Systemic lupus erythematosus complicated by thrombotic microangiopathy

Area of Science:

• Rheumatology
• Hematology
• Nephrology

Background:

• Systemic lupus erythematosus (SLE) is an autoimmune disease with diverse clinical manifestations.
• Vascular complications are a significant cause of morbidity and mortality in SLE patients.
• Thrombotic microangiopathy (TMA) is a rare but severe complication associated with SLE.

Purpose of the Study:

• To describe the clinical features, laboratory findings, and treatment outcomes of SLE patients who developed TMA.
• To highlight the spectrum of vascular diseases in SLE, including non-inflammatory processes.

Main Methods:

• Retrospective case series of seven patients with SLE or SLE-like disease and TMA.
• Review of clinical data, laboratory results, treatment regimens, and patient outcomes.
• Histopathological examination of vascular changes in two cases.

Main Results:

• All seven patients presented with microangiopathic hemolytic anemia and thrombocytopenia; four had nervous system disease and five had renal dysfunction.
• Key laboratory findings included positive antinuclear antibody (ANA), anti-DNA, antiphospholipid antibodies, schistocytes, and elevated lactate dehydrogenase (LDH).
• Six patients improved with treatment including steroids, cyclophosphamide, plasmapheresis, or IVIG, but three of the six survivors died within one year.

Conclusions:

• Thrombotic microangiopathy is a serious complication of SLE that requires prompt recognition and management.
• The vascular disease spectrum in SLE extends beyond vasculitis to include non-inflammatory TMA.
• While treatment can improve acute outcomes, long-term survival in SLE patients with TMA remains challenging.