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For further information, see CMDT Part 24-14: Nephritic Spectrum Glomerular Diseases

KEY FEATURES

  • Results from glomerular mesangial deposition of immune complexes made up of aberrantly glycosylated immunoglobulin A (IgA) and immunoglobulin G (IgG) autoantibodies against these abnormal molecules

  • Can either be a primary (renal-limited) disease

  • Or can be secondary to

    • Cirrhosis

    • Celiac disease

    • HIV infection

    • Cytomegalovirus infection

  • Most common primary glomerular disease worldwide, particularly in Asia

  • Usually occurs in children and young adults

  • Affects males 2–3 times more often than females

CLINICAL FINDINGS

  • Gross hematuria, frequently associated with a mucosal viral infection, often an upper respiratory tract infection (URI)

  • Urine becomes red or cola-colored 1–2 days after onset of URI

  • Can present clinically anywhere along the nephritic spectrum from asymptomatic microscopic hematuria to rapidly progressive glomerulonephritis

  • Rarely, a nephrotic syndrome can be present

DIAGNOSIS

  • Proteinuria: minimal to nephrotic range

  • Glomerular hematuria: microscopic is common; macroscopic (gross) can occur after mucosal infection

  • Positive IgA staining on kidney biopsy

  • Serum complement levels usually normal

  • No serologic tests aid in diagnosis

  • Renal biopsy

    • Light microscopy shows focal glomerulonephritis with mesangial proliferation

    • Immunofluorescence demonstrates diffuse mesangial IgA and C3 deposits

TREATMENT

  • Patients at low risk for progression (no hypertension, normal glomerular filtration rate [GFR], minimal proteinuria) can be monitored expectantly

  • Angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB)

    • Recommended for patients at higher risk for progression (proteinuria > 1.0 g/d, decreased GFR, hypertension, or any combination of these)

    • Therapy should be titrated to reduce proteinuria to < 0.5 g/day and to control blood pressure to 120/70 mm Hg per practice guidelines

  • SGLT2-inhibitors may be added to standard care in proteinuric patients

  • Conflicting data regarding efficacy of corticosteroids for reducing proteinuria and slowing progression

    • May be considered for patients with GFR > 30mL/min/1.73m2 and persistent proteinuria >1 g/d despite maximal ACE inhibitor or ARB for at least 3 months

  • Hydroxychloroquine

    • Efficacy has only been demonstrated in Japanese populations to date

    • Has shown promise in Chinese populations but not in White populations

  • Cyclophosphamide and corticosteroid therapy should be considered for the rare patient with a rapidly progressive clinical course with diffuse crescent formation on biopsy

  • ∼33% of patients experience spontaneous remission

  • Progression to end-stage kidney disease occurs in 20–40%

  • Proteinuria > 1 g/d is most unfavorable prognostic indicator; others include

    • Hypertension

    • Reduced GFR on presentation

    • Glomerulosclerosis or tubulointerstitial fibrosis on biopsy

  • Kidney transplantation

    • An excellent option for patients with end-stage kidney disease

    • However, recurrent disease develops in 30% of patients by 5–10 years posttransplant

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