Antiphospholipid syndrome: What is it and how does it affect the kidneys?
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by a pathological tendency in which the blood has a tendency to clot too easily.
The patient who suffers from APS may experience:
- Thrombosis in veins or arteries
- Successive miscarriages before the 10th week of pregnancy or unexplained fetal death after the 10th week of pregnancy or premature labor during or before the 34th week of pregnancy
- Presence of anticardiolipin antibody syndrome or lupus anticoagulant in the patient’s blood
It is distinguished in:
- primary (when occurs in people who do not have another autoimmune condition)
- secondary (when developed in patients with systemic lupus erythematosus or other autoimmune disease).
Is APS common?
It is the most common acquired coagulation disorder, with a frequency of 10-15% of the thrombotic episodes resulting from it. Antiphospholipid syndrome occurs most commonly in adult women than in men.
How do we diagnose APS and which doctor is monitoring it?
Suspicion and then diagnosis comes with the clinical manifestation of thrombosis and / or obstetric complications, by the existence of antiphospholipid antibodies in the patient's blood and when this test is positive twice within 12 weeks. The rheumatologist along with the hematologist monitor it while the nephrologist is involved only in the case of renal involvement.
Is there a renal involvement in the APS?
Yes, a clot may be developed in the kidney vessels (large or small). In large vessels, renal involvement in APS shows hypertension, acute or chronic renal insufficiency, macroscopic hematuria, renal area pain. Renal involvement may occur due to APS nephropathy with a major feauture of small vessel thrombosis in the glomerulus (Kidney filter). It occurs with various levels of hypertension, albuminuria, gradual - slow decline in renal function. But it can also appear with a clinical picture such as a rapid reduction of renal function, high hypertension and severe albuminuria.
Kidney disease can occur in APS only with thrombosis?
No, patients with primary APS may also develop membranous glomerulonephritis, a disease of minor lesions.
How frequent is the renal involvement in APS?
Approximately 25% of patients with primary APS show renal involvement as well.
How is it treated?
With anticoagulation. With oral anticoagulants (e.g. warfarin) with ultimate goal INR 2-3 (INR is the way we measure the intensity of anticoagulation).
Which patient class should be checked for APS?
Patients with SLE, patients that have experienceda clot, women with multiple miscarriages, young people, adults (<50 years) with stroke, patients showing segmental thinning of the cortex.
By the Scientific Director Aristides Paraskevopoulos
1: Marcantoni C, Emmanuele C, Scolari F. Renal involvement in primary antiphospholipid syndrome. J Nephrol. 2016 Aug;29(4):507-15. doi: 10.1007/s40620-016-0317-2. Epub 2016 May 19. Review. PubMed PMID: 27198137.
2: Sciascia S, Baldovino S, Schreiber K, Solfietti L, Roccatello D. Antiphospholipid Syndrome and the Kidney. Semin Nephrol. 2015 Sep;35(5):478-86.doi: 10.1016/j.semnephrol.2015.08.009. Review. PubMed PMID: 26573550.
3: Abeysekera RA, Wazil AW, Nanayakkara N, Ratnatunga NV, Fernando KM, Thinnarachchi J. Primary antiphospholipid syndrome presenting as antiphospholipid syndrome nephropathy: a case report. J Med Case Rep. 2015 Jan 29;9:28. doi: 10.1186/1752-1947-9-28. PubMed PMID: 25630456; PubMed Central PMCID: PMC4406029.
4: Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014 Jan;3(1):9-17. doi: 10.12860/jnp.2014.03. Epub 2014 Jan 1 Review.
5: Sciascia S, Cuadrado MJ, Khamashta M, Roccatello D. Renal involvement in antiphospholipid syndrome. Nat Rev Nephrol. 2014 May;10(5):279-89. doi: 10.1038/nrneph.2014.38. Epub 2014 Mar 18. Review.
6: Pons-Estel GJ, Cervera R. Renal involvement in antiphospholipid syndrome. CurrRheumatol Rep. 2014 Feb;16(2):397. doi: 10.1007/s11926-013-0397-0. Review. PubMed.
7: Erre GL, Bosincu L, Faedda R, Fenu P, Masala A, Sanna M, Taras L, Longu MG, Piras M, Soro G, Satta AE, Passiu G. Antiphospholipid syndrome nephropathy (APSN) in patients with lupus nephritis: a retrospective clinical and renal pathology study. Rheumatol Int. 2014 Apr;34(4):535-41. PubMed PMID: 24232504.