagnosed with systemic lupus erythematosus (SLE) and lupus nephritis 5 years ago, but she stopped taking medication 2 years ago. Initial laboratory tests revealed severe anemia (hemoglobin: 5.7 g/dL), thrombocytopenia (79 109/L), and elevation of serum CRP (135.8 mg/L) and creatinine (4.8 mg/dL) with proteinuria (280 mg/dL) and hematuria (3+).
- Brief Review
- Diffuse alveolar hemorrhage (DAH) is a potentially catastrophic pulmonary complication of systemic lupus erythematosus (SLE) associated with a high mortality rate. DAH has been reported to complicate 2–5% of all cases of SLE. DAH is typically defined by the presence of three major components: (1) signs (bronchoscopy with bloody return) or symptoms (dyspnea, cough, hemoptysis) of pulmonary hemorrhage, (2) drop of hemoglobin (typically 1.5–2 g/dL) and (3) new onset of diffuse infiltrates on chest imaging. Lupus nephritis has frequently been linked to DAH, with evidence of active kidney disease in patients with lupus DAH.
The pathophysiology of DAH is not well understood, suggested theories postulate immune-mediated alveolar capillary endothelial damage and inflammation that would result in local or diffuse bleeding from those capillaries. Imaging studies are commonly consistent with unexplained new consolidation and ground-glass opacity, usually diffuse but can be seen in the perihilar regions, while apices are spared.
As with most organ-threatening manifestations of SLE, cyclophosphamide has been the mainstay of therapy for DAH, albeit in association with poor outcomes, which probably reflect its preferential use in the most critically ill patients. Other reported therapies include plasmapheresis, extracorporeal membrane oxygenation (ECMO), rituximab, mycophenolate mofetil, recombinant factor VII, and stem cell transplantation. However, the evidence to support any particular therapy is not strong.
- References
- 1. Kazzaz NM, Coit P, Lewis EE, McCune WJ, Sawalha AH, Knight JS. Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival. Lupus science & medicine. 2015;2(1):e000117.
2. Abdalla AO, Al-Khafaji J, Akella PM, Taha M. A fatal case of diffuse alveolar hemorrhage as the initial presentation of systemic lupus erythematosus: A case report and literature review. Respiratory Medicine Case Reports. 2018;24:55-7.
- Keywords
- Lung, Connective tissue diseases,