Elevated serum anti-endothelial cell autoantibodies titer is associated with lupus nephritis in patients with systemic lupus erythematosus.

BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease associated with endothelial dysfunction and the existence of multiple species of autoantibodies. However, the association between endothelial dysfunction and renal manifestations remains unclear in Taiwanese SLE patients. METHODS: Serum samples were collected from SLE patients with biopsy-proven lupus nephritis (n = 32), stable SLE patients (n = 32) and healthy controls (n = 32). The SLE Disease Activity Index (SLEDAI) of SLE patients was scored, and levels of anti-endothelial cell antibodies (AECA) and anti-endothelial activities in serum samples were measured by cell-enzyme-linked immunosorbent assay and crystal violet assay, respectively, using cultured human endothelial EA.hy926 cells. RESULTS: Significantly higher AECA (p<0.001) and anti-endothelial activities (p<0.001) were found in sera from patients with lupus nephritis compared with that from stable SLE patients or controls. Moreover, AECA titers (p<0.001) and anti-endothelial activities (p<0.001) were strongly correlated with SLEDAI scores in these patients. CONCLUSION: The strong correlations of AECA and anti-endothelial activity with lupus nephritis activity support an endothelial origin for renal complications in Taiwanese SLE patients.

Primary Sjögren's syndrome complicated with cryoglobulinemic glomerulonephritis, myocarditis, and multi-organ involvement.

Glomerulonephritis in primary Sjögren's syndrome is rarely reported. Cryoglobulinemic glomerulonephritis with the presence of cryoglobulin deposition in the glomerular capillary lumen in primary Sjögren's syndrome is extremely rare. A 51-year-old woman with primary Sjögren's syndrome for > 10 years complained of fever, hypertension, and proteinuria. In addition, novel manifestations, including myocarditis with heart failure, pericardial effusion, and polyneuropathy (sensory motor neuropathy) were also noted. Cryoglobulinemia test was positive, and kidney biopsy results were consistent with cryoglobulinemic glomerulonephritis. There were no symptoms associated with systemic lupus erythematosus or other connective tissue disease. Treatment with monthly methylprednisolone and cyclophosphamide pulse therapy for 6 months resulted in resolution of proteinuria, heart failure, and neurologic symptoms.

Prolonged survival after single lung transplantation for pulmonary hypertension secondary to systemic lupus erythematosus.

Pulmonary hypertension is one of the life-threatening complications of systemic lupus erythematosus, but these patients are often excluded from lung transplantation candidacy due to the nature of underlying multiple system involvement. We report a long-term survival after single lung transplantation in a case of systemic lupus erythematosus with severe pulmonary hypertension. It suggests that single lung transplantation may be considered in such patients, especially in condition of limited donor organ supply.