Other autoimmune diseases
Antiphospholipid Syndrome – APS
Autoimmune Hepatitis
Autoimmune Inner Ear Disease
Autoimmune Neuropathies – Neuropathic Disease
Autoimmune Thyroiditis
Celiac Disease
Cold Agglutinin Disease
Cryoglobulinemia
Inflammatory Bowel Disease – IBD
Multiple Sclerosis
Myasthenia Gravis – MG
N-methyl-D-Aspartate (NMDA)-Type Glutamate Receptor Autoantibody Disorders – Anti-NMDA-Receptor Encephalitis
Neonatal Alloimmune Thrombocytopenia – NAIT
Neuromyelitis Optica Spectrum Disorders
Primary Biliary Cholangitis – PBC
Primary Membranous Nephropathy – Idiopathic Membranous Glomerulonephritis
Antiphospholipid Syndrome – APS
Definition:
APS is an autoimmune disease which identified by thrombosis in arterial and venous. Antibodies from immune system attack phospholipids which are found in cell membranes. APS can lead to health problems, such as heart attack, kidney damage, deep vein thrombosis and pulmonary embolism. Also, it can cause multiple miscarriages and premature birth due to eclampsia that is a serious situation in pregnant women.
Panel Test:
anti-β2GP1 antibodies, IgG and IgM;
aCL antibodies, IgG and IgM
Lupus Anticoagulant Reflexive Panel
Cardiolipin Antibodies, IgG and IgM
Beta-2 Glycoprotein 1 Antibodies, IgG and IgM
High-Specificity Antiphospholipid Antibodies, IgG and IgM
Phosphatidylserine and Prothrombin Antibodies, IgG and IgM
Prothrombin Antibody, IgG
Cardiolipin Antibody, IgA
Beta-2 Glycoprotein 1 Antibody, IgA
Dilute Russell Viper Venom Time (dRVVT)
Hexagonal Phospholipid Neutralization
References
Lim W. Antiphospholipid syndrome. Hematology Am Soc Hematol Educ Program 2013; 2013:675.
Autoimmune Hepatitis
Definition:
Autoimmune hepatitis (AIH) is a chronic and inflammatory liver disease with unknown etiology. AIH can trigger as acute hepatitis and go forward to chronic liver disease and cirrhosis. Clinical symptoms and biochemistry tests are very different in acute hepatitis and cirrhosis.
Autoimmune hepatitis is identified two types:
Type 1: it’s more common type and the first onset occur at all age. This type is more related to other autoimmune disorders like celiac disease, rheumatoid arthritis and ulcerative colitis.
Type 2: it’s happened in children and young individuals.
Panel Test:
Liver function testing:
Transaminases (AST and ALT)
Hepatitis testing: important to rule out acute or chronic hepatitis
Hepatitis A virus [HAV]
Hepatitis B virus [HBV]
Hepatitis C virus [HCV])
Quantitative immunoglobulins:
IgG usually elevated
Autoantibodies:
Antinuclear antibody (ANA)
Antineutrophil cytoplasmic antibody (ANCA)
Antismooth muscle antibody (anti-SMA)
Anti-F-actin (smooth muscle) antibody
Antimitochondrial antibody (AMA)
Antisoluble liver antigen antibody (anti-SLA)
Antiliver-kidney microsomal-1 antibody (anti-LKM-1; cytochrome P450 2D6)
Antiliver cytosol (anti-LC-1)
Comment: Serum titers of antibodies do not appear to correlate with disease activity. Titers may vary during course of disease without activity correlation.
Immunogenetics:
Association with HLA-B1, B8, DR3 and DR4
References
Guidelines
Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A, Fischler B, Gupte G, Hierro L, Indolfi G, Jahnel J, Smets F, Verkade HJ, Hadzic N. Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement. J Pediatr Gastroenterol Nutr. 2018; 66(2): 345-360. PubMed
Manns MP, Czaja AJ, Gorham JD, Krawitt EL, Mieli-Vergani G, Vergani D, Vierling JM, American Association for the Study of Liver Diseases. Diagnosis and management of autoimmune hepatitis Hepatology. 2010; 51(6): 2193-213. PubMed
International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008; 48(1): 169-76. PubMed
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ, American Association for the Study of Liver Diseases. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010; 51(2): 660-78. PubMed
Gleeson D, Heneghan MA, British society of Gastroenterology. British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. Gut. 2011; 60(12): 1611-29. PubMed
European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol. 2015; 63(4): 971-1004. PubMed
Autoimmune Inner Ear Disease
Definition:
AIED is a rare autoimmune syndrome identified by progressive hearing loss. Diagnosis is mostly based on clinical manifestations and response to immunosuppressive drugs. Some patients don’t respond to steroids and may be candidates for cytotoxic therapy.
