آزمایشگاه ملل شهرک سلامت اصفهان

EN

Vasculitic Disease
Vasculitic Disease

 

Definition:

Goodpasture syndrome is a rare serious disease in which immune system attacks the kidneys and lungs mistakenly. First onset is sudden and may be like flu symptoms and arthralgia. This disease is related to other autoimmne disease such as wegner’s granulomatosis, thyroid disease, Bhecet’s disease, celiac disease, IBD, lymphoma, thymoma and other malignancies.

 

Panel Test:

 

  • Immunopathology:
  • Anti-GBM antibodies
  • Type IV collagen
  • Deposition of C3 (linear staining)

 

  • Immunogenetics:
  • It’s strongly associated with DRw15(DR2)/DQw6, DR4/DQw7

 

  • Autoantibodies:
  • Anti-GBM antibodies: About 90% of patients are positive and it can help for monitoring of therapy
  • ANCA: for screening vasculitis

 

 

References

  1. Olson SW, Arbogast CB, Baker TP, et al. Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease. J Am Soc Nephrol 2011; 22:1946.
  2. Canney M, O’Hara PV, McEvoy CM, et al. Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol 2016; 11:1392.
  3. Pusey CD. Anti-glomerular basement membrane disease. Kidney Int 2003; 64:1535.

 

 

 

Definition:

Behcet disease is an inflammatory disorder that result wide inflammation of veins and arteries (vasculitis) especially small blood vessels in geneital, mouth, skin and eyes. The main clinical manifestation in patients is recurrent painful ulcers in genital track, sores and aphthous in gastrointestinal tracks (more in the tonsils, throat, lips, tongue and the roof of the mouth), skin lesions or artheritis. Inflammation of the eyes (uveitis) is detected in most of patients especially in younger people.

 

 

Panel Test:

There are not definit test for diagnosis. Diagnosis is based on clinical signs.

 

 

 

References

  1. EULAR/EUVAS: Recommendations for the management of Behçet disease(2008).
  1. Mahr A, Belarbi L, Wechsler B, et al. Population-based prevalence study of Behçet’s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum 2008; 58:3951.
  2. Dilek K, Ozçimen AA, Saricaoğlu H, et al. Cytokine gene polymorphisms in Behçet’s disease and their association with clinical and laboratory findings. Clin Exp Rheumatol 2009; 27:S73.
  3. Mahr A, Belarbi L, Wechsler B, et al. Population-based prevalence study of Behçet’s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum 2008; 58:3951.
  4. Yazici H, Seyahi E, Yurdakul S. Behçet’s syndrome is not so rare: why do we need to know? Arthritis Rheum 2008; 58:3640.
  5. Calamia KT, Wilson FC, Icen M, et al. Epidemiology and clinical characteristics of Behçet’s disease in the US: a population-based study. Arthritis Rheum 2009; 61:600.
  6. Mahr A, Maldini C. [Epidemiology of Behçet’s disease]. Rev Med Interne 2014; 35:81.

 

Definition:

Cogan’s syndrome is a rare and chronic immune system mediated disease that mainly affects young people. This disorder characterized by interstitial keratitis, intraocular inflammation, audio vestibular symptoms and hearing loss. The cause of this syndrome is unknown but there were evidences that it relates to Chlamydia and Borrelia infections.

 

Panel Test:

 

 

References

  1. St Clair EW, McCallum RM. Cogan’s syndrome. Curr Opin Rheumatol 1999; 11:47.
  2. Jung DH, Nadol JB Jr, Folkerth RD, Merola JF. Histopathology of the Inner Ear in a Case with Recent Onset of Cogan’s Syndrome: Evidence for Vasculitis. Ann Otol Rhinol Laryngol 2016; 125:20.
  3. Tayer-Shifman OE, Ilan O, Tovi H, Tal Y. Cogan’s syndrome–clinical guidelines and novel therapeutic approaches. Clin Rev Allergy Immunol 2014; 47:65.

