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Rapidly Progressive Glomerulonephritis

RPGN

Rapidly progressive glomerulonephritis is characterized by the acute, rapid loss of renal function due to severe glomerular damage. The glomerular filtration rate decreases significantly over days, weeks, to months and may result in irreversible renal failure.


Presentation

Patients with rapidly progressive glomerulonephritis may initially present with vague, non-specific symptoms such as body aches and pains, pallor, peripheral edema, lethargy, and/or fatigue [1]. Conversely, patients may present with more renal specific symptoms including hematuria, anemia, oliguria, hypertension, and/or peripheral edema [2]. An elevated serum creatinine and renal insufficiency is almost always a presenting feature. The most frequently occurring prodrome of ANCA-associated vasculitis, including rapidly progressive glomerulonephritis, consists of flulike symptoms (e.g., fever, body aches, loss of appetite, chills, weight loss, malaise) [3]. Following this prodrome, patients may develop abdominal pain, a migratory polyarthropathy, and cutaneous ulcerations and/or nodules. Patients may also present with symptoms of sinusitis, cough, and/or hemoptysis when there is pulmonary involvement [4]. Patients with immune-related rapidly progressive glomerulonephritis, such as anti-GBM disease in which the patient has antibodies directed against the alveolar basement membranes, may present with extra-renal symptoms such as hemoptysis and pulmonary hemorrhage. Subsequently, the pulmonary bleeding may result in anemia, iron deficiency, pallor, and weakness.

Recurrent Oral Ulceration
  • A 44-year-old male has suffered from recurrent oral ulcers and retinal vasculitis developed 2 years ago. He complained of abdominal pain and papulopustular skin lesions. Multiple ulcers were seen on the colon on colonoscopy.[ncbi.nlm.nih.gov]
Pansystolic Murmur
  • A new pansystolic murmur over the apex of the heart was heard on auscultation, and echocardiography clearly showed a vegetation about 0.7-0.9 cm in size on the atrial site of the mitral value.[ncbi.nlm.nih.gov]
Oliguria
  • And in this case, despite oliguria and rapid deterioration of renal function, the renal function recovered partially and continued to be stabilized with a favourable response to hemodialysis and combined system immunosuppressive therapy.[ncbi.nlm.nih.gov]
  • Rapidly progressive ("crescentic") glomerulonephritis is a primary glomerulonephritis, which produces nephritic syndrome (hematuria, oliguria, uremia, hypertension and mild proteinuria).[pathologyatlas.ro]
  • RPGN is dominated by a picture of nephritic syndrome (i.e. hematuria, pyuria, secondary hypertension, oliguria, and azotemia). However, nephritic symptoms progress extremely rapidly in RPGN and can quickly result in acute renal failure.[pathwaymedicine.org]
  • Conversely, patients may present with more renal specific symptoms including hematuria, anemia, oliguria, hypertension, and/or peripheral edema. An elevated serum creatinine and renal insufficiency is almost always a presenting feature.[symptoma.com]
  • L oss of renal function associated with severe oliguria and death from renal failure within week to months. B. Gross Findings : T he kidney is enlarged and pale, often with petechial hemorrhages on the cortical surfaces. C.[pathology.class.kmu.edu.tw]
Smoky Urine
  • Abstract A 19 years old, male patient presented with symptoms of smoky urine for 2 weeks, puffiness of face and diminished urine output for 3 weeks associated with occasional lower abdominal and flank pain.[ncbi.nlm.nih.gov]

Workup

Blood chemistry and serologic tests should be ordered 'stat' on any patient suspected of having rapidly progressive glomerulonephritis. If clinically indicated, a renal biopsy may also be performed.

Blood serum test results will consist of an elevated creatinine level usually exceeding 3 mg/dL, decreased glomerular filtration rate, elevated erythrocyte sedimentation rate, and C-reactive protein [5]. Complete blood count with differential will show an elevated neutrophil count and thrombocytes. A urinalysis will usually show dysmorphic hematuria, casts, and proteinuria.

Serologic testing should include the following: ANCA, anti-glomerular basement membrane antibodies, anti-dsDNA antibody, complement assays, antinuclear antibodies, and others as indicated from the clinical history, examination, and biopsy results [6]. C3 and C4 complement levels are usually either normal or slightly elevated in patients with rapidly progressive glomerulonephritis type I and III. Circulating anti-GBM antibodies are present in type I rapidly progressive glomerulonephritis. Most patients with type III disease are positive for myeloperoxidase (MPO)–ANCA [3].

