genitourinary disorders
Immunoglobulin A Nephropathy
Treatment of IgA Nephropathy
 receptor blockers (ARBs) for Hypertension , serum creatinine A sodium-glucose cotransporter 2 (SGLT2) inhibitor may be added for persistent proteinuria despite angiotensin inhibition Corticosteroids for progressive disease, including increasing proteinuria, especially into the nephrotic range, and increasing serum creatinine level for proliferative injury or Rapidly Progressive Glomerulonephritis (RPGN) None Kidney Transplantation in advanced disease Normotensive patients with intact renal function (serum creatinine<1.2 mg/dL [106.08 micromol/L]) and only mild proteinuria (<0.5 g/day) usually are not treated beyond angiotensin inhibition (with an ACE inhibitor or ARB) and an SGLT2 inhibitor. Patients with renal insufficiency or more severe proteinuria and hematuria are usually offered corticosteroids, which ideally should be started before significant renal insufficiency develops. genotype for the ACE gene may be at greater risk of disease progression but may also be more likely to respond to ACE inhibitors or ARBs. For patients with hypertension, ACE inhibitors or ARBs are the antihypertensives of choice even for relatively mild Chronic Kidney Disease . An Sodium-glucose co-transporter 2 inhibitors may also be used for patients with proteinuria secondary to IgA nephropathy that has not improved with ACE inhibitors or ARBs. In a pre-specified subgroup analysis of patients with IgA nephropathy, SGLT2 inhibitors (which improve outcomes in patients with proteinuria due to diabetic kidney disease) reduced the risk of chronic kidney disease progression ( after at least 3 months of supportive therapy), corticosteroids have been shown to slow the rate of progression to kidney failure ( Treatment references ). Higher doses corticosteroid regimens are associated with more serious adverse events (eg, infection requiring hospitalization). There is no consensus among experts regarding the optimal corticosteroid regimen. One protocol usesmethylprednisolone1 g IV once a day for 3 days at the beginning of months 1, 3, and 5 plusprednisone0.5 mg/kg orally every other day for 6 months. Another regimen usesprednisonebeginning 1 mg/kg orally once a day with dose gradually tapered over 6 months. Because of the risk of adverse effects, corticosteroids should probably be reserved for patients with any of the following: Worsening or persistent proteinuria (>1 g/day), especially if in the nephrotic range despite maximal ACE inhibitor or ARB therapy Increasing serum creatinine level Combinations of IV corticosteroids andcyclophosphamideplus oralprednisoneare used for severe disease, such as proliferative or crescentic (rapidly progressive) nephropathy. Evidence formycophenolatemofetil is conflicting; it should not be used as first-line treatment. None of these medications, however, prevents recurrence in transplant patients. Immunosuppressive therapy should also be avoided in patients with advanced fibrotic kidney disease, which is not reversible. Although other interventions have been tried to lower IgA overproduction and to inhibit mesangial proliferation, data supporting any of these are limited or absent, and none can be recommended for routine treatment. These interventions include elimination of gluten, dairy products, eggs, and meat from the diet; tonsillectomy; and immune globulin 1 g/kg IV 2 days a month for 3 months followed by immune globulin 0.35 mL/kg of 16.5% solution IM every 2 weeks for 6 months all theoretically reduce IgA production.Heparin,dipyridamole, and statins are just a few examples of in vitro mesangial cell inhibitors. For patients who progress to end-stage kidney disease, Kidney Transplantation is preferred over dialysis because of improved long-term disease-free survival. The condition recurs in approximately 30% of graft recipients ( 1.Wheeler DC, Toto RD, Stefánsson BV, et al: A pre-specified analysis of the DAPA-CKD trial demonstrates the effects ofdapagliflozinon major adverse kidney events in patients with IgA nephropathy.Kidney Int100(1):215-224, 2021. doi: 10.1016/j.kint.2021.03.033 2.Lv J, Wong MG, Hladunewich MA, et al: Effect of oralmethylprednisoloneon decline in kidney function or kidney failure in patients with IgA nephropathy: The TESTING randomized clinical trial.JAMA327(19):1888-1898, 2022. doi: 10.1001/jama.2022.5368 3.Jäger C, Stampf S, Molyneux K, et al: Recurrence of IgA nephropathy after kidney transplantation: Experience from the Swiss transplant cohort study.BMC Nephrol23(1):178, 2022. doi: 10.1186/s12882-022-02802-x 