Goodpasture syndrome anti glomerular basement membrane disease. Medical search. Frequent questions

Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating anti-glomerular basement membrane (anti-GBM) antibodies. (medscape.com)
The antiglomerular basement membrane antibodies were strongly positive. (medscape.com)
The discovery of anti-GBM antibodies led to the understanding of the pathogenesis of Goodpasture syndrome. (medscape.com)
This binding of antibodies can be visualized as the linear deposition of immunoglobulin along the glomerular basement membrane and, less commonly, the alveolar basement membranes, by direct immunofluorescent techniques. (medscape.com)
Goodpasture-like syndrome associated with anti-myeloperoxidase antibodies following penicillamine treatment. (edu.pl)
The treatment of choice is a combination of plasmapheresis to remove the circulating anti-glomerular basement membrane (anti-GBM) antibodies and immunosuppression with glucocorticoids and cytotoxic agents to inhibit further autoantibody formation. (medscape.com)
Goodpasture syndrome is an autoimmune disorder characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis in association with antibodies to alveolar and glomerular basement membrane (GBM). (rheumaknowledgy.com)
The pathogenesis of Goodpasture's syndrome is related to anti-GBM antibodies. (rheumaknowledgy.com)
The specific antigenic epitopes recognized by anti-GBM antibodies were recently shown to be present on the a3 chain of type IV collagen. (rheumaknowledgy.com)
Tests for serum anti-GBM antibodies are most commonly used to diagnose Goodpasture syndrome because of their widespread availability and high sensitivity and specificity. (rheumaknowledgy.com)
In contrast to some other pulmonary renal syndromes, such as SLE, production of anti-GBM antibodies is usually self-limited. (rheumaknowledgy.com)
Goodpasture's name has been used in a more specific clinical condition known as Goodpasture disease, which is the pulmonary renal syndrome specifically associated with anti-glomerular basement membrane (anti-GBM) antibodies. (medscape.com)
[ 1 ] These anti-GBM antibodies produce a characteristic linear deposition along the GBM, one way in which Goodpasture syndrome is differentiated from Wegener granulomatosis. (medscape.com)
Because pulmonary renal syndrome is discussed extensively elsewhere (see Wegener Granulomatosis), this article focuses on the specific form of this syndrome associated with anti-GBM antibodies. (medscape.com)
The discovery of the role of anti-GBM antibodies by Lerner et al in 1967 helped provide both a better understanding of the pathogenesis for this specific form of pulmonary renal syndrome and a more rational approach to treatment. (medscape.com)
Anti-GBM antibodies interact with the GBM glycoproteins, almost exclusively the epitope of the noncollagenous domain (NC1) of the α3 chain of type IV collagen. (medscape.com)
Anti-GBM disease is the result of antibodies direct against antigens in the glomerular basement membrane. (surgpath4u.com)
The anti-GBM antibodies can also cross-react with basement membranes in other locations such as the lung. (surgpath4u.com)
When antibodies react with lung alveolar and glomerular basement membranes, it results in Goodpasture syndrome. (surgpath4u.com)
Of note, up to 1/3 of patients with anti-GBM disease have antineutrophil cytoplasmic antibodies (ANCA), which usually associated with pauci-immune crescentic glomerulonephritis such as Wegener granulomatosis or microscopic polyangiitis. (surgpath4u.com)
We are presenting a case of renal failure with anti-GBM and p-ANCA antibodies positive. (dokumen.tips)
Patients with dual antibodies are con- sidered to be a vasculitis-variant of anti-GBM antibody nephritis. (dokumen.tips)
We recommend that patients suspected with pulmonary-renal syndrome should be checked for anti-GBM and p-ANCA antibodies, should undergo renal biopsy and should should have close long term follow up to watch for recurrence. (dokumen.tips)
Goodpasture syndrome is a severe disease of the lungs and the kidneys caused by antibodies to the alpha3(IV) collagen chains. (medlineplus.gov)
Antibodies are immune system proteins that normally attack foreign substances such as bacteria or viruses, but in people with Goodpasture syndrome, they target alpha3(IV) collagen chains. (medlineplus.gov)
The antibodies cause inflammation when they attach (bind) to the basement membranes of blood vessels in the air sacs (alveoli) of the lungs and filtering units (glomeruli) of the kidneys. (medlineplus.gov)
70% of primary cases are associated with anti-PLA2R antibodies. (shiken.ai)
5. Glomerulonephritis associated with Anti-glomerular basement membrane antibodies and advancing renal failure or pulmonary hemorrhage (N01.0 - N01.9, N03.8) . (qualchoice.com)
A combination of renal and lung involvement may suggest Goodpasture's syndrome due to the presence of antibodies to the glomerular basement membrane (GBM). (mft.nhs.uk)
This finding suggested an abnormality in type IV collagen, against which the anti-GBM antibodies are directed. (abcmedicalnotes.com)
These diseases are designated as antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). (musculoskeletalkey.com)
Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. (musculoskeletalkey.com)
9. Which of the following antibodies are produced almost exclusively in MALTs (Mucosa Associated Lymphoid Tissues) due to the high concentration of TGF-Beta and thus more likely to be secreted across mucosal membranes? (mockdocs.org)

