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.

• Weakness

  Subsequently, the pulmonary bleeding may result in anemia, iron deficiency, pallor, and weakness. Blood chemistry and serologic tests should be ordered 'stat' on any patient suspected of having rapidly progressive glomerulonephritis. [symptoma.com]

  Rapidly progressive glomerulonephritis in most cases manifested by weakness, hypertension, fever, involvement of lower airways, infection. Renal biopsies were performed in 18 (78.26%) cases, lung biopsy was made for one patient. [publications.lsmuni.lt]

  The symptoms of Goodpasture syndrome may initially include fatigue, nausea, vomiting, and weakness. [niddk.nih.gov]

  Symptoms can include: headaches nausea and vomiting sleepiness weakness on one side of your body memory loss problems with speech and language personality and mood changes muscle weakness double vision or blurred vision loss of appetite seizures Keep [healthline.com]

  The first signs of Goodpasture syndrome usually include tiredness, nausea, vomiting and weakness. The disease can get worse very quickly and can cause coughing and make it hard for you to catch your breath. [kidneyfund.org]

• Sepsis

  Sclerosis  APLA RARELY – Occult viscera sepsis, Sarcoidosis, Obstructive Nephropathy 10. Clinical Approach? 11. [slideshare.net]

  […] glomerulonephritis Goodpasture disease: glomerulonephritis alone (30%,) pulmonary symptoms alone (10%,) Anti-GBM disease is most precise term for both entities Type II hypersensitivity causing linear IG deposition in glomerular and alveolar BM Triggered by: Sepsis [wikem.org]

  The factors associated with increased in-hospital mortality were age older than 70 years, sepsis, the development of respiratory failure, circulatory failure, renal failure, and liver failure, whereas the factors associated with decreased in-hospital [mdpi.com]

  In patients with infection, sepsis, or shock, partial or asymmetric PRES was only occasionally encountered. [ajnr.org]

  Due to concurrent sepsis and/or cardiovascular collapse on arrival in the renal unit, five (4%) patients had no immunosuppression, all died. These were all severely affected cases. [doi.org]

• Streptococcal Infection

  Etiology of rapidly progressive crescentic glomerulonephritis : streptococcal infection, systemic lupus, vasculitis, Goodpasture's syndrome, idiopathic. [pathologyatlas.ro]

  Clinical, bacteriologic, and serologic studies revealed no evidence of previous streptococcal infection, systemic disease, or significant drug ingestion. [annals.org]

  Plasmapheresis or high doses of corticosteroids may lead to recovery of renal function. rap·id·ly pro·gress·ive glo·mer·u·lo·ne·phri·tis glomerulonephritis usually presenting insidiously, without preceding streptococcal infection, with increasing renal [medical-dictionary.thefreedictionary.com]

• Lymphadenopathy

  […] resolve in several days to give reticular opacities in the same distribution complete radiographic resolution is usually seen within 2-3 weeks CT ground glass and airspace opacities that progress to reticular "crazy paving" pattern over a few weeks hilar lymphadenopathy [radiopaedia.org]

  It is not uncommon for patients who develop cutaneous AE to have concomitantly or subsequently multiorgan involvement, such as lymphadenopathy or other manifestations of autoimmunity [40,62,63]. [neurologia.com]

• Hemoptysis

  Patients may also present with symptoms of sinusitis, cough, and/or hemoptysis when there is pulmonary involvement. [symptoma.com]

  If the patient presents with hemoptysis, pulmonary-renal syndrome should be suspected. RPGN requires rapid diagnosis and immediate initiation of immunosuppressive therapy to prevent irreversible kidney damage. [amboss.com]

  Abstract Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. [unboundmedicine.com]

  respiratory failure or pulmonary hemorrhage Renal: glomerulonephritis Hematuria, proteinuria, RBC casts Symptoms are often rapidly progressive Differential Diagnosis Hemoptysis Infectious Bronchitis Pneumonia Lung abscess Tuberculosis Plague Aspergilloma [wikem.org]

  Symptoms are dyspnea, cough, fatigue, hemoptysis, and hematuria. Goodpasture syndrome is suspected in patients with hemoptysis or hematuria and is confirmed by the presence of anti-GBM antibodies in the blood or in a renal biopsy specimen. [msdmanuals.com]

