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ANCA-Associated Renal Disease

Pauci-Immune Crescentic Glomerulonephritis

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is ANCA-Associated Renal Disease?

Pauci-immune (minimal immune deposits) necrotizing crescentic glomerulonephritis.

Three main subtypes: GPA (PR3-ANCA, granulomatous + necrotizing), MPA (MPO-ANCA, non-granulomatous), EGPA (asthma + eosinophilia + vasculitis).

Rapidly progressive renal failure (RPGN) is hallmark; can lead to ESRD without treatment.

Pulmonary-renal syndrome possible (DAH + RPGN).

Symptoms

Hematuria (microscopic to macroscopic), proteinuria.
Rapidly rising serum creatinine (over days to weeks).
Hypertension, oliguria/anuria in advanced disease.
Constitutional symptoms: fever, weight loss, fatigue, arthralgia.
Upper respiratory tract: sinusitis, saddle nose, epistaxis (GPA).
Lower respiratory tract: hemoptysis, nodules, alveolar hemorrhage.
Skin: purpura, livedo reticularis, ulcers.
Peripheral neuropathy (mononeuritis multiplex).

Risk Factors

Older age (peak 65-74 years).
Caucasian ancestry (higher GPA prevalence in northern Europe).
Genetic: HLA-DPB1 (PR3-ANCA), HLA-DQ (MPO-ANCA).
Silica exposure, certain drugs (hydralazine, propylthiouracil, levamisole-cocaine).
Female predominance for MPO-ANCA disease.

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • New-onset rapidly rising creatinine.
  • Hematuria with red blood cell casts.
  • Pulmonary symptoms (hemoptysis) + renal abnormalities.
  • Persistent sinusitis with bloody discharge.
  • Suspected systemic vasculitis with multi-organ involvement.

Treatment Methods

01
Induction: high-dose corticosteroids (methylprednisolone pulses 500-1000 mg/day x 3 + prednisolone) + cyclophosphamide OR rituximab.
02
Rituximab (375 mg/m² weekly x 4 or 1g x 2) preferred in young, fertility concerns, relapsing disease.
03
Plasma exchange (PEX) for severe alveolar hemorrhage or dialysis-dependent renal failure (PEXIVAS controversial benefit).
04
Avacopan (C5a receptor antagonist) for steroid-sparing in induction (ADVOCATE trial).
05
Maintenance: rituximab (every 4-6 months) or azathioprine + low-dose prednisolone.
06
Treatment duration: 24-48 months minimum; longer for relapsing disease.
07
Prophylaxis: TMP-SMX (PCP), bone protection, vaccines.
08
Monitor ANCA titers, CRP, renal function; biopsy if relapse suspected.

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.