Antiphospholipid syndrome and kidney damage - Treatment

Treatment of renal damage associated with antiphospholipid syndrome is not clearly defined, since there are currently no large controlled comparative studies assessing the effectiveness of different treatment regimens for this pathology.

• In the treatment of patients with secondary antiphospholipid syndrome in the context of systemic lupus erythematosus, glucocorticoids and cytostatic drugs are used in doses determined by the activity of the disease. Suppression of the activity of the underlying disease, as a rule, leads to the disappearance of signs of antiphospholipid syndrome. In primary antiphospholipid syndrome, glucocorticoids and cytostatic drugs are not used.
• Despite the fact that treatment with glucocorticoids and cytostatic drugs leads to normalization of the aPL titer and disappearance of lupus anticoagulant in the blood, it does not eliminate hypercoagulation, and prednisolone even enhances it, which maintains conditions for recurrent thrombosis in different vascular pools, including the renal vascular bed. In this regard, when treating nephropathy associated with antiphospholipid syndrome, it is necessary to prescribe anticoagulants as monotherapy or in combination with antiplatelet agents. By eliminating the cause of renal ischemia (thrombotic occlusion of intrarenal vessels), anticoagulants are able to restore renal blood flow and lead to an improvement in renal function or slow the progression of renal failure, which, however, requires confirmation in the course of studies assessing the clinical effectiveness of both direct and indirect anticoagulants in patients with antiphospholipid syndrome-associated nephropathy.
  • Patients with acute nephropathy associated with antiphospholipid syndrome are indicated for the administration of unfractionated heparin or low molecular weight heparins, but the duration of treatment and doses of the drug have not yet been clearly defined.
  • Due to frequent recurrences of thrombosis in patients with antiphospholipid syndrome (including in intrarenal vessels), after completion of heparin treatment it is advisable to prescribe indirect anticoagulants for prophylactic purposes. Currently, warfarin is considered the drug of choice; its use is also indicated in the case of a combination of nephropathy associated with antiphospholipid syndrome with damage to the central nervous system, heart and skin. In the case of chronic nephropathy associated with antiphospholipid syndrome with slowly progressing renal failure, warfarin can apparently be prescribed without a previous course of direct anticoagulants. The effectiveness of warfarin treatment is monitored using the international normalized ratio (INR), the value of which should be maintained at 2.5-3.0. The therapeutic dose of drugs that allows maintaining the target INR level is 2.5-10 mg/day. The duration of warfarin use is not defined, and the possibility of lifelong treatment cannot be ruled out.
• For the treatment of catastrophic antiphospholipid syndrome, regardless of its nature (primary, secondary), intensive therapy methods are used, including pulse therapy with methylprednisolone and cyclophosphamide, direct anticoagulants (low molecular weight heparins) and plasmapheresis to remove antibodies to phospholipids and mediators of intravascular blood coagulation.

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Prognosis of antiphospholipid syndrome

The prognosis for antiphospholipid syndrome and kidney damage associated with it, in its natural course, is unfavorable: 10-year renal survival is 52%.

Risk factors for chronic renal failure in patients with antiphospholipid syndrome-associated nephropathy in primary and secondary antiphospholipid syndrome are severe arterial hypertension, episodes of transient deterioration in renal function, signs of renal ischemia according to ultrasound Doppler imaging, and morphological changes in renal biopsy specimens (arteriolosclerosis and interstitial fibrosis). Chronic renal failure develops more frequently in patients with antiphospholipid syndrome-associated nephropathy with a history of extrarenal arterial thrombosis. The only factor that has a favorable effect on the prognosis of antiphospholipid syndrome-associated nephropathy is anticoagulant treatment at any stage of the disease. Anticoagulant therapy helps to increase 10-year renal survival from 52 to 98%.