Goodpasture syndrome is an eponym that has been used to describe the clinical entity of diffuse pulmonary hemorrhage (as seen in the images below) and acute or rapidly progressive glomerulonephritis. (medscape.com)
Goodpasture's syndrome (pulmonary haemorrhage associated with glomerulonephritis). (medscape.com)
The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. (medscape.com)
The evidence of circulating autoantibodies, a typical histological appearance of the kidney biopsy with finding of the crescent glomerulonephritis and clinical presentation of nephritic syndrome play an important role in diagnosis. (bvsalud.org)
Our case report describes a case of a young male with anti-GBM nephritis that presents as rapidly progressive glomerulonephritis (RPGN) with progression to dialysis-dependent renal failure. (bvsalud.org)
Idiopathic Immune Complex Glomerulonephritis, IgA Nephropathy, Rapidly Progressive Glomerulonephritis with Heart Failure (Diseases in which the glomerulus of the kidney is damaged. (askdrshah.com)
Goodpasture syndrome (GS) is the clinical entity of acute glomerulonephritis and pulmonary alveolar hemorrhage, and the term Goodpasture syndrome is used interchangeably with pulmonary renal syndrome. (medscape.com)
Anti-GBM disease is defined as the triad of glomerulonephritis (usually rapidly progressive or crescentic), pulmonary hemorrhage, and anti-GBM antibody formation. (medscape.com)
On the basis of this clinical report, Goodpasture's name is often linked to the pulmonary renal syndrome of alveolar hemorrhage and necrotizing and proliferative glomerulonephritis, although vasculitis and not anti-GBM disease is believed to be the cause of the pulmonary renal syndrome in Goodpasture's original patient. (medscape.com)
Anti-GBM antibody-induced nephritis manifests as rapidly progressive (crescentic) glomerulonephritis. (surgpath4u.com)
We reviewed the literature of cases and studies on cresenteric glomerulonephritis with anti-GBM and p-ANCA positive patients. (dokumen.tips)
Under the same conditions, kidney diseases characteristic of membranoproliferative glomerulonephritis (MPGN) or poststreptococcal glomerulonephritis (PSGN) also may be considered. (medscape.com)
In addition, normal complement levels suggest kidney diseases such as immune complex disease, idiopathic rapidly progressive glomerulonephritis, and immunoglobulin G (IgG) or immunoglobulin A (IgA) nephropathy. (medscape.com)
Nephritic syndrome is less commonly used and typically refers to severe and acute presentations of glomerulonephritis with hypertension and reduced urine output. (shiken.ai)
Anti-GBM disease presents with rapidly progressive glomerulonephritis (GN) with or without pulmonary hemorrhage. (arkanalabs.com)
Anti-glomerular basement membrane (GBM) nephritis is clinically and pathologically one of the most aggressive form of glomerulonephritis. (arkanalabs.com)
Serum immunoglobulins may be low in poorly selective proteinuric forms of nephrotic syndrome (focal glomerulonephritis). (mft.nhs.uk)
The disease characteristics of AS mainly include progressive glomerulonephritis, renal failure, sensorineural deafness, and specific ocular abnormalities (lenticular nucleus and macular spots). (researchsquare.com)
Historically, pauci-immune glomerulonephritis has been described as a form of glomerulonephritis with no evidence of linear immunoglobulin deposition (type I glomerulonephritis, as in Goodpasture disease) or immune complex deposition (type II glomerulonephritis, as in lupus nephritis). (musculoskeletalkey.com)
The glomerular lesion in patients with systemic and renal-limited ANCA-associated diseases is identical, that is, crescentic glomerulonephritis characterized by necrotizing inflammation and paucity of immune deposits. (musculoskeletalkey.com)
Glomerulonephritis is a collection of glomerular diseases mediated by different immunologic pathogenic mechanisms, resulting in varied clinical presentation and therapeutic outcomes. (basicmedicalkey.com)
In the absence of specific and effective therapy for many types of glomerulonephritis, supportive treatments for edema, hypertension, hyperlipidemia, and intravascular thrombosis play important roles in reducing the complications associated with the disease. (basicmedicalkey.com)
This chapter provides an overview of the primary causes of glomerulonephritis with a focus on their etiology, the pathophysiologic mechanisms responsible for glomerular injury, and the clinical presentation of the eight predominant types of glomerulonephritis. (basicmedicalkey.com)

