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IgA nephropathy

Last updated: October 16, 2019

Summary

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.

Epidemiology

  • IgA nephropathy is the most common primary glomerulonephritis in adults.
  • Peak incidence: second to third decades of life
  • Sex: > (2:1)
  • Ethnicity: more common in the Asian population (worldwide)

Epidemiological data refers to the US, unless otherwise specified.

Pathophysiology

Clinical features

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

  • Asymptomatic
  • Recurring episodes of:
  • Can progress to RPGN and/or nephrotic syndrome (< 10% of patients)
  • Up to 50% of patients progress to end-stage renal disease within 20–25 years.

IgA nephropathy and Henoch-Schonlein purpura (HSP) are both IgA-mediated vasculitides triggered by a mucosal infection. HSP 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.

References:[2][3][4][5][6][7][8]

Diagnostics

Diagnosis is based on clinical presentation and laboratory results. In some cases, renal biopsy may be indicated to confirm the diagnosis. [9]

The renal manifestation of Henoch-Schönlein purpura is pathologically the same as IgA nephropathy.

Differential diagnoses

The differential diagnoses listed here are not exhaustive.

Treatment

References

  1. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  2. 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
  3. 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
  4. 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
  5. 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
  6. Lewis EJ, Carpenter CB, Schur PH.. Serum complement component levels in human glomerulonephritis.. Ann Intern Med. 1971; 75 (4): p.555-60.
  7. Chee Kay Cheung, PhD, MRCPJonathan Barratt, PhD, FRCP. Clinical presentation and diagnosis of IgA nephropathy. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-presentation-and-diagnosis-of-iga-nephropathy.Last updated: December 19, 2017. Accessed: June 4, 2019.
  8. Mohammad Ilyas, MD, FAAP, Richard Neiberger, MD, PhD. Pediatric IgA Nephropathy Workup. In: Craig B Langman, MD, Pediatric IgA Nephropathy Workup. New York, NY: WebMD. https://emedicine.medscape.com/article/981516-workup. Updated: March 20, 2017. Accessed: June 4, 2019.
  9. Fischer C. Master the Boards USMLE Step 2 CK. Kaplan Publishing ; 2017
  10. 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.
  11. Goldman L, Schafer AI. Goldman-Cecil Medicine, 25th Edition. Elsevier ; 2016