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Anti-glomerular basement membrane disease

MedGen UID:
140788
Concept ID:
C0403529
Disease or Syndrome
Synonyms: Anti-glomerular basement membrane antibody disease; Glomerulonephritis - pulmonary hemorrhage; Goodpasture syndrome; Pulmonary renal syndrome; Rapidly progressive glomerulonephritis with pulmonary hemorrhage
SNOMED CT: Goodpasture syndrome (50581000); Anti-glomerular basement membrane disease (788613004); Anti GBM - antiglomerular basement membrane disease (788613004); Goodpasture disease (788613004); Goodpasture's disease (788613004); Goodpasture's syndrome (50581000); Hemorrhagic pneumonia AND glomerulonephritis (50581000); Pulmonary-renal syndrome (50581000); Lung purpura with nephritis syndrome (50581000); Pulmonary hemorrhage with glomerulonephritis (50581000); Pulmonary hemosiderosis with glomerulonephritis (50581000); Anti-GBM nephritis with pulmonary hemorrhage (50581000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0009303
OMIM®: 233450
Orphanet: ORPHA375

Definition

A rare, fulminant small vessel vasculitis that affects the capillary beds of the kidneys and lungs and characterized by the presence of anti-glomerular basement membrane (GBM) and, in its full-blown form, anti-alveolar basement membrane (ABM) antibodies. Consequently, it may manifest as a rapidly progressive, isolated glomerulonephritis (anti-GBM nephritis) or as a pulmonary-renal syndrome with severe lung hemorrhage. [from ORDO]

