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Diagnosis: Lupus Nephritis and Thrombotic Microangiopathy
Thrombotic microangiopathy (TMA) is not an uncommon vascular change in patients with lupus nephritis (LN), especially in those with severe diffuse proliferative glomerulonephritis. TMA is found in about 2% - 24% of patients with LN. Biopsy specimens of LN patients with TMA have significantly higher LN activity indices than of those without TMA (Kotb HA, et al. Thrombotic microangiopathy in lupus nephritis patients. Kasr Al Ainy Medical Journal 2016, 22:12–17 [Free full text link]).
Diagnosis of TMA in systemic lupus erythematosus (SLE) is sometimes difficult because these two disorders share similar clinical features, including haemolytic anaemia, thrombocytopenia, neurological deficits, renal involvement and fever. Active SLE disease has been considered as an independent risk factor for the development of TMA, and without careful and extensive studies, TMA may be neglected in SLE patients with high disease activity.
Secondary antiphospholipid syndrome should be ruled out, and tests for anticardiolipin antibodies (aCL), lupus anticoagulant (LAC) and anti-β2 glycoprotein I (β2GPI) antibodies are recommended.
The pathogenesis of TMA in SLE remains to be elucidated and may be multifactorial. This includes low ADAMTS13 levels, SLE disease activity, infection, drugs, malignant hypertension, pregnancy, etc. SLE disease activity (SLEDAI score >10) can be the major factor for TMA. A study showed that infection was one of the major triggers for the flare-up of SLE disease activity and occurrence of TMA in SLE (Chen MH, et al. Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology (Oxford). 2011;50(4):768-75. [PubMed link]).
TMA in LN can be limited to the kidney and in these cases it is only possible to be diagnosed by biopsy.
See the chapter: Lupus Nephritis of our Tutorial.
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References
- Park MH, Caselman N, Ulmer S, Weitz IC. Complement-mediated thrombotic microangiopathy associated with lupus nephritis. Blood Adv. 2018;2(16):2090-2094. [PubMed link]
- Yue C, Su J, Gao R, Wen Y, Li C, Chen G, Zhang X, Li X. Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus-associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor Profiles. J Rheumatol. 2018 Jul 15. [Epub ahead of print] [PubMed link]
- Pattanashetti N, Anakutti H, Ramachandran R, Rathi M, Sharma A, Nada R, Gupta KL. Effect of Thrombotic Microangiopathy on Clinical Outcomes in Indian Patients With Lupus Nephritis. Kidney Int Rep. 2017;2(5):844-849. [PubMed link]
- Barrera-Vargas A, Rosado-Canto R, Merayo-Chalico J, Arreola-Guerra JM, Mejía-Vilet JM, Correa-Rotter R, Gómez-Martín D, Alcocer-Varela J. Renal Thrombotic Microangiopathy in Proliferative Lupus Nephritis: Risk Factors and Clinical Outcomes: A Case-Control Study. J Clin Rheumatol. 2016;22(5):235-40. [PubMed link]
- Chen MH, Chen MH, Chen WS, Mu-Hsin Chang P, Lee HT, Lin HY, Huang DF. Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology (Oxford). 2011;50(4):768-75. [PubMed link