A kidney biopsy can be used to achieve the diagnosis of various types of rapidly progressive glomerulonephritis (types I, II, III). A microscopic examination of renal tissue will show crescentic glomerulonephritis [7]. Crescent formation is a result of a response to injury to the glomerular capillary walls [8]. The extent of crescent formation is correlated with the disease severity; patients that have crescent formations in less than half of their glomeruli are likely to have a mild disease course and full recovery, whereas those with greater than three-quarters of glomeruli present with advanced renal failure that is refractory to therapy [9].

Chest X-ray and/or chest computed tomography (CT) may be useful for patients with vasculitis and Goodpasture syndrome [7] [10]. Patients may have a normal/negative chest X-ray and a chest CT that shows reticulonodular infiltrates. An abdominal and renal ultrasound can be used to evaluate echogenicity, renal atrophy or enlargement, and rule out obstruction [11].

Creatinine Increased
  • The initial presentation usually is with oliguria, creatinine increase, variable proteinuria, macro or microhematuria, and arterial hypertension. Occasionally there is nephrotic syndrome.[kidneypathology.com]
Chlamydia
  • Abstract Herein we describe a case of a patient with rapidly progressive glomerulonephritis after Chlamydia pneumoniae infection.[ncbi.nlm.nih.gov]

Treatment

  • Several new treatment strategies, such as leukocytapheresis (LCAP) and intravenous immunoglobulin (IVIg), have become available during the past decade and these treatments have made it possible to treat high-risk RPGN patients without inducing serious[ncbi.nlm.nih.gov]
  • This is, to our knowledge, a very rare case of crescentic glomerulonephritis, probably associated with vasculitis during a course of treatment with two different kinds of drugs.[ncbi.nlm.nih.gov]
  • The prompt discontinuation of D-penicillamine and vigorous treatment measures could allow for a good prognosis as in this case.[ncbi.nlm.nih.gov]
  • KEYWORDS: ANCA Associated Vasculitis; Goodpasture’s Syndrome; Plasmapheresis; Treatment[ncbi.nlm.nih.gov]
  • Abstract Plasma exchange has been used for the treatment of rapidly progressive glomerulonephritis (RPGN) for more than 40 years.[ncbi.nlm.nih.gov]

Prognosis

  • Thirteen patients exhibited a good clinical prognosis (68.4%), with 6 exhibiting a poor prognosis (31.6%), 4 of whom progressed to end-stage renal disease (ESRD), one experiencing a relapse and one developing chronic kidney disease.[ncbi.nlm.nih.gov]
  • The prognosis for patients with anti-GBM antibody disease is poor.[ncbi.nlm.nih.gov]
  • CONCLUSION: Early diagnosis was the most important factor for improving the prognosis of RPGN patients.[ncbi.nlm.nih.gov]
  • The prompt discontinuation of D-penicillamine and vigorous treatment measures could allow for a good prognosis as in this case.[ncbi.nlm.nih.gov]
  • Abstract Fibrillary glomerulonephritis (FGN) is a rare disorder with poor renal prognosis. It is a heterogeneous disease associated with significant risk of end-stage renal disease (ESRD).[ncbi.nlm.nih.gov]

Etiology

  • The most common underlying etiologies were post-infectious glomerulonephritis (PIGN) (63.2%) and lupus nephritis (21.1%).[ncbi.nlm.nih.gov]
  • Its etiology and pathogenesis have not been clearly identified. We report a case of a patient presenting with hypertensive crisis, nephrotic range proteinuria, and rapidly progressive glomerulonephritis (RPGN).[ncbi.nlm.nih.gov]
  • We report a 62 year old male patient on azathioprine therapy, 9 years after left cadaveric renal transplantation due to end stage renal disease of unknown etiology, who presented with progressive deterioration in renal function and hematuria.[ncbi.nlm.nih.gov]
  • BACKGROUND: The etiology, prevalence, and prognosis of rapidly progressive glomerulonephritis (RPGN) including renal vasculitis vary among races and periods.[ncbi.nlm.nih.gov]
  • Etiology of rapidly progressive crescentic glomerulonephritis : streptococcal infection, systemic lupus, vasculitis, Goodpasture's syndrome, idiopathic.[pathologyatlas.ro]