Clinical features

From HPO
Chest pain
MedGen UID:
2992
Concept ID:
C0008031
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the chest.
Fatigue
MedGen UID:
41971
Concept ID:
C0015672
Sign or Symptom
A subjective feeling of tiredness characterized by a lack of energy and motivation.
Chills
MedGen UID:
43211
Concept ID:
C0085593
Sign or Symptom
A sudden sensation of feeling cold.
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Cylindruria
MedGen UID:
101807
Concept ID:
C0151990
Body Substance
The presence of renal casts (cylindrical, cigar-shaped structures produced by the kidney in certain disease states) in the urine.
Macroscopic hematuria
MedGen UID:
633103
Concept ID:
C0473237
Finding
Hematuria that is visible upon inspection of the urine.
Erythrocyte cylindruria
MedGen UID:
637069
Concept ID:
C0522129
Finding
Presence of erythrocyte casts (cylindrical structures produced by the kidney in certain disease states) in the urine.
Renal insufficiency
MedGen UID:
332529
Concept ID:
C1565489
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
Glomerular crescent formation
MedGen UID:
1785072
Concept ID:
C5539893
Finding
Glomerular crescent refers hyperplastic lesions involving 10% or more of the circumference of Bowman's capsule. Crescents can be composed of a variable mixture of epithelial/leukocyte hypercellularity, fibrous matrix, and fibrin.
Pulmonary hemorrhage
MedGen UID:
57732
Concept ID:
C0151701
Pathologic Function
Pulmonary hemorrhage is a bleeding within the lungs. Older children and adults may spit blood or bloody sputum. Neonates, infants and young children usually do not spit up blood. Anemia, pulmonary infiltrates, increasingling bloody return on BAL and the presence of hemosiderin-laden macrophages in broncho-alveolar lavage (BAL) fluid or lung biopsy can diagnose lung bleeding. Alveolar macrophages contain phagocytosed red blood cells and stain positive for hemosiderin, a product of hemoglobin degradation, after about 48-72 hours following pulmonary hemorraghe. Previous or recurrent bleeding can thus be distinguished from fresh events. A differentiation into local or diffuse is of importance. Also differentiate if pulmonary hemorrhage is due to a primary lung disorder or a manifestation of a systemic disease.
Weight loss
MedGen UID:
853198
Concept ID:
C1262477
Finding
Reduction of total body weight.
Anemia
MedGen UID:
1526
Concept ID:
C0002871
Disease or Syndrome
A reduction in erythrocytes volume or hemoglobin concentration.
Cough
MedGen UID:
41325
Concept ID:
C0010200
Sign or Symptom
A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation.
Hemoptysis
MedGen UID:
5502
Concept ID:
C0019079
Sign or Symptom
Coughing up (expectoration) of blood or blood-streaked sputum from the larynx, trachea, bronchi, or lungs.
Crackles
MedGen UID:
11118
Concept ID:
C0034642
Finding
Crackles are discontinuous, explosive, and nonmusical adventitious lung sounds normally heard in inspiration and sometimes during expiration. Crackles are usually classified as fine and coarse crackles based on their duration, loudness, pitch, timing in the respiratory cycle, and relationship to coughing and changing body position.
Exertional dyspnea
MedGen UID:
68549
Concept ID:
C0231807
Sign or Symptom
Perceived difficulty to breathe that occurs with exercise or exertion and improves with rest.
Tachypnea
MedGen UID:
66669
Concept ID:
C0231835
Finding
Very rapid breathing.
Pulmonary infiltrates
MedGen UID:
116009
Concept ID:
C0235896
Finding
A finding indicating the presence of an inflammatory or neoplastic cellular infiltrate in the lung parenchyma.
Restrictive ventilatory defect
MedGen UID:
478856
Concept ID:
C3277226
Finding
A functional defect characterized by reduced total lung capacity (TLC) not associated with abnormalities of expiratory airflow or airway resistance. Spirometrically, a restrictive defect is defined as FEV1 (forced expiratory volume in 1 second) and FVC (forced vital capacity) less than 80 per cent. Restrictive lung disease may be caused by alterations in lung parenchyma or because of a disease of the pleura, chest wall, or neuromuscular apparatus.
Increased DLCO
MedGen UID:
893148
Concept ID:
C4073174
Finding
Increased ability of the lungs to transfer gas from inspired air to the bloodstream as measured by the diffusing capacity of the lungs for carbon monoxide (DLCO) test.
Reticular pattern on pulmonary HRCT
MedGen UID:
1386432
Concept ID:
C4476748
Finding
On pulmonary high-resolution computed tomography, reticular pattern is characterised by innumerable interlacing shadows suggesting a mesh.
Nodular pattern on pulmonary HRCT
MedGen UID:
1380219
Concept ID:
C4476750
Finding
A nodular pattern is characterised on pulmonary high-resolution computed tomography by the presence of numerous rounded opacities that range from 2 mm to 1 cm in diameter, with micronodules defined as smaller than 3 mm in diameter.
Parenchymal consolidation
MedGen UID:
1687071
Concept ID:
C5139174
Finding
Consolidation refers to an exudate or other product of disease that replaces alveolar air, rendering the lung solid (as in infective pneumonia).
Hemosiderin-laden macrophages in bronchoalveolar fluid
MedGen UID:
1717924
Concept ID:
C5397971
Finding
Hemosiderin-laden macrophages (HLM) in bronchoalveolar lavage (BAL) fluid were originally known as adiagnostic biomarker of alveolar hemorrhage, but have also been observed in idiopathic pulmonary fibrosis (IPF) with histopathological pattern of usual interstitial pneumonia (UIP).
Bloody bronchoalveolar lavage fluid
MedGen UID:
1724345
Concept ID:
C5421662
Finding
Return of lavage fluid characteristically has a bloody appearance in pulmonary alveolar hemorrhage. This is due to admixture of blood, which typically increases with each consecutive lavage portion. Microscopically erythrocytes can be seen. After 24 to 48 h macrophages contain phagocytosed erythrocytes, indicating previous bleeding.
Ground-glass opacification
MedGen UID:
1779663
Concept ID:
C5539411
Finding
On chest radiographs, ground-glass opacity appears as an area of hazy increased lung opacity, usually extensive, within which margins of pulmonary vessels may be indistinct. On CT scans, it appears as hazy increased opacity of lung, with preservation of bronchial and vascular margins. It is caused by partial filling of airspaces, interstitial thickening (due to fluid, cells, and/or fibrosis), partial collapse of alveoli, increased capillary blood volume, or a combination of these, the common factor being the partial displacement of air. Ground-glass opacity is less opaque than consolidation, in which bronchovascular margins are obscured.
Glomerulonephritis
MedGen UID:
6616
Concept ID:
C0017658
Disease or Syndrome
Inflammation of the renal glomeruli.
Cytoplasmic antineutrophil antibody positivity
MedGen UID:
1690999
Concept ID:
C5139210
Finding
The presence of autoantibodies in the serum that react against proteins predominantly expressed in cytoplasmic granules of neutrophils.
Anti-glomerular basement membrane-antibody positivity
MedGen UID:
1747042
Concept ID:
C5421560
Laboratory or Test Result
The presence of autoantibodies in the serum that react to the glomerular basement membrane.
Anti-myeloperoxidase antibody positivity
MedGen UID:
1784416
Concept ID:
C5539684
Laboratory or Test Result
The presence of autoantibodies in the blood circulation that react against myeloperoxidase.
Fever
MedGen UID:
5169
Concept ID:
C0015967
Sign or Symptom
Body temperature elevated above the normal range.
Increased blood urea nitrogen
MedGen UID:
760252
Concept ID:
C0151539
Finding
An increased amount of nitrogen in the form of urea in the blood.
Cyanosis
MedGen UID:
1189
Concept ID:
C0010520
Sign or Symptom
Bluish discoloration of the skin and mucosa due to poor circulation or inadequate oxygenation of arterial or capillary blood.
Pallor
MedGen UID:
10547
Concept ID:
C0030232
Finding
Abnormally pale skin.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnti-glomerular basement membrane disease
Follow this link to review classifications for Anti-glomerular basement membrane disease in Orphanet.