As an immune suppressor it is used in nephrotic syndrome, granulomatosis with polyangiitis, and following organ transplant, among other conditions. (wikipedia.org)
The atypical sign of the case was development of nephrotic syndrome with volume-dependent hypertension. (bvsalud.org)
With the qualitative estimation of proteinuria, determination of high-molecular-weight (HMW) protein (eg, fractional excretion of IgG [FEIgG]) and low-molecular-weight (LMW) protein (eg, alpha-1-microglobulin), may help predict the clinical outcome and may help in guiding steroid and immunosuppressive therapy, especially in patients with primary glomerular diseases with nephrotic syndrome. (medscape.com)
Nephrotic syndrome and nephritic syndrome are terms describing a group of symptoms caused by different diseases. (shiken.ai)
Nephrotic syndrome is an important clinical entity and a well-defined group of symptoms. (shiken.ai)
The only exception is focal segmental glomerulosclerosis (FSGS), a type of nephrotic syndrome defined by focal and segmental glomerulosclerosis on histology. (shiken.ai)
Nephrotic syndrome is typically caused by diseases which damage the filtration barrier at the glomerular basement membrane and is defined by a triad of proteinuria, hypoalbuminemia and oedema. (shiken.ai)
Hypoalbuminemia is caused by loss of albumin in the urine, usually compensated by the liver in healthy individuals, but unable to do so in nephrotic syndrome. (shiken.ai)
Oedema is thought to be related to either hypoalbuminemia or excess salt and water retention in the kidney triggered by nephrotic syndrome, presenting with peripheral oedema. (shiken.ai)
The commonest cause of nephrotic syndrome in adults. (shiken.ai)

Cyclophosphamide is also used to treat minimal change disease, severe rheumatoid arthritis, granulomatosis with polyangiitis, Goodpasture syndrome and multiple sclerosis. (wikipedia.org)
Other vascular conditions, like granulomatosis with polyangiitis , also known as Wegener granulomatosis (i.e., a condition that causes inflammation of blood vessels), or anti-glomerular basement membrane disease, otherwise known as Goodpasture syndrome (i.e., an autoimmune disease that can cause inflammation of the alveolar membranes), can involve the lungs and cause hemoptysis . (osmosis.org)

Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture's disease (anti-GBM). (edu.pl)
Dammacco F, Battaglia S, Gesualdo L, Racanelli V. Goodpasture's disease: a report of ten cases and a review of the literature. (edu.pl)
Goodpasture's syndrome: a clinical update. (edu.pl)
Cutting edge issues in Goodpasture's disease. (medscape.com)
Alport's syndrome, Goodpasture's syndrome, and type IV collagen. (rheumaknowledgy.com)
We report the findings of lung biopsies from five patients with anti-basement membrane antibody-associated Goodpasture's syndrome (ABMA-GS). (elsevierpure.com)
Goodpasture's disease and anti-glomerular basement membrane nephritis (anti-GBM nephritis) are rare autoimmune small vessel vasculitis predominantly affecting young men. (bvsalud.org)
Goodpasture's disease plays an important part in differential diagnosis of pulmonary - renal syndrome. (bvsalud.org)
Thus it seems unlikely that there would be a relationship between exposure and the development of autoimmune disease (ie, Goodpasture's disease) and subsequent end stage renal disease except under conditions resulting in frank pulmonary injury. (europa.eu)

2015 ). AS remains a genetic disease that is often missed or misdiagnosed and its pathophysiology is still not fully understood, although some studies have reported that it may be related to chronic inflammation and metabolic dysfunction(Warady et al. (researchsquare.com)
Diabetes mellitus is an important secondary cause of glomerular injury, and a thorough discussion of the pathophysiology and management of this condition can be found in Chapter 57 . (basicmedicalkey.com)

A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires' Disease. (edu.pl)
Pulmonary Renal syndrome (PRS), in a simple language, can be understood as a condition that affects both lungs and kidneys. (askdrshah.com)
How is Pulmonary Renal Syndrome diagnosed? (askdrshah.com)
Homoeopathy offers a supportive role in the treatment of Pulmonary Renal Syndrome in addition to conventional treatment. (askdrshah.com)
Contact us today to know how Homoeopathy can help your Pulmonary Renal Syndrome. (askdrshah.com)

Various other pulmonary/renal syndromes , such as SLE , cryoglobulinemia , and several types of vasculitis, must be distinguished. (rheumaknowledgy.com)
The presence of ANCA in patients with anti-GBM disease usually indicates that there is small vessel vasculitis beyond the kidney and lung (Rosai). (surgpath4u.com)
Awareness of atypi- cal presentations, the risk of recurrence, and overlap between ANCA vasculitis and anti-GBM nephritis is critical for early diagnosis, appropriate treatment, and improved renal out- come in these patients. (dokumen.tips)
Herein, we present a 57 years old Caucasian female with anti-GBM nephritis who presented with vasculitis symptoms and additionally had positive p- ANCA titers. (dokumen.tips)
Microscopic polyangiitis (MPA) is a necrotizing vasculitis without granulomatous inflammation that predominantly affects small vessels (ie, capillaries, venules, or arterioles) and can present with pulmonary capillaritis or in the context of interstitial lung disease [ 3 ]. (medilib.ir)
It is either the renal manifestation of Wegener's granulomatosis, microscopic polyangiitis of Churg-Strauss syndrome, or a renal-limited vasculitis. (musculoskeletalkey.com)
Pauci-immune renal disease can be renal-limited vasculitis (RLV) or the renal manifestations of microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), or Churg-Strauss syndrome (CSS). (musculoskeletalkey.com)

The principal component of basement membrane is type IV collagen, which acts as a support structure and is composed of building blocks that are linked end-to-end. (medscape.com)
In most patients, the autoantibody in Goodpasture syndrome is directed against a 28-kd monomeric subunit present within the noncollagenous domain of the alpha3 chain of type IV collagen (alpha3[IV]NC1). (medscape.com)
Type IV collagen is the major component of basement membranes and provides structural support. (rheumaknowledgy.com)
Anti-GBM disease is due to circulating autoantibodies directed against cryptic epitopes in the NC1 domain of the alpha-3 chain of type IV collagen (a3NC1), also known as the Goodpasture antigen. (arkanalabs.com)

Anti-GBM disease is an autoimmune disorder characterized by autoantibodies directed against the glomerular/alveolar basement membrane. (medscape.com)
These autoantibodies are believed to cross-react with the alveolar basement membrane and cause similar damage. (medscape.com)
The degree of cross-linking of the α3NC1 hexamer subunits is approximately 3 times greater in the alveolar basement membrane than in the GBM. (medscape.com)

