Table 1. Comparison of common clinical characteristics of causes of peripartum TMA [17, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34].
TTP | “Typical” HUS | Atypical HUS | Pre-eclampsia | HELLP | SRC | APS | |
---|---|---|---|---|---|---|---|
Clinical presentation | Fever, HTN, neurologic symptoms, bleeding, purpuric rash | Abdominal pain, bloody diarrhea due to verotoxin-production; neurologic symptoms possible | None or non-specific prodrome with malaise, fatigue, upper respiratory symptoms | HTN, nausea, vomiting, abnormal vision | Abdominal pain, headache, malaise, nausea, vomiting, HTN | Dyspnea, altered mentation, HTN | HTN, arterial and venous thromboses, fetal demise |
Typical laboratory findings | MAHA, ↓platelets, AKI, proteinuria, hematuria |
MAHA, ↓platelets, AKI, proteinuria, hematuria |
MAHA, ↓platelets, AKI, proteinuria, hematuria, ↓complement |
Proteinuria, hyperuricemia |
MAHA, ↑AST, ↓platelets |
MAHA, AKI, proteinuria, hematuria |
MAHA, ↓platelets, AKI, proteinuria, hematuria, antiphospholipid antibodies |
Other possible laboratory findings | Low level of ADAMTS13 | Stool culture positive for verotoxin-producing E. coli | Abnormalities of complement regulatory proteins (Factors B, H, and I, MCP, C3, thrombomodulin) | MAHA, AKI, and ↑AST may occur in severe pre-eclampsia | AKI | Autoantibodies suggestive of scleroderma | Autoantibodies suggestive of SLE |
Occurrence and timing related to pregnancy |
Rare; generally < 23 – 26 weeks gestation | Rare; post-partum | Rare; post-partum | > 20 weeks gestation; occasionally postpartum | > 20 weeks gestation; occasionally postpartum | Unclear; > 24 weeks gestation when observed | 1/3 of cases reported during pregnancy or postpartum period |
Treatment | Plasma exchange, steroids, rituximab | Supportive | Plasma exchange, eculizumab | Anti-HTN therapy; when severe, magnesium sulfate and delivery | Delivery | ACE-inhibitor therapy | Anticoagulation, plasma exchange |
Renal prognosis | ESRD is rare (0 – 6%) | CKD in 5 – 25% | ESRD in 20 – 60% | Low risk of ESRD (~ 8%) | ESRD is rare (0 – 2%) | ESRD in 20% treated with ACE-inhibitor | ESRD is rare (few case reports) |
TMA = thrombotic microangiopathy; TTP = thrombotic thrombocytopenic purpura; HUS = hemolytic uremic syndrome; SRC = scleroderma renal crisis; APS = antiphospholipid antibody syndrome; HELLP = hemolysis, elevated liver enzymes, low platelets; HTN = hypertension; MAHA = microangiopathic hemolytic anemia; AKI = acute kidney injury; AST = aspartate aminotransferase; ADAMTS = a disintegrin-like and metalloprotease with thrombospondin type 1 motif; MCP = membrane cofactor protein; SLE = systemic lupus erythematosus; ACE = angiotensin converting enzyme; ESRD = end-stage renal disease; CKD = chronic kidney disease.