Epidemiology

  • At a population level, little is known of the epidemiology and outcome of RPGN. Between 1 April 1986 and 31 March 1996, 141 cases of biopsy proven pauci-immune RPGN were seen in the Wessex region. The records of 128 patients were reviewed.[ncbi.nlm.nih.gov]
  • The epidemiology of rapidly progressive glomerulonephritis according to Hedger, et al., is an incidence rate of 3.9 individuals per million (3.3–4.7) with a 95% confidence intervals. Greenhall, George H. B.; Salama, Alan D. (2015-02-19).[en.wikipedia.org]
  • A "Mini-Epidemic" of anti-glomerular basement membrane disease: Clinical and epidemiological study. Saudi J Kidney Dis Transpl. 2017 Sep-Oct. 28 (5):1057-1063. [Medline]. [Full Text].[emedicine.medscape.com]
  • Epidemiology of renal disease in Romania: a 10 year review of two regional renal biopsy databases. Nephrol Dial Transplant. 2006;21(2):419–24. CrossRef PubMed Google Scholar 19. 20. Sinha A, Puri K, Hari P, Dinda AK, Bagga A.[link.springer.com]
Sex distribution
Age distribution

Pathophysiology

  • Understanding the pathophysiology and complex management of this disease poses challenges to clinicians. CASE REPORT: A 42-year-old woman presented with acute renal and hepatic failure. She had been on PTU for 11 months for Graves' disease.[ncbi.nlm.nih.gov]
  • There are three different pathophysiological mechanisms that can result in RPGN: anti-glomerular basement membrane antibody disease ( Goodpasture syndrome ), immune complex glomerulonephritis (e.g., lupus nephritis ), and glomerulonephritis associated[amboss.com]
  • In the pathophysiology of rapidly progressive glomerulonephritis the antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in cytoplasm of neutrophils.[en.wikipedia.org]
  • Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices.[books.google.com]
  • The pathophysiology of crescents remains poorly understood.[ajkdblog.org]

Prevention

  • Abstract Rapidly progressive glomerulonephritis (RPGN) must be diagnosed and treated quickly to prevent irreversible organ injury.[ncbi.nlm.nih.gov]
  • Prevention & Expectations What can be done to prevent the disease? Depending on the cause, some cases of RPGN may be prevented.[odlarmed.com]
  • Detection of ANCA and autoantibodies (autoAbs) to GBM and dsDNA enables early diagnosis and appropriate treatment of RPGN aiding in preventing end-stage renal disease.Determination of ANCA on neutrophils (ANCA) as well as autoAbs to myeloperoxidase (MPO-ANCA[ncbi.nlm.nih.gov]
  • In glomerulonephritis, transferred iTreg cells did not prevent renal injury or modulate systemic T helper type 1 immune responses.[ncbi.nlm.nih.gov]
  • Early diagnosis and appropriate treatment is mandatory to prevent death and/or renal failure.[ncbi.nlm.nih.gov]

References

Article

  1. Arimura Y, Muso E, Fujimoto S, et al. Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. Clin Exp Nephrol. 2016;20:322-41.
  2. Kouri AM, Andreoli SP. Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis. Pediatr Nephrol. 2016.
  3. Gupta R, Singh L, Sharma A, Bagga A, Agarwal SK, Dinda AK. Crescentic glomerulonephritis: a clinical and histomorphological analysis of 46 cases. Indian J Pathol Microbiol. 2011;54:497-500.
  4. Jennette JC, Olsen JL, Schwartz M, Silva FB, Heptinstall RH.Heptinstall’s pathology of the kidney. Philadelphia: Lippincott Williams & Wilkins. 1999.
  5. Reinhold-Keller E. Diagnostics and therapy of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides. Curr Pharm Des. 2012;18:4537-4
  6. Lerner RA, Glassock RJ, Dixon FJ. The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exp Med 1967;126: 989-1004.
  7. Jennette JC, Olson JL, Schwartz MM, Silva FG. Pauci-immune and ANCA-mediated crescentic glomerulonephritis and vasculitis. In: Heptinstall's Pathology of the Kidney, vol. 1, chapter 14, 6th edition. Lippincott Williams & Wilkins;2007.
  8. Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int. 2003;63:1164.
  9. Baldwin DS, Neugarten J, Feiner HD, Gluck M, Spinowitz B. The existence of a protracted course in crescentic glomerulonephritis. Kidney Int. 1987;31:790.
  10. Phelps RGRees AJ. The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity.Kidney Int.1999;56: 1638-53.
  11. Rowaiye OO, Kusztal M, Klinger M. The kidneys and ANCA-associated vasculitis: from pathogenesis to diagnosis. Clin Kidney J. 2015;8:343-50.

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Last updated: 2018-06-21 17:20