A variant in one of the two copies of the COL4A3 gene can cause thin basement membrane nephropathy. (medlineplus.gov)
Thin basement membrane nephropathy rarely progresses to kidney failure. (medlineplus.gov)
The clinical features of thin basement membrane nephropathy. (medlineplus.gov)

Go to Pediatric Anti-GBM Disease (Goodpasture Syndrome) for complete information on this topic. (medscape.com)

In 1919, Ernest Goodpasture described an 18-year-old man with a fever and cough, followed by hemoptysis and renal failure. (medscape.com)
Airway and lung involvement - The most common respiratory symptoms in GPA and MPA are cough, hemoptysis (due to alveolar hemorrhage and/or tracheobronchial disease), dyspnea, and pleuritic pain. (medilib.ir)

Acute kidney injury (AKI) is a clinical syndrome manifested by a rapid or abrupt decline in kidney function and subsequent dysregulation of the body electrolytes and volume, and abnormal retention of nitrogenous waste. (medscape.com)
[ 1 ] Anti-GBM disease, a more precise term, should be used to refer to either of the two distinct clinical manifestations of this disorder. (medscape.com)
Despite this triad of clinical findings, patients with anti-GBM disease may present with a spectrum of conditions ranging from pulmonary hemorrhage with minimal or no renal involvement to full-blown renal failure with limited or no pulmonary involvement. (medscape.com)
This section deals with clinical conditions associated with renal disease which are covered by the Immunology department. (mft.nhs.uk)
Methods A retrospective analysis of clinical and genetic data of a patient with Alport syndrome was performed. (researchsquare.com)
The authors also describe why the disease is sometimes limited to the kidneys, the clinical course of renal disease, treatment issues, how to deal with disease relapses, and how to prevent them from occurring. (musculoskeletalkey.com)
This article discusses why the disease is sometimes limited to the kidneys, the clinical course of renal involvement, treatment issues, how to deal with disease relapses, and strategies to prevent disease recurrence. (musculoskeletalkey.com)
The optimal treatment for lupus nephritis depends on the underlying lesion and disease activity, as well as the severity and duration of the clinical presentation. (basicmedicalkey.com)

These people are said to have anti-glomerular basement membrane nephritis. (medlineplus.gov)
Low serum complement levels suggest the following systemic diseases: cryoglobulinemia, systemic lupus erythematosus (SLE), bacterial endocarditis, and shunt nephritis. (medscape.com)
In patients with new-onset nephritis syndrome and a low C3 level, Chauvet et al report that the presence of anti-factor B autoantibodies may help distinguish new-onset PSGN from hypocomplementemic C3 glomerulopathy. (medscape.com)

Urinalysis and sediment examination are crucial in the evaluation of patients with acute nephritic syndrome. (medscape.com)
Patients typically present with nephritic syndrome and rapidly progressive acute kidney injury. (arkanalabs.com)
Know acute management of the hyperkalemic patient including steps to stabilize the myocardial membrane, shift potassium into the cells, and lower the total body potassium. (uaphxim.com)

Immunofluorescent staining of biopsy specimens from the lungs and kidneys of patients with Goodpasture syndrome reveals a characteristic linear pattern of anti-GBM antibody deposition. (rheumaknowledgy.com)
The diagnosis of Goodpasture syndrome may be secured by demonstrating linear antibody staining of the basement membrane of renal or pulmonary biopsy specimens. (rheumaknowledgy.com)
However, patients are often quite ill during the initial course of the disease, making biopsy more difficult. (rheumaknowledgy.com)
On biopsy typical disease displays bright polytypic linear GBM staining for IgG by immunofluorescence and diffuse crescentic/necrotizing GN on light microscopy. (arkanalabs.com)
Renal biopsy revealed that the basement membrane was of varying thickness, with a thickened dense layer, some of which was tear-like and arachnoid. (researchsquare.com)
Furthermore, the necessity of renal biopsy and repeat biopsy, the usefulness of rapid detection of ANCA for diagnosis, the relevance of serial measurement of ANCA during follow-up, the effect of dialysis on the disease process, and the issue of renal transplantation after disease remission are also discussed. (musculoskeletalkey.com)

