Highlights
- •Systemic auto-immune diseases (SAID) in thrombotic microangiopathies (TMA) [SAID-TMA] include mainly systemic lupus erythematosus, systemic sclerosis and primary Sjögren's syndrome.
- •SAID-TMA improves with the treatment of the underlying SAID
- •TPE does not seem to have an early effect on TMA features at Day-7 nor Day-15
Abstract
Context
The management of systemic auto-immune diseases (SAID) -associated thrombotic microangiopathies
(TMA) [SAID-TMA] remains debated.
Objectives
To provide a demographic, clinical and therapeutic picture of SAID-TMA.
Methods
A cross-sectional analysis was conducted on adult patients presenting with SAID and
TMA from the French National TMA Registry over a 20-year period. Clinical features
were extracted and compared to those from a historical cohort of atypical haemolytic
and uremic syndrome (aHUS) patients.
Results
Forty-one patients with SAID-TMA were compared to 78 patients with aHUS from a historical
cohort. Connective tissue diseases (CTD) were systemic lupus erythematosus (n=18),
primary Sjögren's syndrome (n=7), systemic sclerosis (n=11), mixed CTD (n=2) and 2
cases of vasculitides, including 7 overlapping forms and 8 cases of primary antiphospholipid
syndromes (APLS). Patients with SAID-TMA generally had pre-existing chronic kidney
failure (OR= 3.17, 95%CI: 1.204 to 7.923; p= 0.016) compared to aHUS patients, though
creatinine levels were significantly lower (216 [IQR, 108-334] µmol/L vs. 368 [IQR,
170-722] µmol/L; p= 0.002). Patients were less likely to recover if renal replacement
therapy was needed at onset (OR= 0.07; 0.02 to 0.34; p <0.0005). Two patients died.
Thirty patients responded to immunosuppressive treatment and complete remission was
achieved in 25 cases. By contrast, therapeutic plasma exchange (TPE) did not have
an early effect on TMA features at Day-7 nor Day-15 (p >0.05).
Conclusion
The management of SAID-TMA implies an early initiation of immunosuppressive drugs
for flares of the associated SAID, whereas TPE seem ineffective.
Key messages
- •Systemic auto-immune diseases (SAID) in thrombotic microangiopathies (TMA) [SAID-TMA] include mainly systemic lupus erythematosus, systemic sclerosis and primary Sjögren's syndrome.
- •SAID-TMA improves with the treatment of the underlying SAID
- •Therapeutic plasma exchange does not seem to have an early effect on TMA features at Day-7 nor Day-15
Keywords
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Article info
Publication history
Published online: June 23, 2021
Accepted:
May 28,
2021
Received in revised form:
May 16,
2021
Received:
February 2,
2021
Identification
Copyright
© 2021 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