Panel Test:
Heat Shock Protein 70, IgG
Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
Antinuclear Antibodies (ANA),
Rheumatoid Factors, IgA, IgG, and IgM
Thyroid Stimulating Hormone
Free Thyroxine
Treponema pallidum Antibody, IgG
Borrelia burgdorferi Antibodies
Hemoglobin A1c
Human Immunodeficiency Virus (HIV)
C-Reactive Protein
Sedimentation Rate, Westergren (ESR)
References
Shamriz O, Tal Y, Gross M. Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan’s Syndrome. J Immunol Res. 2018 Apr 23;2018:1498640.
Autoimmune Neuropathies – Neuropathic Disease
Definition:
The immune-mediated neuropathies are a mainly group of neuropathies that often lead to severe neurologic dysfunction. Autoimmune neuropathies present with a broad range of symptom but main signs are including tingling in the feet or hands, burning, stabbing pain in affected regions, loss of balance and coordination, muscle weakness in the feet.
The overlap of symptoms among syndromes often makes diagnosis difficult. Autoimmune neuropathies can also happen as paraneoplastic disorders in the setting of cancer.
Panel Test:
Motor and Sensory Neuropathy Evaluation
Purkinje cell/neuronal nuclear IgG
neuronal nuclear (Hu, Ri, and Yo) IgG
neuronal nuclear antibody (ANNA) IgG titer
MAG IgM
SGPG Igasialo-GM1, GM1, GD1a, GD1b IgG and IgM
GQ1b abs
SPEP (Serum protein electrophoresis); immunoglobulins A, G, and M
Paraneoplastic antibodies:
ANNA-1 (Hu)
ANNA-2 (Ri)
PCCA-1 (Yo),
amphiphysin,
CV2.1 antibody.
References:
Chaudhry HM, Mauermann ML, Rajkumar V. Monoclonal Gammopathy-Associated Peripheral Neuropathy: Diagnosis and Management. Mayo Clin Proc. 2017; 92(5): 838-850. PubMed
Autoimmune Thyroiditis
Definition:
Autoimmune thyroiditis is an inflammatory condition of the thyroid gland that results from the interaction between genetic variants that promote susceptibility and environmental factors. Making antibodies to thyroid antigens leads to two main pathogenic processes with opposing clinical results: Hashimoto’s thyroiditis and Graves’ disease. High iodine intake, selenium deficiency, stress, sex steroids, smoking, pregnancy, infectious diseases, and iodine- containing drugs and radiation exposure are signified in the development of autoimmune thyroiditis, primarily in genetically susceptible people.
Panel test:
Thyroid stimulating hormone (TSH)
free T4
Antibody screening: TPO Antibodies: usually IgG1, IgG4.
TSH Receptor Antibodies (TRAb): TSH-specific receptor controls thyroid function and cell growth. TRAb targets TSH receptors and competes with TSH for receptor binding. 3 classes (IgG antibodies)
Stimulating antibodies – also known as LATS or thyroid-stimulating antibodies
Blocking antibodies – may be etiology of hypothyroidism
Neutral antibodies- This test measures both stimulating and blocking antibodies
Thyroglobulin Antibodies
Algorithm:
References
Pearce EN, Farwell AP, Braverman LE. Thyroiditis. N Engl J Med 2003; 348:2646.
Kon YC, DeGroot LJ. Painful Hashimoto’s thyroiditis as an indication for thyroidectomy: clinical characteristics and outcome in seven patients. J Clin Endocrinol Metab 2003; 88:2667.
Paes JE, Burman KD, Cohen J, et al. Acute bacterial suppurative thyroiditis: a clinical review and expert opinion. Thyroid 2010; 20:247.