The autoantibodies bind to their reactive epitopes in the basement membranes and activate the complement cascade, resulting in tissue injury. (medscape.com)
This interaction results in complement activation with glomerular infiltration of polymorphonuclear leukocytes (PMNs) and monocytes. (medscape.com)
Normal serum complement levels suggest a visceral abscess, polyarteritis nodosa, Goodpasture syndrome, or Henoch-Schönlein purpura. (medscape.com)
In SLE, monitoring anti-DNA antibody levels and complement C4 is more useful than monitoring ANA. (mft.nhs.uk)

Subepithelial deposition of immune complexes with basement membrane thickening -produces a spiked appearance on silver stain. (shiken.ai)

The pathogenesis of anti-glomerular basement membrane (anti-GBM) disease (Goodpasture disease) is linked to the presence of autoantibodies that react with the alveolus in the lung and the basement membrane of the glomerulus in the kidney. (medscape.com)

Microscopically, there is crescent formation and glomerular fibrinoid necrosis. (surgpath4u.com)
The most frequently encountered histologic appearance is focal segmental glomerular fibrinoid necrosis with crescent formation and only mild hypercellularity, although neutrophils are modestly present at sites of necrosis ( Figs. 1 and 2 ). (musculoskeletalkey.com)

Ernest Goodpasture first described this disorder in 1919. (medscape.com)

This provides an explanation for why end-organ involvement of Goodpasture syndrome is typically limited to the lungs and kidneys. (rheumaknowledgy.com)
In the kidney, other types of collagen build up in the basement membranes, eventually scarring the kidneys and leading to kidney failure. (medlineplus.gov)
Glomerulopathies can further be classified as primary or secondary, with primary diseases directly affecting the kidneys and secondary diseases caused by another disease or process. (shiken.ai)
Pain around the kidneys is an uncommon symptom in kidney disease except with kidney stones , and usually has alternative explanations. (edren.org)

Plasmapheresis to remove the anti-GBM antibody and immunosuppressive therapy. (surgpath4u.com)
In this immune reaction, antibody immunosuppressive, immunostimulatory, and autoim- is directed against a person's own cellular surface anti- mune. (cdc.gov)
Steroids and immunosuppressive agents are reserved for patients with severe disease. (basicmedicalkey.com)

We conclude that, when faced with a nephritic syndrome clinic, the viral history should be taken into account to make an early diagnosis. (bvsalud.org)
These can be divided into nephrotic and nephritic syndrome, and this article will cover the causes and management of a patient with suspected glomerular disease. (shiken.ai)
Nephritic syndrome occurs due to inflammation and damage to endothelial cells of glomerular capillaries and can be triggered by several disease processes. (shiken.ai)

The basement membranes are complex structures that support layers of endothelium and epithelium. (medscape.com)
Anti-GBM autoantibodies that are present in the circulation of patients with anti-GBM disease cross the fenestrated endothelium in the glomerulus and bind with the underlying GBM, inducing renal injury. (medscape.com)
The capillary wall, which serves as a filtration barrier, consists of three well-defined layers: fenestrated endothelium, glomerular basement membrane (GBM), and epithelial cell layer. (basicmedicalkey.com)

Because of the lack of consistently effective treatment for primary focal segmental glomerular sclerosis, angiotensin-converting enzyme inhibitors or angiotensin receptor blockers are commonly used for patients with mild disease to control symptoms. (basicmedicalkey.com)

When describing diseases of the glomerulus, terminology can be confusing. (shiken.ai)

The role of HLA-DRB1 alleles on susceptibility of Chinese patients with anti-GBM disease. (medscape.com)

This previously fatal disease has nowadays a very good survival rate because of immunosuppresion therapy, plasmapheresis and hemodialysis. (bvsalud.org)

Many present with a reno-pulmonary syndrome -- the combination of renal and pulmonary insufficiency. (surgpath4u.com)

