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.

• 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]

• 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]

• 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]

  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]

  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]

• 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]

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]

• Leukocytes Decreased

  If the fiber diameter is too large, the amount and rate of adsorption of leukocytes decrease; if too small, the amount of platelet adsorption increases. [google.ch]

• Multiple Ulcerations

  Multiple ulcers were seen on the colon on colonoscopy. Routine renal work-up revealed heavy proteinuria and hematuria. Renal biopsy demonstrated crescentic glomerulonephritis. [ncbi.nlm.nih.gov]

• Cerebral Angiitis

  She also developed seizures and MRA of her head revealed cerebral infarct, with findings suggestive of cerebral angiitis. She did not recover and expired 20 days after presentation. [ncbi.nlm.nih.gov]

  […] in a Case of Rapidly Progressive Glomerulonephritis. ( 26301224 ) Mavani G.P....Rosenstock J. 2015 34 A fatal case of propylthiouracil-induced ANCA-associated vasculitis resulting in rapidly progressive glomerulonephritis, acute hepatic failure, and cerebral [malacards.org]

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]

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 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]

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]

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]

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]

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]

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]

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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.