McLaughlin SA, Smith SL, Meek SE. Acute suppurative thyroiditis caused by Pasteurella multocida and associated with thyrotoxicosis. Thyroid 2006; 16:307.
McAninch EA, Xu C, Lagari VS, Kim BW. Coccidiomycosis thyroiditis in an immunocompromised host post-transplant: case report and literature review. J Clin Endocrinol Metab 2014; 99:1537.
Celiac disease
Definition:
Celiac disease is usual inflammatory disease of the small intestine, triggered by an immune response to digestion of gluten and villi are injured. so, the intestine can’t absorb food nutrients. This can result to bone loss of bone density, infertility or neurological disease. Diagnosis is pathology testing based on duodenal villous atrophy, and most patients have circulating antibodies against tissue transglutaminase.
Panel Test:
Autoantibodies:
Total serum IgA
Tissue transglutaminase (tTG) antibodies (IgA, IgG)
IgA Endomysial antibody (EMA)
Deaminated gliadin peptide (DGP IgA and IgG)
Antiactin (F-Actin) IgA: Antiactin (F-actin) IgA testing by enzyme-linked immunosorbent assay (ELISA) should be performed in biopsy-confirmed cases of CD and can provide information about prognosis.
Genetic test: for human leukocyte antigens (HLA-DQ2 and HLA-DQ8) can be useful to rule out celiac disease.
References
Feuerstein JD, Nguyen GC, Kupfer SS, Falck-Ytter Y, Singh S, American Gastroenterological Association Institute Clinical Guidelines Committee. American Gastroenterological Association Institute Guideline on Therapeutic Drug Monitoring in Inflammatory Bowel Disease. Gastroenterology. 2017; 153(3): 827-834. PubMed
World Gastroenterology Organisation Global Guidelines – Celiac Disease. World Gastroenterology Organisation. Milwaukee, WI [Accessed: Jan 2018]
Cold Agglutinin Disease
Definition:
CAD is a type of autoimmune hemolytic anemia (AIHA) in which autoantibodies (cold agglutinins) at temperatures below 30°C bind to RBC antigens and can cause agglutination of the RBCs and extravascular hemolysis. It happens more in the age of 55. A cold environment may start acute hemolysis with hemoglobinemia and hemoglobinuria.
Panel Test:
Direct Coombs (Anti-Human Globulin)
Cold Agglutinins
Complement Components 3 and 4
Complement Activity
Immunoglobulins (IgA, IgG, IgM),
CBC and Automated Differential
Reticulocytes, Percent and Number
Bilirubin, Direct,
Lactate Dehydrogenase,
References
Berentsen S. How I manage cold agglutinin disease. Br J Haematol 2011; 153:309.
Berentsen S, Ulvestad E, Langholm R, et al. Primary chronic cold agglutinin disease: a population based clinical study of 86 patients. Haematologica 2006; 91:460.
Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013; 122:1114.
Cryoglobulinemia
Definition:
Cryolobulinemia is a group of rare disorder that result damage of the blood vessels and vital organs. The presence of cold sensitive immunoglobulins restricts blood flow in skin, muscles, nerves and kidneys. These abnormal proteins are in patients who have autoimmune disease or hepatitis C virus (type II and III or mixed cryoglobulinemia). Type I cyroglubulinemia is related to leukemia, multiple mylemoa, rheumatoid arthritis and SLE.
Panel Test:
Cryoglobulin, (IgA, IgG, and IgM)
C-Reactive Protein
Protein Electrophoresis, Serum
Sedimentation Rate, Westergren (ESR)
Creatinine, Serum or Plasma Urinalysis
Urea Nitrogen, Serum or Plasma
Rheumatoid Factor
Complement Activity (CH50)
Complement Activity, Alternative Pathway (AH50)
Connective Tissue Diseases tests:
Smith (ENA),
Sm/RNP,
SSA, SSB,
Jo-1, RPP,
centromere and Scl-70 antibodies
Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodie
Autoimmune Liver Disease:
Smooth Muscle Antibody (SMA), IgG (IFA)
LC-1 and soluble liver antigen (SLA)
mitochondrial M2 antibody, IgG;
liver-kidney microsome-1 antibody, IgG;
F-actin (smooth muscle) antibody, IgG
Hepatitis Virus test:
HAV IgM,
HBV core antibody IgM
HBV surface antigen
HCV antibody (if results is positive it’s must be confirm by molecular test)
Human Immunodeficiency Virus (HIV)
Hepatic Function:
albumin;
ALP; AST; ALT;
bilirubin, direct; and bilirubin, total
protein, total;
Thyroid function
T3
T4
TSH
References
Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012; 379:348.