• Rhinitis

  Granulomatous vasculitis Necrotizing vasculitis (no granulomas) Connective tissue disease Clinical findings Hemoptysis, cough, dyspnea History of chronic sinusitis, nasal ulcers, perforation of the nasal septum Palpable purpura Peripheral neuropathy Asthma, rhinitis [amboss.com]

  She had been in excellent health, except for a two-day episode of rhinitis and headache one week before admission. She denied chills, fever, sputum production, or previous pulmonary or cardiovascular complaints. [jamanetwork.com]

   The early (prodromal) stage is marked by airway inflammation; almost all patients experience asthma and/or allergic rhinitis.  The second stage is characterized by abnormally high numbers of eosinophils (hypereosinophilia), which causes tissue damage [slideshare.net]

• Saddle Nose

  nose deformity -Respiratory symptoms include hoarseness, cough, wheezing, stridor, dyspnea, and hemoptysis -Dermal findings include leukocytoclastic angiitis in the lower extremities Granulomatosis with polyangiitis (Wegener's granulomatosis) 1. [studyblue.com]

• Ulcer

  In the remaining 5 cases of gastric ulcers and 2 cases of esophageal ulcer with underlying MA or 4 cases of duodenal ulcers, in whom assumed the bleeding form of oozing from the marginal zone of ulcers. [ncbi.nlm.nih.gov]

  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. [symptoma.com]

  Oral ulcer or butterfly rash is indicative of lupus Skin petechiae may indicate lupus or vasculitis Evidence of atheroembolic disease Upper Respiratory tract involvement – sinuses* RS – signs of asthma/alveolar hmghe CNS- peripheral neuropathy 13 [slideshare.net]

  […] membrane Possibly triggered by viral infections, lymphoma Granulomatous vasculitis Granulomatous vasculitis Necrotizing vasculitis (no granulomas) Connective tissue disease Clinical findings Hemoptysis, cough, dyspnea History of chronic sinusitis, nasal ulcers [amboss.com]

• Asthenia

  She reported asthenia, but no weight loss, cigarette smoking (20 pack-years) that was not stopped afterwards, no exposure to toxic chemicals. [bmcnephrol.biomedcentral.com]

• Oliguria

  Rapidly progressive ("crescentic") glomerulonephritis is a primary glomerulonephritis, which produces nephritic syndrome (hematuria, oliguria, uremia, hypertension and mild proteinuria). [pathologyatlas.ro]

  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]

  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]

  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]

  It is characterised clinically by rapid deterioration of renal function, nephritic syndrome and severe oliguria. Untreated, it can lead to death within weeks to months. [renalpathology.wordpress.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]

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]

Even though anti-GBM serology tests are often delayed, treatment for anti-GBM antibody disease should be delayed until the tests are recieved and the diagnosis is definitive False, time is crucial in patients with anti-GBM antibody disease and treatment [studyblue.com]

Progression to renal failure is rapid without treatment.. [geekymedics.com]

Several new treatment strategies, such as leukocytaphereis (LCAP) and intravenous immunoglobulin (IVIg), have become available and these treatments have made it possible to treat high-risk RPGN patients without inducing serious immunosuppressive states [genome.jp]

The prompt discontinuation of D-penicillamine and vigorous treatment measures could allow for a good prognosis as in this case. [ncbi.nlm.nih.gov]

Specialty Nephrology Symptoms Hematuria[2] Types Type I, II and III[3] Diagnostic method Serum analysis[2] Treatment Corticosteroids and Cyclophosphamide[4] Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized [en.wikipedia.org]

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

Poor prognosis – most progress to end stage renal failure Rapidly progressive glomerulonephritis (crescentic) Also known as crescentic glomerulonephritis. Carries poor prognosis – rapid progression to kidney failure over weeks. [geekymedics.com]

The prognosis is severe, with rapid and irreversible evolution to renal failure. Etiology of rapidly progressive crescentic glomerulonephritis : streptococcal infection, systemic lupus, vasculitis, Goodpasture's syndrome, idiopathic. [pathologyatlas.ro]

Etiology of rapidly progressive crescentic glomerulonephritis : streptococcal infection, systemic lupus, vasculitis, Goodpasture's syndrome, idiopathic. [pathologyatlas.ro]

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]

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]

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]

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

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

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]

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