When I was hospitalized for my first autoimmune disease, I spent a month in hospital. (whenpigstakeflight.com)
Six years later, when I called him late one Monday night, frightened by my own thoughts and confused by my own behavior as my second autoimmune disease began to set my brain afire, he was there yet again, as I'd always known he would be. (whenpigstakeflight.com)

Finally, the importance of histopathological examination in the diagnosis of Goodpasture syndrome and the Goodpasture-like syndrome with atypical course is emphasized. (edu.pl)
September 2001, Prague, Czech Republic tional respiratory disease diagnosis. (cdc.gov)
The same tests are also useful for the diagnosis of chronic renal disease. (mft.nhs.uk)

This agent interferes with the inflammatory response by decreasing bone marrow response through the interference of DNA cross-linking and decreases anti-glomerular basement membrane (anti-GBM) antibody production. (medscape.com)

Many variants (also called mutations) in the COL4A3 gene can cause Alport syndrome, a condition characterized by kidney disease, hearing loss, and eye abnormalities. (medlineplus.gov)
Variants in a single copy of the COL4A3 gene can cause a form of the condition called autosomal dominant Alport syndrome. (medlineplus.gov)
Individuals with this form typically have the kidney problems that are characteristic of Alport syndrome, including blood in the urine (hematuria), excess amounts of protein in the urine (proteinuria), and a gradual loss of kidney function. (medlineplus.gov)
Variants in both copies of the COL4A3 gene cause autosomal recessive Alport syndrome. (medlineplus.gov)
Purpose We report a novel gene mutation in a female patient with Alport syndrome who was planning to undergo assisted reproduction to achieve pregnancy. (researchsquare.com)
Conclusion Genetic testing prior to pregnancy is recommended in Alport syndrome patients or family members prior to pregnancy to determine the specific location of the gene mutation. (researchsquare.com)

Some of these tests are also used to monitor renal disease (ANCA, C4). (mft.nhs.uk)
In these situations, more frequent testing of ANCA or anti-GBM is appropriate. (mft.nhs.uk)
Thus, a patient can have "severe" disease in the lungs in the presence of antineutrophil cytoplasmic antibody (ANCA)-associated diffuse alveolar hemorrhage with mild extrapulmonary involvement [ 8 ]. (medilib.ir)

Polymyositis or Dermatomyositis (Conditions in which the muscles or skin get inflamed), Rheumatoid Arthritis (Disease in which the joints become painful or deformed), Systemic Lupus Erythematosus (A Chronic Inflammatory Condition). (askdrshah.com)

In the first group the disease was histologically and clinically more severe, and lmmunofluorescence hlstology was heterogeneous and often nonspecific except for three cases In which there were linear Immunoglobulin deposits. (coek.info)

Collagen type IV molecules assemble to form a sheet-like network which is involved in maintaining the structural integrity of basement membranes. (lookformedical.com)

Cyclophosphamide decreases the immune system's response, and although concerns about toxicity restrict its use to patients with severe disease, it remains an important treatment for life-threatening autoimmune diseases where disease-modifying antirheumatic drugs (DMARDs) have been ineffective. (wikipedia.org)
For severe or rapidly progressing disease, methylprednisolone at a dose of 250 mg every 6 hours should be administered. (medscape.com)
Methylprednisolone is the drug of choice for severe disease. (medscape.com)
1. Severe (grades 3-5) Guillain Barre` syndrome (G61.0) (consistent with guidelines from the American Academy of Neurology, it is generally considered medically necessary to initiate PE within 2 weeks of onset of neuropathic symptoms for ambulant individuals and within 4 weeks of symptom onset for non-ambulant individuals). (qualchoice.com)
The term was confusing, as "limited" disease could be severe. (medilib.ir)
Abnormal α-chain structures destroy the integrity of the basement membrane, which initially leads to hematuria and later develops into moderate-to-severe proteinuria, progressive renal failure, high-pitched sensorineural hearing impairment, and eye disease. (researchsquare.com)
Interstitial disease is present frequently, mostly characterized by a mild to severe interstitial infiltrate ( Figs. 3 and 4 ). (musculoskeletalkey.com)
It is more likely to be connected to kidney disease in people who are young, or have severe high blood pressure. (edren.org)
One notable condition found in Pfizer's data is 1p36 deletion syndrome, which the National Library of Medicine describes as "a disorder that typically causes severe intellectual disability. (bigleaguepolitics.com)