Inflammatory Bowel Disease – IBD
Definition:
IBD is an inflammatory disorder in digestive tract. Two main and common types of disease are Crohn’s Disease and Ulcerative Colitis. In UC, long inflammation causes sores or ulcers in the large intestine, colon and rectum. CD is identified by inflammation in digestive tract which sometimes develops deep into intestinal tissues. Patients affected by these diseases experience abdominal symptoms, including diarrhea, abdominal pain, bloody stools, fatigue, weight loss and vomiting.
Panel tests:
Autoantibodies:
ANCA test: Anti-neutrophil cytoplasmic antibody (ANCA) is classified according to two staining patterns:
cytoplasmic ANCA (cANCA): in which the entire cytoplasm is stained
perinuclear ANCA (pANCA): in which the area around the nucleus is stained.
pANCA (Perinuclear anti-neutrophil cytoplasmic antibody). More common with UC, it is found in about 60% to 80% of people with UC but only about 5% to 15% of people with CD.
Antimicrobial antibodies:
Anti-Saccharomyces cerevisiae antibody (ASCA),
Anti-CBir1 (Clostridium species antibodies).
Anti-Omp C (Escherichia coli antibodies).
Anti-I-2 (Pseudomonas fluorescens antibodies)
Calprotectin
Stool exam and stool culture
Lactoferrin
CBC with automated differential
CRP and ESR
Albumin
ALT and AST
References
Lamb CA, Kennedy NA, Raine T, et al. British Society of Gastroenterology consensus guidelines on the management of inflammatory bowel disease in adults. Gut 2019;68:s1-s106.
Multiple Sclerosis
Definition:
Multiple sclerosis (MS) is a chronic inflammatory disease pftem potentially disabling, demyelinating and neurodegenerative of the brain and spinal cord in young adults. This disorder is a heterogeneous, multifactorial, immune-mediated disease that is influenced by both genetic and environmental factors. Multiple sclerosis symptoms could be differing greatly in patients and over the course of the disease depending on the location and the amount of damage in affected nerve fibers. MS symptoms include visual impairments, tingling and numbness, episodic bouts of fatigue, intestinal and urinary system disorders, spasticity, and learning and memory impairment.
Panel test:
Oligoclonal Band in CSF and Serum
IgG – serum, CSF, index
Albumin – CSF, serum by nephelometry, index
CSF – IgG/albumin ratio, IgG synthesis rate, OCBs
Aquaporin-4 Receptor Antibody: Aid in evaluation of Neuromyelitis optica (NMO) and NMO spectrum disorders
Anti- myelin oligodendrocyte glycoprotein (MOG)
Interferon Beta Neutralizing Antibody
Natalizumab Antibodies
Reference:
Thompson AJ, Banwell BL, Barkhof F, Carroll WM, Coetzee T, Comi G, Correale J, Fazekas F, Filippi M, Freedman MS, Fujihara K, Galetta SL, Hartung HP, Kappos L, Lublin FD, Marrie RA, Miller AE, Miller DH, Montalban X, Mowry EM, Sorensen PS, Tintoré M, Traboulsee AL, Trojano M, Uitdehaag BM, Vukusic S, Waubant E, Weinshenker BG, Reingold SC, Cohen JA. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 2018; 17(2): 162-173. PubMed
Myasthenia Gravis – MG
Definition:
Myasthenia gravis is a chronic neuromuscular disease in which antibodies attached to acetylcholine receptors which is critical factor for relation between nerve and muscles.
The main symptom is weakness of skeletal muscles, talking problems, problems lifting objects or arising stairs. The weakness typically increases with exercise and repetitive muscle use (fatigue) and varies over the course of a day and from day to day.
Panel test:
Autoantibodies:
Acetylcholine receptor (AChR) antibodies: There are three types of AChR antibodies: binding (most frequently tested), blocking, and modulating.
