SLE and TTP Flashcards
SLE and TTP
Epidemiology
TTP typically precedes the diagnosis of SLE in children. 6% of patients with TTP have SLE.
SLE typically precedes the diagnosis of TTP in adults. <5% of SLE patients have TTP.
SLE and TTP
Epidemiology
Differential diagnoses of MAHA other than TTP in patients with SLE:
Disseminated intravascular coagulopathy
Malignant hypertension
SLE and TTP
Epidemiology
Differential diagnoses of MAHA other than TTP in patients with SLE:
(±Catastrophic) antiphospholipid antibody syndrome
Active lupus complicated by hemolytic anemia
Small-vessel vasculitis with end-organ damage
SLE and TTP
Clinical Manifestations
Presence of MAHA, anemia, thrombocytopenia
SLE and TTP
Clinical Manifestations
Patients with SLE-associated TTP tend to be younger males, have worse kidney function (both proteinuria and serum creatinine), class IV diffuse proliferative LN, lower C3 levels, and other complications such as APS, serositis, pulmonary hypertension, nephritis, and central nervous system involvement.
SLE and TTP
Clinical Manifestations
Mortality in SLE-associated TTP is approximately 50%, commonly associated with infections
SLE and TTP
Diagnosis
Diagnosis is based on clinical assessment, serologic and coagulation tests, and exclusion of all other differential diagnoses above.
SLE and TTP
Diagnosis
Unclear utility of ADAMTS13 in SLE: reduced ADAMTS13 levels and/or presence of ADAMTS13 antibodies may be detected in SLE patients without overt TMA. However, SLE patients with TTP do have severely depressed ADAMTS13 levels or high titers of anti-ADAMTS13 inhibitory antibodies.
SLE and TTP
Management
Poor data
Plasma exchange plus immunosuppressive therapy (e.g., corticosteroids and CYC) to control both TTP and active SLE, respectively.
SLE and TTP
Management
Rituximab may be considered if above fails. Again, poor data.
