Goodpasture syndrome differential diagnosis: Difference between revisions

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* [[Idiopathic]]
* [[Idiopathic]]
* [[Protein]] [[Protein tyrosine phosphatase|tyrosine phosphatase]] receptor type O (glomerular epithelial protein 1- GLEPP1)
* [[Protein]] [[Protein tyrosine phosphatase|tyrosine phosphatase]] receptor type O  
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* Young children
* Young children
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* [[Atopy]]
* [[Atopy]]
* [[Hodgkin's lymphoma|Hodgkin lymphoma]]
* [[Hodgkin's lymphoma|Hodgkin lymphoma]]
* [[Thrombosis]] (due to [[Urinary system|urinary]] loss of [[Antithrombin III|antithrombin-III]])
* [[Thrombosis]]  
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* Idiopathic
* Idiopathic
* [[Human Immunodeficiency Virus (HIV)|HIV]]
* [[Human Immunodeficiency Virus (HIV)|HIV]] or [[Heroine hydrochloride|heroine]] use
* [[Heroine hydrochloride|Heroine]] use
* [[Sickle-cell disease|Sickle cell disease]]
* [[Sickle-cell disease|Sickle cell disease]]
* [[Interferon]]
* [[Interferon]]
* Severe [[obesity]]
* Severe [[obesity]]
* [[Cryoglobulinemia|Mixed cryoglobulinemia]] ([[Hepatitis C]])
* [[Cryoglobulinemia|Mixed cryoglobulinemia]]  
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* Adults
* Adults
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* [[Solid tumors]]
* [[Solid tumors]]
* [[Systemic lupus erythematosus]]
* [[Systemic lupus erythematosus]]
* Drugs ([[NSAIDS]], pencilamine, [[gold]], [[captopril]])
* Drugs
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* Young children
* Young children
* History of [[mucosal]] [[infections]] (e.g. [[gastroenteritis]]) and [[upper respiratory tract infection]]
* History of [[mucosal]] [[infections]] (e.g. [[gastroenteritis]]) and [[upper respiratory tract infection]]
* 2-3 days after [[infection]] (synpharyngitic)
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* Hypercellular and [[inflamed]] [[glomeruli]] (Crescent formation)
* Hypercellular and [[inflamed]] [[glomeruli]] (crescent formation)
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*  Diffuse thickening of the [[glomerular basement membrane]] with absence of sub-[[Epithelial cells|epithelial]] and sub-[[endothelial]] deposits 
*  Diffuse [[glomerular basement membrane]] thickening
* Absence of sub-[[Epithelial cells|epithelial]] and sub-[[endothelial]] deposits 
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Revision as of 18:05, 2 May 2018


Goodpasture syndrome Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]

Overview

Goodpasture syndrome should be differentiated from other diseases presenting as fever, hematuria and hemoptysis. It should also be differentiated from other causes of small vessel vasculitis.

Differential Diagnosis

Goodpasture syndrome should be differentiated from other diseases presenting as fever, hematuria, hemoptysis. It should also be differentiated from other causes of small vessel vasculitis. The differentials include the following:

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound/ Echocardiography Other
Goodpasture syndrome or Anti-glomerular basement membrane disease[1] + +/- - - - - + - Anti-GBM antibodies Hypochromic microcytic anemia, Thrombocytopenia - C3 level Pulmonary hemorrhage - Normal kidneys Alveolar infiltrates spreading from the hilum in CXR Cellular crescents in the glomeruli, Intra-alveolar hemorrhages Anti-GBM antibodies Hemoptysis, Hematuria
Small-Vessel Vasculitis ANCA-associated vasculitis Granulomatosis with polyangiitis (Wegener’s)[2] + +/- +/- - - - - + Anti-PR3 antibody (C-ANCA) (90%), Anti-MPO antibody (P-ANCA) (10%) Leukocytosis, Normochromic normocytic anemia Cr or BUN, Hypoalbuminemia Consolidation, Patchy or diffuse ground-glass opacities Occlusion or stenosis of LAD and RCA in coronary angiography - Single or multiple nodules and masses with cavitation in CXR Parenchymal necrosis, Granulomatous inflammation Histological confirmation Conjunctivitis,

