Up to 80% of individuals with Goodpasture syndrome exhibit both respiratory and renal symptoms. Acute presentation of symptoms occurs more frequently than chronic symptoms. An individual may initially complain of loss of appetite, fatigue and weakness. Renal involvement is accompanied by blood in urine, difficulty while urination, nausea, pallor and edema of extremities. Pulmonary involvement includes dry cough with shortness of breath and coughing out blood [26].

• Rapidly Progressive Glomerulonephritis

  Only those with rapidly progressive glomerulonephritis should be regarded as examples of Goodpasture’s syndrome. [histopathology-india.net]

  Cardinal Findings: Approximately 75% of those with Goodpasture syndrome have both pulmonary and renal involvement; the remainder have only rapidly progressive glomerulonephritis. Isolated pulmonary disease is rare. [rheumaknowledgy.com]

  […] reaction type II disease that is characterized by glomerulonephritis located in kidney and hemorrhaging located in lung anti-glomerular basement membrane disease Rapidly progressive glomerulonephritis with pulmonary hemorrhage Anti-GBM syndrome Glomerulonephritis [wikidata.org]

  Affected individuals present with symptoms of rapidly progressive glomerulonephritis Rapidly Progressive Glomerulonephritis Rapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function [lecturio.com]

  It presents with rapidly progressive renal dysfunction often combined with lung haemorrhage. [bestpractice.bmj.com]

• Fatigue

  Other common symptoms include Dyspnea Cough Fatigue Fever Weight loss Hematuria Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [merckmanuals.com]

  Other common symptoms include Cough Dyspnea Fatigue Fever Hematuria Weight loss Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [msdmanuals.com]

  (see Fatigue, [[Fatigue]]) Fever (see Fever, [[Fever]]) Malaise Treatment Plasmapheresis (see Plasmapheresis, [[Plasmapheresis]]) Indications Diffuse Alveolar Hemorrhage (DAH) (see Diffuse Alveolar Hemorrhage, [[Diffuse Alveolar Hemorrhage]]) Clinical [mdnxs.com]

• Anemia

  Diagnostic Tests: Laboratory studies often reveal an anemia that may be related to both anemia of chronic disease and iron deficiency, depending on the severity and chronicity of the pulmonary hemorrhage. Patients may also be hypoxic. [rheumaknowledgy.com]

  Anemia from pulmonary bleeding is usual. Radiographic examination reveals diffuse bilateral alveolar filling. The diagnosis is made on the basis of renal biopsy. [histopathology-india.net]

  (see Anemia, [[Anemia]]) Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests, [[Pulmonary Function Tests]]) Increased DLCO : during active diffuse alveolar hemorrhage Increase of 30% above baseline indicates acute bleed May precede abnormal [mdnxs.com]

  Blood testing often shows anemia. Laboratory tests reveal the characteristic antibodies in the blood. Doctors usually remove a small piece of kidney tissue (kidney biopsy) for analysis. [msdmanuals.com]

  A blood smear revealed normochromic microcytic anemia The platelet count was 250,000, and clotting time was eight seconds. [pubs.rsna.org]

• Fever

  Fever, fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. [lecturio.com]

  The patient has intermittent fever and cough for 45 days and oliguria for 6 days. [ncbi.nlm.nih.gov]

  (see Fever, [[Fever]]) Malaise Treatment Plasmapheresis (see Plasmapheresis, [[Plasmapheresis]]) Indications Diffuse Alveolar Hemorrhage (DAH) (see Diffuse Alveolar Hemorrhage, [[Diffuse Alveolar Hemorrhage]]) Clinical Efficacy Retrospective Review of [mdnxs.com]

  She had fever, cough, severe dyspnea and lately hemoptysis and severe hypoxia with bilateral alveolar opacity in chest imaging, with no response to broad spectrum antibiotic. [caspjim.com]

  She denied chills, fever, sputum production, or previous pulmonary or cardiovascular complaints. She had been treated for a urinary tract infection with antibiotics at 11 years of age. [jamanetwork.com]

• Leg Edema

  […] in the legs (edema = swelling) Tests to diagnose the syndrome Urinalysis. [quizlet.com]

• Cough

  Pulmonary involvement includes dry cough with shortness of breath and coughing out blood. A physical exam is first conducted assess any signs of respiratory or renal discomfort. [symptoma.com]

  Other common symptoms include Dyspnea Cough Fatigue Fever Weight loss Hematuria Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [merckmanuals.com]

  Common symptoms of Goodpasture syndrome include Shortness of breath Cough Fever Unplanned weight loss Fatigue Blood in the urine Coughing up blood (hemoptysis) Fatigue and paleness (pallor) may be symptoms of anemia that results from blood loss. [msdmanuals.com]

