IgA Nephropathy Clinical Presentation

Updated: Dec 24, 2021
  • Author: Sohail Abdul Salim, MD, FASN, FACP; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Patients with Iga nephropathy may be asymptomatic, with persistent microscopic hematuria and proteinuria and often hypertension. This presentation occurs mostly in adults. Impairment of renal function can occur in such cases, and remission is uncommon. 

Symptomatic presentations in patients with IgA nephropathy include the following:

  • Episodic gross hematuria
  • Rapidly progressive glomerulonephritis –  This is sometimes seen as a late presentation; these patients  may progress to needing renal replacement therapy rapidly.
  • Proteinuria- less than 3 gms/day. (More common)
  • Nephrotic syndrome – More than 3.5 gms of proteinuria with edema, hypoalbuminemia, hypertension, and hyperlipidemia.
  • Chronic renal failure 

Episodic gross hematuria from IgA nephropathy has the following features:

  • Eighty percent of these episodes are associated with upper respiratory tract infections, mainly acute pharyngotonsillitis; this synchronous association of pharyngitis and macroscopic hematuria has been dubbed synpharyngitic nephritis.
  • Gross hematuria usually appears simultaneously or within the first 48-72 hours after the infection begins; persists less than 3 days; and, in about a third of patients, is accompanied by loin pain, presumably due to renal capsular swelling
  • Seen mostly in children and young adults
  • These patients have a high likelihood of remission.




Physical examination findings in patients with IgA nephropathy are usually unremarkable. A minority of patients present with hypertension. More commonly, however, hypertension manifests later in the course of the disease or when patients develop chronic kidney disease and end-stage renal disease (ESRD). Nephrotic syndrome could manifest as edema in lower extremities.



Most cases of IgA nephropathy are idiopathic, but the onset or exacerbation of the disease is often preceded by a respiratory tract infection. Association with some bacteria, such as Haemophilus parainfluenzae, has been reported. A variety of other disorders have also been linked with IgA nephropathy, as discussed below.

Cirrhosis and other liver diseases

Glomerular IgA deposition is a common finding in cirrhosis, occurring in more than one-third of patients. Liver disease is accompanied by impaired removal of IgA-containing complexes by the Kupffer cells, predisposing patients to IgA deposition in the kidney.

Glomerular IgA deposits are common in advanced liver disease, but most adults have no clinical signs of glomerular disease, whereas up to 30% of children may have asymptomatic hematuria or proteinuria. Those abnormalities usually resolve after successful liver transplantation.

Gluten enteropathy (celiac disease)

Glomerular IgA deposition occurs in up to a third of patients with gluten enteropathy. Most of these patients have no clinical manifestations of the disease. However, IgA nephropathy and gluten hypersensitivity are associated, and withdrawal of gluten from the diet of these patients has resulted in clinical and immunological improvement of the renal disease.

HIV infection

IgA nephropathy has been reported in patients with HIV infection, both whites and blacks, despite the rarity of typical IgA nephropathy in the black population. [23] Clinically, patients have hematuria, proteinuria, and, possibly, renal insufficiency.

Histologically, findings range from mesangial proliferative glomerulonephritis to collapsing glomerulosclerosis with mesangial IgA deposits. Several patients have had circulating immune complexes containing IgA antibodies against viral proteins.

Familial IgA nephropathy

Although IgA nephropathy is usually a sporadic disease, data suggest that genetic factors are important in susceptibility to development of mesangial glomerulonephritis. Several cases of familial disease have been reported in Italy and the United States, and an autosomal dominant form has been linked to band 6q22-23. [24] Additionally, increased frequency of specific HLA groups (HLA-DRB1 and HLA-DQB1) has been reported in some populations. [25] A study of genetic risk for IgA nephropathy in Han Chinese identified three non-HLA gene regions (FBXL21, CCR6, and STAT3) and one HLA gene region (GABBR1) with suggestive significance; further analysis identified five novel susceptibility genes, TGFBI, CCR6, STAT3, GABBR1, and CFB, that may be involved in IgA nephropathy. [26]

Ai and colleagues reported increased risk for IgA nephropathy in association with low copy number of the α-defensin gene (DEFA1A3). Low total copy numbers also showed significant association with renal dysfunction in patients with IgA nephropathy. [27] Single-nucleotide polymorphisms (SNPs) of the enabled homolog gene (ENAH) have been associated with increased susceptibility to childhood IgA nephropathy, as well as to the development of proteinuria and gross hematuria, and pathological progression in children with the disease. [28]