Episcleritis,

Uveitis,

Optic nerve vasculitis

Immune complex small-vessel vasculitis Cryoglobulinemic vasculitis[3] +/- +/- - + +/- - - - C4 component LeukocytosisAnemia ANA, hypocomplementemia R/O underlying malignancy Stenosis or occlusions of the visceral arteries Bacterial endocarditis in echocardiography Interstitial involvement or pleural effusions in CXR HCV-associated proteins in vasculitic skin, Intraluminal cryoglobulin deposits  Histological confirmation Acrocyanosis, Retinal hemorrhage, Purpura
Hepatitis C virus-associated cryoglobulinemic vasculitis[4] +/- +/- + + - - + +/- HCV RNA, Cryoglobulins LeukocytosisAnemia Serum C4, Positive RF Increased hepatic echogenicity - Hepatomegaly, Splenomegaly Increased hepatic echogenicity in MRI Vasculitic skin, Antigen infilteration in lesions HCV RNA, Histological confirmation Palpable purpura, Microscopic hematuria
Gastrointestinal disease Acute mesenteric ischemia[5] - +/- - - - - - - I-FABP, Alpha-GST, Ischemia-modified albumin (IMA) Leukocytosis, ↑HCT -  ↑Amylase Bowel wall thickening, Intestinal pneumatosis, Portomesenteric thrombosis Mesenteric venous thrombosis  Arterial stenosis or occlusion of the celiac or superior mesenteric arteries in duplex ultrasound Ileus with distended loops of bowel, Bowel wall thickening in abdominal X-ray Superficial mucosal hemorrhage, edema and necrosis History and physical examination Abdominal pain, Distension, Absent bowel sounds
Cardiovascular disease Infective Endocarditis[6] + + + - - - - + NT-proBNP Normochromic-normocytic anemia Hyperglobulinemia, Cryoglobulinemia Metastatic infections, such as splenic infarct, renal infarcts, or psoas abscess - Vegetation, abscess, or new dehiscence of a prosthetic valvein echocardiography Vertebral osteomyelitis in MRI Vegetation or intracardiac abscess demonstrating active endocarditis Echocardiography (TTE) Janeway lesions, Osler nodes, Roth spots, Vertebral osteomyelitis
Pulmonary disease Non-Small Cell Lung Cancer[7] - +/- + - - - - +/- EGFR, ROS1, EML4-ALK,  PD-L1 Leukocytosis, Anemia Hypercalcemia, Hyponatremia Pulmonary lesion or mass - Pulmonary marginal lesions Staging and response to treatment in PET-CT Adenocarcinoma, Squamous cell carcinoma High resolution CT-scan Cough, Hemoptysis
Small Cell Lung Cancer[8] - +/- + - - - - +/- p53, Thyroid transcription factor-1 (TTF1) Anemia Hyponatremia Large hilar mass with bulky mediastinal adenopathy - Endobronchial ultrasound (EBUS) Standard staging Spindled cells with dark nuclei, scant cytoplasm, and fine, granular nuclear chromatin High resolution CT-scan Cough, Hemoptysis
Hematologic disease Hemolytic-Uremic Syndrome[9] + + - + - - + + C5b-9, ADAMTS13 Anemia, Thrombocytopenia, Reticulocytosis  Lactate dehydrogenase (LDH), Hypercalcemia  Thalami, brainstem, or cerebellum abnormality Cerebral microangiopathy or hypertension Hypoechoic kidney  Abnormal hyperintensity in the brain cisterns in MRI Microthromboses include fibrin thrombi that may occlude the glomerular tuft Clinical findings coupled with laboratory abnormalities Hematuria, Proteinuria 
Chronic Lymphocytic Leukemia (CLL)[10] + + + + +/- - - +/- CD5, CD19, CD20, IgVH Absolute lymphocytosis, Smudge cells Flow cytometry Staging - - - Large atypical cells, cleaved cells, and prolymphocytes  Chromosomal and genetic testing Easy bruising
Hypereosinophilic Syndrome[11] +/- +/- - - - - - - IgE, CD117 with CD2 Eosinophilia - ↑Serum tryptase Lymphadenopathy and splenomegaly - Intracardiac thrombi in echocardiography - Reticulin stain for myelofibrosis and tryptase staining for mast cells Clinical findings coupled with laboratory abnormalities Splinter hemorrhages, Raynaud phenomenon
Non-Hodgkin Lymphoma[12] + + + + +/- +/- +/- +/- MYCBCL2BCL6, and TP53 Lymphocytosis, Anemia, Thrombocytopenia Lactate dehydrogenase (LDH), Hypercalcemia  Enlarged lymph nodes, Hepatosplenomegaly, Filling defects in the liver and spleen - Hepatosplenomegaly Mediastinal lymphadenopathy Small cleaved or noncleaved, intermediate, or large cell with a follicular or diffuse pattern Surgically excised tissue biopsy Easy bruising, Testicular mass, Skin lesion
Serum Sickness[13] + + - +/- +/- - - +/- IL-1, IL-6, TNF Leukopenia  Polyclonal gammopathy, ↑Cr, Cryoglobulinemia - - - - Arteritic lesions are focal, necrotizing, and inflammatory involving all layers of the artery Clinical findings coupled with laboratory abnormalities Hematuria, Skin rash
Disseminated Intravascular Coagulation[14] +/- + - +/- - - + + Fibrin degradation product (FDP) Thrombocytopenia, Schistocytes D-dimer, aPTT and PT Intracranial hemorrhage - - - Ischemia and necrosis due to fibrin deposition in small and medium-sized vessels Clinical findings coupled with laboratory abnormalities  Acral cyanosis, Hemorrhagic skin infarctions
Idiopathic Thrombocytopenic Purpura[15] + +/- - + - - - + FC gamma receptors (FCGR) IIb Anemia, Thrombocytopenia - HIV, ANA R/O other causes - R/O splenomegaly - Increased number of normal morphologic megakaryocytes Clinical findings coupled with thrombocytopenia Easy bruising, Purpura
Systemic disease Sarcoidosis[16] + + + + - - - +/-  IL-2 and IFN-γ Mild anemia ACE, ↑1, 25-dihydroxyvitamin D Active alveolitis or fibrosis - Hepatosplenomegaly Bilateral hilar adenopathy Noncaseating granulomas (NCGs) Histological confirmation Heart block, Ocular lesion
Legionella Infection[17] + + + + - - - +/- Inflammatory cytokines Leukocytosis with left shift, Thrombocytosis D-dimer, FDP, Hyponatremia Pleural effusion - - Nonspecific and indistinguishable CXR Intra-alveolar inflammation, Microabscesses in the parenchyma Sputum culture Cough, Diarrhea
Systemic lupus erythematosus[18] + + + + - - + + Anti dsDNA, ANA  Leukopenia, Lymphopenia, Anemia, Thrombocytopenia Cr or BUN,