  As the disease advances, the lung symptoms are: Dry cough Coughing up blood Difficulty breathing If the kidneys are affected, symptoms include: Burning sensation during urination Blood in the urine High blood pressure Edema (swollen body parts) Low urine [davita.com]

• Hemoptysis

  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. [merckmanuals.com]

  Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. [ncbi.nlm.nih.gov]

  She had an episode of hemoptysis in 2001. She was labeled as recurrent acute respiratory distress syndrome (ARDS) in 2002. [jmedicalcasereports.biomedcentral.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]

• Dyspnea

  Other common symptoms include Dyspnea Cough Fatigue Fever Weight loss Hematuria Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [merckmanuals.com]

  Other common symptoms include Cough Dyspnea Fatigue Fever Hematuria Weight loss Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [msdmanuals.com]

  Although the patients may be adults of any age, they are typically young men and present with hemoptysis, dyspnea, and acute renal disease. Either the renal symptoms or the pulmonary symptoms may come first. [histopathology-india.net]

  Other symptoms include cough and dyspnea. Smoking history may be associated with pulmonary hemorrhage. Renal manifestations generally lag behind pulmonary signs and symptoms. [rheumaknowledgy.com]

• Tachypnea

  Tachypnea and basal râles were heard bilaterally. The liver was palpated two fingerbreadths below the right costal margin. Blood pressure was 140/80 mm. Hg and the pulse rate was 80/min. [pubs.rsna.org]

  […] manifestations include hematuria, edema, high blood pressure and eventually uremia Significant anemia may result from persistent intrapulmonary bleeding Physical Examination Physical examination findings in patients with anti-GBM disease include the following: Tachypnea [emedicine.medscape.com]

  Pulmonary hemorrhage can progress rapidly, and patients may develop worsening respiratory distress, hypoxia, and tachypnea during the ED course.10,11 Patients may have systemic symptoms including fevers, chills, malaise, and arthralgias. [emdocs.net]

  […] respiratory failure may occur Renal manifestations are rapidly progressive glomerulonephritis that may lead to azotemia and volume overload Arthralgias Physical examination Physical examination findings in patients with anti-GBM disease include the following: Tachypnea [catatanseorangdoktor.wordpress.com]

• Rales

  […] is more suggestive of an alternative or simultaneous different vasculitis.6 Patients may also have signs of iron deficiency anemia from persistent alveolar bleeding.10 Physical exam can show hypertension, tachypnea, respiratory distress, inspiratory rales [emdocs.net]

  […] hemosiderin The alveolar haemorrhage syndrome is an acute condition, which requires diagnosis and rapid and efficient treatment for the multiple clinical manifestations (dysp-noea, coughing up blood, auscultation of bilateral basal crepitant and subcrepitant rales [pneumologia.eu]

• Nausea

  Common Symptoms Bleeding in the lungs, fatigue, nausea, breathing issues, vomiting, pale skin, burning sensations when urinating, blood in the urine, protein in the urine, coughing, foamy urine, swelling of the legs, and high blood pressure. [autoimmuneinstitute.org]

  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]

  If the kidney disease is very severe, patients may notice that they are passing only small amounts of urine and may have nausea and vomiting as well. Treatments include medications and a procedure called plasmapheresis. [my.clevelandclinic.org]

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

• Vomiting

  Common Symptoms Bleeding in the lungs, fatigue, nausea, breathing issues, vomiting, pale skin, burning sensations when urinating, blood in the urine, protein in the urine, coughing, foamy urine, swelling of the legs, and high blood pressure. [autoimmuneinstitute.org]

  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]

  If the kidney disease is very severe, patients may notice that they are passing only small amounts of urine and may have nausea and vomiting as well. Treatments include medications and a procedure called plasmapheresis. [my.clevelandclinic.org]

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

• Kidney Failure

  Coexisting Diseases and Conditions Hemoptysis, dyspnea, hypertension, arthritis, anemia, acute kidney failure, chronic kidney disease, heart disease, and respiratory failure. Sources Article Sources and Footnotes Goodpasture Syndrome. (n.d.-a). [autoimmuneinstitute.org]

  As a result, people with Goodpasture syndrome can develop kidney failure and bleeding in the lungs, which causes them to cough up blood. In some people, antibodies attack only the kidneys. [ghr.nlm.nih.gov]

  Most of the time, the damage to your lungs is not permanent, but the damage to your kidneys is permanent. If your kidney damage leads to kidney failure, you will need dialysis or a kidney transplant to survive. [kidneyfund.org]

