Truly a Catastrophe: A Case of Severe Anti-Phospholipid Syndrome in the Setting of Lupus
- Glomerular Diseases: From Inflammation to Fibrosis - I
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
- Pedapati, Shilpa, Banner University Medical Center Tucson, Tucson, Arizona, United States
- Vahdani, Golnaz, Banner University Medical Center Tucson, Tucson, Arizona, United States
Antiphospholipid syndrome(APS) is characterized by venous or arterial thrombosis in the presence of antiphospholipid antibodies. It can occur as a primary vs secondary condition in which its associated with inflammatory disorders like systemic lupus erythematosus(SLE). APS related kidney disease can manifests itself in various ways from non-inflammatory occlusive diseases with arterial and venous thrombosis, TMA as well as glomerular diseases. Lupus can affect kidneys in the form of glomerulopathies in addition to TMA. It is important to distinguish inflammatory versus thrombotic lesions in order to ensure timely diagnosis and initiation of emergent management.
49-year-old female with a history of SLE not on any immunosuppression was admitted due to diarrhea and was found to have severe acute renal failure with a creatinine of 13. Serologies demonstrated low C3 and C4, Positive ANA, double-stranded DNA, cardiolipin IgG antibody, beta-2 glycoprotein IgG and c-ANCA. Duplex was negative for renal artery stenosis or renal vein thrombosis. She was initiated on hemodialysis secondary to volume overload. Anticoagulation was also initiated due to new diagnosis of bilateral upper extremity deep vein thrombosis. Kidney biopsy performed demonstrating chronic TMA in the glomeruli, negative for acute thrombi, crescents/features of lupus or a full house pattern on immunofluorescence. MR angio was also obtained in the setting of concern for APS showing complete intraluminal thrombus occlusion of aorta at the level of renal arteries down to the bifurcation, with occlusion of the right renal artery and narrowing of left renal artery. Due to concern for catastrophic APS she was promptly started on plasmapheresis and steroids. She remained dialysis dependent and had recurrent hospitalizations for pulmonary edema secondary to hypertension, and eventually underwent aortoiliac bypass with improvment of hypertension and remained dialysis dependent.
Renal prognosis remains poor in patients affected by SLE in the presence of aPLs and may lead to ESRD .Prompt initiation of anticoagulation, Immunosuppression and plasmapheresis remains the mainstay of treatment. Having a high index of suspicion is very important to diagnose thrombotic lesions as it affects not only kidney outcomes but also contributes to overall morbidity and mortality.