IgA nephropathy

Last updated: July 21, 2023

Summarytoggle arrow icon

IgA nephropathy (Berger disease) is the most common primary glomerulonephritis worldwide. It most frequently affects males in the second to third decades of life. Clinical manifestations are usually triggered by upper respiratory tract or gastrointestinal infections and include gross hematuria and flank pain. In some cases, it may present as rapidly progressive glomerulonephritis (RPGN). Urinalysis of asymptomatic patients often shows persistent microhematuria and minor proteinuria, while more severe cases may manifest with recurrent episodes of nephritic syndrome. A kidney biopsy is indicated in patients with signs of severe or progressive disease to make a definitive diagnosis. Treatment consists of measures to slow the progression of the disease (e.g., ACE inhibitors) as well as immunosuppressive therapy in more severe cases. Even with the appropriate treatment, up to 50% of patients progress to end-stage renal disease within 20–25 years.

Epidemiologytoggle arrow icon

IgA nephropathy is the most common primary glomerulonephritis in adults. [1]

  • Peak incidence: second to third decades of life [2]
  • Sex: > (2:1) [3]
  • Ethnicity: more common in the Asian population (worldwide) [4]

Epidemiological data refers to the US, unless otherwise specified.

Pathophysiologytoggle arrow icon

Clinical featurestoggle arrow icon

The course of the disease is highly variable and can manifest in the following forms:

IgA nephropathy and IgA vasculitis are both IgA-mediated vasculitides triggered by a mucosal infection. IgA vasculitis most commonly occurs in children < 10 years of age and affects multiple organ systems (palpable purpura, abdominal pain, arthralgia). IgA nephropathy is limited to the kidneys and typically affects adults.


Diagnosticstoggle arrow icon

Diagnosis is based on clinical presentation and laboratory results. In some cases, renal biopsy may be indicated to confirm the diagnosis.

The renal manifestation of IgA vasculitis is pathologically the same as IgA nephropathy.

Differential diagnosestoggle arrow icon

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

Referencestoggle arrow icon

  1. Penfold RS, Prendecki M, McAdoo S, Tam FW. Primary IgA nephropathy: current challenges and future prospects.. International journal of nephrology and renovascular disease. 2018; 11: p.137-148.doi: 10.2147/IJNRD.S129227 . | Open in Read by QxMD
  2. Barratt J, Feehally J. Immunoglobulin A Nephropathy and Related Disorders. Elsevier ; 2014: p. 185-192
  3. Ichida K, Hosoyamada M, Hosoya T, Endou H. Primary Metabolic and Renal Hyperuricemia. Elsevier ; 2009: p. 651-660
  4. Prakash S, Kanjanabuch T, Austin PC, et al. Continental variations in IgA nephropathy among Asians.. Clin Nephrol. 2008; 70 (5): p.377-84.doi: 10.5414/cnp70377 . | Open in Read by QxMD
  5. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  6. Jean-Claude Davin. Henoch-Schönlein Purpura Nephritis: Pathophysiology, Treatment, and Future Strategy. CJASN. 2011; 6 (4): p.679-689.doi: 10.2215/CJN.06710810 . | Open in Read by QxMD
  7. Suzuki K, Honda K, Tanabe K, Toma H, Nihei H, Yamaguchi Y.. Incidence of latent mesangial IgA deposition in renal allograft donors in Japan.. Kidney Int. 2003; 63 (6): p.2286-94.doi: 10.1046/j.1523-1755.63.6s.2.x . | Open in Read by QxMD
  8. Geddes CC, Rauta V, Gronhagen-Riska C, Bartosik LP, Jardine AG, Ibels LS, Pei Y, Cattran DC.. A tricontinental view of IgA nephropathy.. Oxford Academic. 2003; 18 (8): p.1541-8.doi: 10.1093/ndt/gfg207 . | Open in Read by QxMD
  9. Waldherr R, Rambausek M, Duncker WD, Ritz E.. Frequency of mesangial IgA deposits in a non-selected autopsy series.. Oxford Academic. 1989; 4 (11): p.943-6.doi: 10.1093/ndt/4.11.943 . | Open in Read by QxMD
  10. Lewis EJ, Carpenter CB, Schur PH.. Serum complement component levels in human glomerulonephritis.. Ann Intern Med. 1971; 75 (4): p.555-60.
  11. Greenspan SL , Harris ST, Bone H et al. Bisphosphonates: Safety and Efficacy in the Treatment and Prevention of Osteoporosis. Am Fam Physician. 2000; 1 (61): p.2731-2736.
  12. Yeo SC, Cheung CK, Barratt J. New insights into the pathogenesis of IgA nephropathy. Pediatr Nephrol. 2017; 33 (5): p.763-777.doi: 10.1007/s00467-017-3699-z . | Open in Read by QxMD

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