IgA Nephropathy Clinical Presentation

Updated: Oct 11, 2017
  • Author: Mona Brake, MD; Chief Editor: Vecihi Batuman, MD, FASN  more...
  • Print
Presentation

History

Two common presentations of patients with IgA nephropathy are episodic gross hematuria and persistent microscopic hematuria. Recurrent macroscopic hematuria, usually associated with an upper respiratory tract infection, or, less often, gastroenteritis is the most frequent clinical presentation and is observed in 40-50% of presenting patients. In 30-40% of patients, the disease is asymptomatic, with erythrocytes (RBCs), RBC casts, and proteinuria discovered on urinalysis. Patients with IgA nephropathy can also present with acute kidney injury or chronic kidney disease.

Gross hematuria

Many patients present with episodes of recurrent macroscopic hematuria, as follows:

  • 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
  • Urine is usually brown rather than red, and clots are unusual

Episodes of gross hematuria in IgA nephropathy have been associated with a variety of other infections, as follows:

  • Urinary tract infections
  • Pneumonia
  • Staphylococcal sepsis
  • Staphylococcal osteomyelitis
  • Acute gastroenteritis
  • Influenza
  • Infectious mononucleosis

Gross hematuria has also occurred after the following:

  • Tonsillectomy
  • Vaccinations
  • Strenuous physical exercise
  • Trauma

Episodic, grossly visible hematuria is a more common presentation in younger people, whereas that of abnormal urine sediment is more frequent in older individuals. Between episodes of gross hematuria, many patients have persistent microhematuria, proteinuria, or both.

Next:

Physical

Physical examination findings in patients with IgA nephropathy are usually unremarkable. A minority of patients have hypertension. More commonly, however, hypertension manifests as the course of the disease lengthens or when patients develop chronic kidney disease and end-stage renal disease (ESRD).

Previous
Next:

Causes

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 up to a 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 disease

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. [12] 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. [13] Additionally, increased frequency of specific HLA groups has been reported in some patients.

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. [14] 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. [15]

Previous