Professional guidelines

PubMed

Jaryal A, Vikrant S
Clin Nephrol 2022 Dec;98(6):280-287. doi: 10.5414/CN110851. PMID: 36282172
Henderson SR, Salama AD
Nephrol Dial Transplant 2018 Feb 1;33(2):196-202. doi: 10.1093/ndt/gfx057. PMID: 28459999
Clark WF, Huang SS, Walsh MW, Farah M, Hildebrand AM, Sontrop JM
Kidney Int 2016 Nov;90(5):974-984. Epub 2016 Aug 3 doi: 10.1016/j.kint.2016.06.009. PMID: 27498206

Recent clinical studies

Etiology

Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
Prendecki M, Pusey C
Presse Med 2019 Nov;48(11 Pt 2):328-337. Epub 2019 Nov 5 doi: 10.1016/j.lpm.2019.03.017. PMID: 31703956
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article
Silvariño R, Noboa O, Cervera R
Isr Med Assoc J 2014 Nov;16(11):727-32. PMID: 25558706

Diagnosis

Reggiani F, L'Imperio V, Calatroni M, Pagni F, Sinico RA
Clin Exp Rheumatol 2023 Apr;41(4):964-974. Epub 2023 Mar 30 doi: 10.55563/clinexprheumatol/tep3k5. PMID: 36995324
Ponticelli C, Calatroni M, Moroni G
Autoimmun Rev 2023 Jan;22(1):103212. Epub 2022 Oct 14 doi: 10.1016/j.autrev.2022.103212. PMID: 36252931
Gulati K, McAdoo SP
Rheum Dis Clin North Am 2018 Nov;44(4):651-673. Epub 2018 Sep 7 doi: 10.1016/j.rdc.2018.06.011. PMID: 30274629
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article
Hellmark T, Segelmark M
J Autoimmun 2014 Feb-Mar;48-49:108-12. Epub 2014 Jan 21 doi: 10.1016/j.jaut.2014.01.024. PMID: 24456936

Therapy

Ponticelli C, Calatroni M, Moroni G
Autoimmun Rev 2023 Jan;22(1):103212. Epub 2022 Oct 14 doi: 10.1016/j.autrev.2022.103212. PMID: 36252931
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
Uhlin F, Szpirt W, Kronbichler A, Bruchfeld A, Soveri I, Rostaing L, Daugas E, Lionet A, Kamar N, Rafat C, Mysliveček M, Tesař V, Fernström A, Kjellman C, Elfving C, McAdoo S, Mölne J, Bajema I, Sonesson E, Segelmark M
J Am Soc Nephrol 2022 Apr;33(4):829-838. Epub 2022 Mar 8 doi: 10.1681/ASN.2021111460. PMID: 35260419Free PMC Article
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article

Prognosis

Uhlin F, Szpirt W, Kronbichler A, Bruchfeld A, Soveri I, Rostaing L, Daugas E, Lionet A, Kamar N, Rafat C, Mysliveček M, Tesař V, Fernström A, Kjellman C, Elfving C, McAdoo S, Mölne J, Bajema I, Sonesson E, Segelmark M
J Am Soc Nephrol 2022 Apr;33(4):829-838. Epub 2022 Mar 8 doi: 10.1681/ASN.2021111460. PMID: 35260419Free PMC Article
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
McAdoo SP, Pusey CD
Semin Respir Crit Care Med 2018 Aug;39(4):494-503. Epub 2018 Nov 7 doi: 10.1055/s-0038-1669413. PMID: 30404116
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article
Silvariño R, Noboa O, Cervera R
Isr Med Assoc J 2014 Nov;16(11):727-32. PMID: 25558706

Clinical prediction guides

Floyd L, Bate S, Hadi Kafagi A, Brown N, Scott J, Srikantharajah M, Myslivecek M, Reid G, Aqeel F, Frausova D, Kollar M, Kieu PL, Khurshid B, Pusey CD, Dhaygude A, Tesar V, McAdoo S, Little MA, Geetha D, Brix SR
J Am Soc Nephrol 2023 Mar 1;34(3):505-514. Epub 2022 Nov 29 doi: 10.1681/ASN.2022050581. PMID: 36446430Free PMC Article
Hu X, Shen C, Meng T, Ooi JD, Eggenhuizen PJ, Zhou YO, Luo H, Chen JB, Lin W, Gong Y, Xiong Q, Xu J, Liu N, Xiao X, Tang R, Zhong Y
Front Immunol 2022;13:991469. Epub 2022 Oct 27 doi: 10.3389/fimmu.2022.991469. PMID: 36389826Free PMC Article
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
McAdoo SP, Pusey CD
Clin J Am Soc Nephrol 2017 Jul 7;12(7):1162-1172. Epub 2017 May 17 doi: 10.2215/CJN.01380217. PMID: 28515156Free PMC Article
L'Imperio V, Ajello E, Pieruzzi F, Nebuloni M, Tosoni A, Ferrario F, Pagni F
J Nephrol 2017 Aug;30(4):503-509. Epub 2017 Apr 5 doi: 10.1007/s40620-017-0394-x. PMID: 28382508

Recent systematic reviews

Shiroshita A, Oda Y, Takenouchi S, Hagino N, Kataoka Y
Am J Nephrol 2021;52(7):531-538. Epub 2021 Aug 19 doi: 10.1159/000518362. PMID: 34515033
Syeda UA, Singer NG, Magrey M
Semin Arthritis Rheum 2013 Jun;42(6):567-72. Epub 2013 Jan 24 doi: 10.1016/j.semarthrit.2012.10.007. PMID: 23352254

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