The mesangium, which consists of mesangial cells embedded in an extracellular matrix, provides support for the glomerular capillaries and also modulates blood flow through the capillaries. (basicmedicalkey.com)

Glomerulonephropathies are diseases which cause inflammation in the glomeruli. (shiken.ai)

Anti-glomerular basement membrane autoantibodies against different target antigens are associated with disease severity. (medscape.com)
Hypersensitivity Pneumonitis Hypersensitivity pneumonitis is a syndrome of cough, dyspnea, and fatigue caused by sensitization and subsequent hypersensitivity to environmental (frequently occupational or domestic) antigens. (msdmanuals.com)

This pattern of lung disease has not been previously described in ABMA-GS. (elsevierpure.com)
A raredisease which can involve lung disease and a quickly worsening kidney. (askdrshah.com)
The Col 4α3NC1 epitope is thought to be less accessible for anti-GBM binding in the lung, and partial denaturation of NC1 domains may be required for full exposure of this sequestered epitope to the antibody. (medscape.com)
A full medical history, including tobacco use , inhalation exposures, and history of lung disease, may be obtained. (osmosis.org)

6. Goodpasture`s syndrome (M31.0) . (qualchoice.com)

In both cases the course of the disease was atypical, with a massive respiratory tract hemorrhage being the most significant symptom. (edu.pl)

Anti-glomerular basement membrane disease: outcomes of different therapeutic regimens in a large single center Chinese cohort study. (arkanalabs.com)
The precise pathogenetic mechanisms of many glomerular diseases remain unknown, and the available therapeutic regimens are still far from optimal. (basicmedicalkey.com)

People with this condition typically have blood in their urine (hematuria) but no other signs or symptoms of kidney disease. (medlineplus.gov)
Urine test results revealed 60% severely deformed red blood cells, indicating persistent glomerular hematuria. (researchsquare.com)

Rupture of the glomerular basement membrane and Bowman's capsule may be seen, especially if highlighted by silver stain. (surgpath4u.com)

Mostly this is accompanied by findings suggestive of kidney disease. (askdrshah.com)
Genetic factors such as APOL-L1 mutations are associated with an increased risk of FSGS and progression to end-stage kidney disease. (shiken.ai)
60 years old, patients with chronic kidney disease and patients with diabetes. (uaphxim.com)
Most people with high blood pressure do not have serious kidney disease, but high blood pressure can be a sign of kidney disease. (edren.org)
Otherwise pain is unusual in kidney disease. (edren.org)

Anatomy16

Organisms3

Diseases26

Chemicals and Drugs29

Analytical, Diagnostic and Therapeutic Techniques and Equipment12

Phenomena and Processes10

Disciplines and Occupations1

Technology, Industry, Agriculture1

Information Science3

Named Groups3

Antibodies33

Glomerulonephritis23

Nephrotic syndrome10

Granulomatosis with polyangiitis2

Goodpasture's9

Pathophysiology2

Pulmonary-renal syndrome5

Vasculitis7

Type IV colla4

Alveolar basement membrane3

Nephropathy3

Pediatric Anti-GBM Disease1

Hemoptysis2

Clinical8

Nephritis3

Acute3

Biopsy6

Complement4

Deposition1

Pathogenesis1

Fibrinoid necrosis2

Ernest Goodpasture1

Kidneys4

Immunosuppressive3

Nephritic syndrome3

Endothelium3

Segmental1

Glomerulus1

Patients with anti-GBM disease1

Plasmapheresis1

Combination of renal1

Autoimmune disease2

Diagnosis3

Antibody production1

Alport6

ANCA3

Rheumatoid arthritis1

Immunoglobulin1

Collagen ty1

Severe9

Capillaries1

Cause inflammation1

Antigens2

Lung4

M31.01

Atypical1

Therapeutic regimens2

Hematuria2

Bowman's1

Kidney Disease5