• Hematuria

  Other common symptoms include Dyspnea Cough Fatigue Fever Weight loss Hematuria Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [merckmanuals.com]

  and glomerulonephritis (hematuria) along with circulating anti-glomerular basement membrane (anti-GBM) antibodies [ 1 ]. [jmedicalcasereports.biomedcentral.com]

  Other common symptoms include Cough Dyspnea Fatigue Fever Hematuria Weight loss Up to 40% of patients have gross hematuria, although pulmonary hemorrhage may precede renal manifestations by weeks to years. [msdmanuals.com]

• Oliguria

  The patient has intermittent fever and cough for 45 days and oliguria for 6 days. [ncbi.nlm.nih.gov]

  Renal Symptomology Inflammation of the glomerulus initiates the pathogenesis of a nephritic syndrome with symptomology of hematuria, pyuria, secondary hypertension, oliguria, and azotemia (See nephritic syndrome page). [pathwaymedicine.org]

  The results are proteinuria, hemauria and oliguria. Immunopathogenesis[edit | edit source] The autoantibodies are mainly of the IgG1 and IgG4 subclasses, but in one third of the cases IgA and IgM autoantibodies are also present. [wikilectures.eu]

  Presence of oliguria or decrease in frequency and amount of urine, greater than 50% crescents on renal biopsy, fibrosis and reliance on dialysis tend to have poorer prognosis. [symptoma.com]

• Headache

  Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by headache, altered consciousness, seizures, and cortical blindness. [ncbi.nlm.nih.gov]

  It involves headache, altered state of consciousness, visual abnormalities (blurred vision, scotoma, cortical blindness) and tonic-clonic seizures. [revistanefrologia.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]

• Lethargy

  Initial symptoms may include: fatigue, weakness, or lethargy nausea or vomiting loss of appetite unhealthy, pale appearance When the disease affects your lungs, the following symptoms may occur: dry cough coughing up blood (hemoptysis) shortness of breath [healthline.com]

  Symptoms Early symptoms include general body aches and pains, fatigue, weakness or lethargy, pale skin, nausea, lack of appetite, and shortness of breath. [vasculitisfoundation.org]

  When severe, patient can demonstrate symptoms of uremia, such as nausea, vomiting, fatigue, confusion, lethargy, and/or coma.3,9 Pulmonary symptoms are often seen at initial presentation with hemoptysis ranging from minimal to massive leading to respiratory [emdocs.net]

A physical exam is first conducted assess any signs of respiratory or renal discomfort. Urinalysis reveals abnormal finding such as presence of red blood cells and protein in the urine.

An important diagnostic test to determine evidence of the syndrome is testing the serum for anti-glomerular basement membrane antibodies. A positive antibody test confirms Goodpasture syndrome.

Other tests are used to confirm symptoms such as tests for serum BUN and creatnine levels, chest X-rays, arterial blood gas analysis are carried out. Kidney biopsies are preferred over lung biopsies to assist in diagnosis as signs of crescent formation, glomerular membrane damage, interstitial fibrosis and tubular atrophy can determine extent of renal injury [27] [28].

• Pulmonary Infiltrate

  A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. [ncbi.nlm.nih.gov]

  […] by diffuse parenchymal infiltrates and findings suggesting renal disease. [merckmanuals.com]

  Pulmonary manifestations include dyspnea, cough, sometimes hemoptysis, pulmonary infiltrates on chest x-ray, and an increased carbon monoxide diffusing capacity (DLCO) due to the presence of hemoglobin in the alveoli. [ispub.com]

• HLA-DRw2

  Individuals carrying alleles HLA-DRW2, HLA-DRB4 and HLA-DRB1 within the Major Histocompatibility Complex (MHC) locus are susceptible to events that trigger autoantibody activation. [symptoma.com]

  […] subtypes, especially HLA DRw2, DRB1 1501 AND DRB1 1502 If one twin develops the syndrome it invariably manifests in the second twin ANCA and Anti-GBM Disease There is a consistently strong association between the presence of antineutrophil cytoplasmic [news-medical.net]

  There is an inherited predisposition to this syndrome (HLA-DRw2 is associated with anti-GBM disease). Incidence The incidence is estimated at approximately 1:100,000 people worldwide. It is most often present in males at the age of 20 years. [accesspediatrics.mhmedical.com]

  Genetic predisposition with presence of HLA-DRw2. HLA-B7 relates to more severe anti-GBM nephritis and is seen to be more frequent. Exposure to hydrocarbons and organic solvents. Smoking. Exposure to metal dusts. [epainassist.com]

• HLA-DR1

  Anti-GBM disease is seen less often with HLA-DR1 and HLA-DR7; both have strong negative associations, and both are highly protective. Environmental insults Smoking is closely linked with hemoptysis. [emedicine.com]

Plasmapharesis is carried out to purify the blood and remove anti-GPDM antibodies from the plasma [30].