Anti-MuSK (muscle-specific kinase) antibodies
Anti-striated muscle antibodies
Titin antibodies: Rheumatoid factor (RF)
Cyclic citrullinated peptide antibody (CCP)
Antinuclear antibody (ANA)
Thyroid panel and thyroid antibodies
Immunogenetic:
HLA-A1, B8, DR3, HLA-Bw35/DR1 (induced by penicillamine)
Reference:
Gilhus NE. Myasthenia Gravis. N Engl J Med. 2016; 375(26): 2570-2581. PubMed
Li Y, Arora Y, Levin K: Myasthenia gravis: Newer therapies offer sustained improvement. Cleve Clin J Med 2013 Nov;80(11):711-721.
Skjei KL, Lennon VA, Kuntz NL: Muscle specific kinase autoimmune myasthenia gravis in children: A case series. Neuromuscul Disord 2013 Nov;23(11):874-882.
N-methyl-D-Aspartate (NMDA)-Type Glutamate Receptor Autoantibody Disorders – Anti-NMDA-Receptor Encephalitis
Definition:
Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a form of encephalitis associated with antibodies against NR1 or NR2 subunits of the NMDA receptor.
Affected individuals typically develop prominent psychiatric symptoms, cognitive dysfunction, seizures, orofacial dyskinesias, and autonomic instability (8, 9).
Panel test:
Autoantibodies:
NDMA receptor antibody
glutamic acid decarboxylase (GAD) antibody
VGKC antibody
aquaporin-4 receptor antibody
aquaporin-4 receptor antibody
LGI1 antibody
CASPR2 antibody
AMPA receptor antibody
GABA receptor antibody
MOG antibody
Voltage-gated calcium channel (VGCC) antibody
Acetylcholine receptor modulating antibody
Titin antibody
Striated muscled antibody
Reference:
Venkatesan A, Tunkel AR, Bloch KC, Lauring AS, Sejvar J, Bitnun A, Stahl J, Mailles A, Drebot M, Rupprecht CE, Yoder J, Cope JR, Wilson MR, Whitley RJ, Sullivan J, Granerod J, Jones C, Eastwood K, Ward KN, Durrheim DN, Solbrig MV, Guo-Dong L, Glaser CA, International Encephalitis Consortium. Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. Clin Infect Dis. 2013; 57(8): 1114-28. PubMed
Neonatal Alloimmune Thrombocytopenia – NAIT
Definition:
NAIT is characterized by reduced platelet count less than 150000/µL following from maternal/fetal platelet antigens incompatible. Two mechanisms have been suggested to pathogenesis of NAIT. The first one mechanism is maternal exposure to human platelet antigen [HPA]-1a on fetal platelets lead to fetomaternal bleeding or on adult platelets due to previous platelet transfusions, the other on is maternal exposure to integrinb-3 on placental cells within pregnancy.
Panel Test:
Routine test:
CBC with Platelet Count
Platelet Antibodies, Indirect
Platelet Antigen Genotyping
Genetics test:
HPA genes GP1BA, ITGA2B, ITGB3, ITGA2, ITGB3, and CD109
References
Curtis BR. Recent progress in understanding the pathogenesis of fetal and neonatal alloimmune thrombocytopenia. Br J Haematol 2015; 171:671.
Neuromyelitis Optica Spectrum Disorders
Definition:
NMOSD or Devic disease is an autoimmune disorder of the CNS and identified by demyelination and damage of optic nerves and spinal cord and often mistaken with multiple sclerosis (MS). Specific anti-NMO antibodies in serum of patient to aquaporin-4 (AQP4) are diagnostic and complement system is cause of demyelination in NMOSD. NMOSD could be related to SLE, Sjogren’s syndrome, vasculitis or mixed connective tissue disease.
Panel Test:
Autoantibodies:
Aquaporin-4 Receptor Antibody (serum and CSF),
Myelin Oligodendrocyte Glycoprotein (MOG) Antibody,
CSF Analysis
Cell Count, CSF
Oligoclonal Band Profile
References
Jarius S, Aboul-Enein F, Waters P, et al. Antibody to aquaporin-4 in the long-term course of neuromyelitis optica. Brain 2008; 131:3072.
Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol 2010; 17:1019.
Bennett JL, Lam C, Kalluri SR, et al. Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. Ann Neurol 2009; 66:617.