ALT or AST, Proteinuria

Interstitial lung disease, Pneumonitis, Pulmonary emboli, Alveolar hemorrhage Aneurysms Pericardial effusion, pulmonary hypertension, or verrucous Libman-Sacks endocarditis in echocardiography Central nervous system (CNS) lupus white-matter changes in MRI Staging lupus nephritis Anti-dsDNA antibody test Skin rashes or photosensitivity
Relapsing polychondritis[19] - +/- +/- + + - - - - Leukocytosis, Anemia - Cryoglobulins, ANA, C-ANCA Calcification of cartilaginous structures Aortic root dilatation Aortic root dilatation and degree of aortic regurgitation in echocardiography Tracheal stenosis in CXR Chondrolysis, Chondritis, Perichondritis Clinical findings coupled with imaging Ear pain and redness, Polyarthritis

Differentials Based On Renal Involvement

Goodpasture syndrome is associated with renal involvement in the form of rapidly progressive glomerulonephritis. The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features. The following table differentiates between various types of glomerulonephritides:[20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43]

Glomerulonephritis Sub-entity Causes and associations History and Symtoms Laboratory Findings
Hyperlipidemia and hypercholesterolemia Nephrotic features Nephritic features ANCA Anti-glomerular basement membrane antibody (Anti-GBM antibody) Immune complex formation Light microscope Electron microscope Immunoflourescence pattern
History Pitting edema Hemeturia (pre-dominantly microscopic) Hypertension Hemoptysis Oliguria Peri-orbital edema
Non-proliferative Minimal change disease

+

-

-

-

+/-

-

+

+

-

-

-

-

  • Normal

-

Focal segmental glomerulosclerosis
  • Adults
+ - - - +/- - + + - - - - -
Membranous glomerulonephritis + - - - +/- - + + - - - + -
Proliferative IgA nephropathy +/- + + - + +/- - - + - - +
  • Crescent formation
-
Rapidly progressive glomerulonephritis
  • Young adults
+/- + + + + + - - + - + + + (Linear)
+/- + + + + + - - + - - + + (Granular)
+/- + + + + + - - + + (C-ANCA) - - - (pauci-immune) +/-
+/- + + + + + - - +

+ (C-ANCA)

- - - (pauci-immune) -
+/- + + + + + - - +

+ (P-ANCA)

- - - (pauci-immune) -
Membranoproliferative glomerulonephritis +/- + + + + + - + - - - + + (Granular)

References

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  2. Kubaisi B, Abu Samra K, Foster CS (2016). "Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations". Intractable Rare Dis Res. 5 (2): 61–9. doi:10.5582/irdr.2016.01014. PMC 4869584. PMID 27195187.
  3. Ferri C, Mascia MT (January 2006). "Cryoglobulinemic vasculitis". Curr Opin Rheumatol. 18 (1): 54–63. PMID 16344620.
  4. Guo QY, Wu M, Wang YW, Sun GD (2017). "Hepatitis C virus-associated cryoglobulinemia with membrano-proliferative glomerulonephritis treated with prednisolone and interferon: A case report". Exp Ther Med. 14 (2): 1395–1398. doi:10.3892/etm.2017.4671. PMC 5525644. PMID 28810602.
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  42. Alchi B, Jayne D (August 2010). "Membranoproliferative glomerulonephritis". Pediatr. Nephrol. 25 (8): 1409–18. doi:10.1007/s00467-009-1322-7. PMC 2887509. PMID 19908070.
  43. Davis AE, Schneeberger EE, Grupe WE, McCluskey RT (May 1978). "Membranoproliferative glomerulonephritis (MPGN type I) and dense deposit disease (DDD) in children". Clin. Nephrol. 9 (5): 184–93. PMID 657595.

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