Steroids may be recommended to reduce renal or pulmonary inflammation [31]. Symptomatic treatment includes blood pressure medication, diuretics etc.

In extreme cases, dialysis and/or renal transplantation may be recommended [32].

The prognosis is good if disease is detected and treated early. Individuals with pulmonary hemorrhage tend to get treated quicker than those with asymptomatic renal injury.

Presence of oliguria or decrease in frequency and amount of urine, greater than 50% crescents on renal biopsy, fibrosis and reliance on dialysis tend to have poorer prognosis [29].

Goodpasture syndrome is characterized by the presence of autoantibodies that target the α3, α4 and α5 chain of non collagenous 1 (NC1) domain of type IV collagen found within the glomerular and pulmonary basement membrane layers [6] [7] [8].

Individuals carrying alleles HLA-DRW2, HLA-DRB4 and HLA-DRB1 within the Major Histocompatibility Complex (MHC) locus are susceptible to events that trigger autoantibody activation [9] [10] [11]. The events implicated include inhalation of cigarette smoke, metal dust or drugs such as cocaine, exposure to hydrocarbons and infectious diseases such as influenza A2 that also affect the respiratory system [12] [13] [14] [15] [16].

Goodpasture syndrome is rare in occurrence with approximately one case reported per two million individuals in Europe3. A study conducted on patients with end stage renal disease (ESRD) in Australia and New Zealand noted that 0.8 percent individuals suffered from Goodpasture syndrome [4].

Demographically, young and elderly adult Caucasian and Asian males have a higher incidence of developing this syndrome5. Younger males show symptoms of lung hemorrhage prior to renal disease while older and elderly males will initially present with renal disease [4] [6].

The basement membrane layer serves as a foundation for epithelial cell organization. It is formed by the polymerization of agrin, laminin and Type IV collagen fibers to various glycoprotein molecules [17]. In the renal glomerulus, the basement membrane is flanked by fenestrated endothelial cells of the glomerular capillary and podocytes. This layer provides mechanical support to the capillary wall, provide a base for receptor ligand interaction and assists in the ultrafiltration process of the kidney [18]. Three novel collagen IV chains α3,α4 and α5 first identified by scientists in 1987, serve as epitopes for binding of autoantibodies.

Researchers hypothesize that in the presence of triggering factors such as cigarette smoke or hydrocarbon exposure in susceptible individuals damage the basement membrane. This results in a conformational change of the collagen IV molecule that then exposes the α3,α4 and α5 epitopes to both CD4+ reactive T cells and B cells [19] [20] [21] [22]. These antibodies are highly specific and have been observed in serum samples of patients suspected of Goodpasture’s disease [23].

Downstream effects that occur after antibody target interaction results in activation of complements, cytokines and proteases interferes with the glomerular filtration process resulting in glomerulonephritis, identified by crescent formation and proteinuria. Continued antibody assault will result in migration of macrophages and neutrophils into the kidney leading to interstitial nephritis and fibrosis in kidney. 2-D electrophoresis and western blot analysis of collagen domains isolated from human alveolar basement membrane also showed a positive reaction to anti-glomerular basement membrane antibodies [23] [24].

The alveolar basement membrane is impervious to anti-glomerular basement membrane antibodies under normal circumstances. However, environmental factors damage the alveolar basement layer especially in susceptible individuals and expose the epitopes to antibody binding [25].

There are no guidelines for prevention of Goodpasture syndrome.

Goodpasture syndrome is an autoimmune condition that affects both the pulmonary and the renal system as evidenced by glomerulonephritis and pulmonary hemorrhage [1].

It is named after Dr. Ernest Goodpasture (1886-1960), an American physician and pathologist. During the influenza pandemic in 1919, he observed that some infected patients suffered acute symptoms of renal failure along with hemoptysis or coughing up blood [2].

Goodpasture syndrome is a rare autoimmune disease. Individuals with acute renal failure and pulmonary hemorrhage should be evaluated for this condition. Presence of anti-glomerular basement membrane antibodies in sera of affected patients is diagnostically relevant.

Plasmapharesis and steroids are usually recommended for treatment. The prognosis is good when diagnosed and treated promptly. However, delay in treatment will lead to complications such as renal failure and death.

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