Papadopoulos MC, Verkman AS. Aquaporin 4 and neuromyelitis optica. Lancet Neurol 2012; 11:535.
Hinson SR, McKeon A, Lennon VA. Neurological autoimmunity targeting aquaporin-4. Neuroscience 2010; 168:1009.
Primary Biliary Cholangitis – PBC
Definition:
Primary biliary cholangitis (PBC), previously referred to as primary biliary cirrhosis, is an autoimmune and chronic liver disorder in which bilary ducts are damaged. PBC is more common in women than in men, and usually arises at 40-50 years of age. Symptoms of PBC may include chronic itchy skin, fatigue, dry eyes and mouth, abdominal pain, jaundice, and arthralgia. The combination of the following factors could increase incidence of PBC:
genetics, sex, age, environment factors such as smoking, infections, toxic materials.
Panel Test:
Hepatic Function tests:
Albumin
ALP, AST, ALT,
bilirubin direct and total
protein
CBC and Automated Differential
Prothrombin Time/INR
Autoantibodies:
Autoimmune Liver Disease:
AMA, IgG
liver-kidney microsome-1 antibody, IgG
F-actin (smooth muscle) antibody, IgG
SMA, IgG
Mitochondrial M2 antibody, IgG
Antinuclear antibody (ANA) with HEp-2
Centromere antibody, IgG
Immunoglobulin Tests
Immunoglobulin M and G (IgG, IgM)
Other Serologic Tests:
HBV antigen
HCV antibody
Hyaluronic Acid, Serum
Cholesterol
Thyroid Stimulating Hormone
Vitamin A (Retinol)
Vitamin D, 25-Hydroxy
Vitamin E, Serum or Plasma
Prothrombin Time/International Normalized Ratio
References
Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019; 69(1): 394-419. PubMed
Hirschfield GM, Dyson JK, Alexander GJ, Chapman MH, Collier J, Hübscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones DE. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018; 67(9): 1568-1594. PubMed
European Association for the Study of the Liver (EASL). EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017; 67(1): 145-172. PubMed
Bowlus CL, Gershwin E. The diagnosis of primary biliary cirrhosis. Autoimmun Rev. 2014; 13(4-5): 441-4. PubMed
Liberal R, Grant CR, Sakkas L, Bizzaro N, Bogdanos DP. Diagnostic and clinical significance of anti-centromere antibodies in primary biliary cirrhosis. Clin Res Hepatol Gastroenterol. 2013; 37(6): 572-85. PubMed
Kwo PY, Cohen SM, Lim JK. ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries. Am J Gastroenterol. 2017; 112(1): 18-35. PubMed
Primary Membranous Nephropathy – Idiopathic Membranous Glomerulonephritis
Definition:
PMN is an autoimmune disorder mediated by antigen-antibody complex in which autoantibodies against to antigens in kidney leading to glomerulopathy. The antigens are: NEP (neutral endopeptidase, M-type phopholipase A2 receptor (PLA2R) and thrombospodin type 1 domain containing 7A (THSD7A). Deposition of immune complexes between the layers of glomerulus activate the inflammation and lead to kidney damage. Diagnosis of PMN is based on kidney in which there are GBM thickening and deposition of IgG and C3 in glomerular capillary loops under immunofluorescence.
Panel Test:
Protein, Total, Urine
Albumin (Nephelometry)
Phospholipase A2 Receptor (PLA2R) Antibody, IgG
Antinuclear Antibodies (ANA), IgG
Hepatitis B Virus Surface Antigen
Hepatitis C Virus Antibody by CIA
References
Fogo AB, Lusco MA, Najafian B, Alpers CE. AJKD Atlas of Renal Pathology: Membranous Nephropathy. Am J Kidney Dis 2015; 66:e15.
Debiec H, Ronco P. Immunopathogenesis of membranous nephropathy: an update. Semin Immunopathol 2014; 36:381.
Cattran DC, Brenchley PE. Membranous nephropathy: integrating basic science into improved clinical management. Kidney Int 2017; 91:566.
Xu X, Wang G, Chen N, et al. Long-Term Exposure to Air Pollution and Increased Risk of Membranous Nephropathy in China. J Am Soc Nephrol 2016; 27:3739.
