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2.1
Rapidly Progressive Glomerulonephritis
Rapidly Progressive Inflammation of the Renal Corpuscles

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.

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WIKIDATA, CC BY-SA 3.0
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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.

Cardiovascular

  • 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]

Jaw & Teeth

  • 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]

Skin

  • Subcutaneous Nodule

    […] diagnosed as rheumatoid arthritis  Skin: subcutaneous nodules (granulomas) on the elbow, purpura, various others (see cutaneous vasculitis)  Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex  Heart, gastrointestinal [slideshare.net]

Urogenital

  • 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]

    Progressive decline in GFR over weeks to months (more subacute presentation) -Rapid loss of renal function over weeks to months -Often accompanied with oliguria or anuria -Urinalysis shows dysmorphic RBCs, RBC casts, with or without proteinuria Rapidly [studyblue.com]

  • Dark Urine

    Patient complained of dark urine and was found to have proteinuria and hematuria. Creatinine was rising despite hydration, so a kidney biopsy was planned and vasculitis work up was done. It came back positive for ANA and pANCA. [journal.chestnet.org]

  • Smoky Urine

    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].

Serum

  • 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]

Colonoscopy

  • Multiple Ulcerations

    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]

Treatment

We describe a rare case that developed a rapidly progressive glomerulonephritis twice in a 69-year-old man during a course of treatment, first with allopurinol and then with piperacillin. [ncbi.nlm.nih.gov]

The goals of treatment include: To remove anti-GBM autoantibodies from the plasma of the patient To suppress antibody production by inhibiting immune cell activity To inhibit the autoimmune response Treatment of Goodpasture syndrome The treatment objectives [news-medical.net]

Prognosis

The prognosis for patients with anti-GBM antibody disease is poor. [ncbi.nlm.nih.gov]

The overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival. [radiopaedia.org]

Etiology

The most common underlying etiologies were post-infectious glomerulonephritis (PIGN) (63.2%) and lupus nephritis (21.1%). [ncbi.nlm.nih.gov]

Abstract Summary: The clinical course and outcome of rapidly progressive glomerulonephritis (RPGN) of variable etiology are not well defined in children. [nature.com]

Outcomes were unsatisfactory irrespective of etiology, with progressive renal impairment in 86% cases. [indianpediatrics.net]

Epidemiology

Goodpasture syndrome Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Goodpasture syndrome from other Diseases Epidemiology and Demographics Risk Factors Natural History, Complications [wikidoc.org]

The case presentation is followed by a brief overview of the epidemiology of AKI in nephrotic syndrome as well as a discussion of its risk factors and potential mechanisms involved. [ncbi.nlm.nih.gov]

Epidemiology[edit] The incidence rate of rapidly progressive glomerulonephritis is approximately 3.9 individuals per million.[11] References[edit] ^ RESERVED, INSERM US14-- ALL RIGHTS. [en.wikipedia.org]

Epidemiology The incidence of Goodpasture syndrome shows a bimodal distribution with one peak among young men in their late-twenties and another peak among elderly men and women after their sixtieth year of age. [pathwaymedicine.org]

Pathophysiology

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 with [amboss.com]

Goodpasture syndrome Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Goodpasture syndrome from other Diseases Epidemiology and Demographics Risk Factors Natural History, Complications [wikidoc.org]

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]

Updated: Sep 25, 2008 ^ "rapidly progressive glomerulonephritis" at Dorland's Medical Dictionary ^ "Rapidly Progressive Glomerulonephritis: Background, Pathophysiology, Epidemiology". 2018-04-05. ^ Morita T, Suzuki Y, Churg J (1973). [en.wikipedia.org]

Prevention

Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 Goodpasture syndrome On the Web Most recent articles Most cited articles Review articles CME Programs Powerpoint slides Images American Roentgen [wikidoc.org]

Abstract Rapidly progressive glomerulonephritis (RPGN) must be diagnosed and treated quickly to prevent irreversible organ injury. [ncbi.nlm.nih.gov]

In this way it can prevent the production of both autoantibodies and the cytotoxic T cells which are crucial in the cell-mediated immune component of Goodpasture syndrome. [news-medical.net]

Prevention & Expectations What can be done to prevent the disease? Depending on the cause, some cases of RPGN may be prevented. [odlarmed.com]

Prevention Can I prevent Goodpasture syndrome? You may not be able to prevent Goodpasture syndrome. But you can lower your risk by avoiding tobacco and chemicals that may trigger the disorder. [my.clevelandclinic.org]

References

  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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