 

 

 

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:

 

References

  1. Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012; 379:348.
  2. Giuggioli D, Manfredi A, Lumetti F, et al. Cryoglobulinemic vasculitis and skin ulcers. Our therapeutic strategy and review of the literature. Semin Arthritis Rheum 2015; 44:518.
  3. Quartuccio L, Isola M, Corazza L, et al. Validation of the classification criteria for cryoglobulinaemic vasculitis. Rheumatology (Oxford) 2014; 53:2209.

 

 

 

 

Definition:

Also known Churg-strauss syndrome, is a scare autoimmune disorder that causes inflammation in small and medium sized blood vessels. The lungs, nose, sinuses, skins, skin, joints, nerves, intestinal tract, heart and kidney are injured. Most of the people have a history of asthma and allergies. Almost eosinophilia is in peripheral blood EGPA patients and there is glanulomas in the most tissue samples.

 

Panel Test:

 

 

 

References

  1. Puéchal X, Pagnoux C, Baron G, et al. Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial. Arthritis Rheumatol 2017; 69:2175.
  2. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med 2017; 376:1921.

 

 

 

Definition:

Giant cell arteritis or temporal arteritis is a common form of vasculitis in which inflammation affects narrowing or blockage of the vessels in head, neck and arms. GCA is happened in older people and the average age at first onset is 72.

 

Panel Test:

 

 

 

References

  1. Kermani TA, Schäfer VS, Crowson CS, et al. Increase in age at onset of giant cell arteritis: a population-based study. Ann Rheum Dis 2010; 69:780.
  2. Linxweiler M, Hasenfus A, Wolf G, Schick B. Perivascular marginal zone lymphoma mimicking temporal arteritis. Otolaryngol Head Neck Surg 2015; 152:187.
  1. Crowson CS, Matteson EL, Myasoedova E, et al. The lifetime risk of adult-onset rheumatoid arthritis and other inflammatory autoimmune rheumatic diseases. Arthritis Rheum 2011; 63:633.

 

 

Definition:

Also named Wegner’s, is a rare inflammatory disorder with unknown source that causes blood vessel damage in organs. The reduced blood flow in organs can develop areas of inflammation and necrosis named granulomas which can affect organs action.

 

Panel Test:

 

 

References

  1. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum 2011; 63:863.
  2. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. J Am Soc Nephrol 2011; 22:587.
  3. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Ann Rheum Dis 2011; 70:704.
  4. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.

 

 

Definition:

It’s a the most common form of systemic vasculitis which affects capillaries in the kidney and skin. In HSP deposition of immunoglobulin A (IgA1) and C3 (complement) demonstrate on the vein walls. The renal injury could be mediated by IgA and IgG autoantibodies against mesangial cell antigens. IgAV occurs often in children more than in adults but involvement in kidney is more occur and more severe in older adults.

 

 

Panel Test:

 

References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  2. Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol 2013; 25:171.
  3. Bonetto C, Trotta F, Felicetti P, et al. Vasculitis as an adverse event following immunization – Systematic literature review. Vaccine 2016; 34:6641.
  4. Woerner A, Rudin C, Bonetto C, et al. IgA vasculitis (Henoch-Schönlein): Case definition andguidelines for data collection, analysis, and presentation of immunisation safety data. Vaccine 2017; 35:1559.

 

 

Definition:

MPA is an uncommon disorder results from blood vessel inflammation that can cause damage tor organs such as kidney, lung, nerve and joint. People of all ages can affect MPA and appears in both sexes equally. Laboratory evidences strongly support that the immune system has a censorious role in MPA.

 

Panel Test:

 

 

References

  1. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum 2011; 63:863.
  2. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. J Am Soc Nephrol 2011; 22:587.
  3. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Ann Rheum Dis 2011; 70:704.
  4. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.

 

 

Definition:

PAN is an idiopathic systemic vasculitis identified by necrotizing lesions in involved organs such as kidneys, muscles, joints, nerves, skin and gastrointestinal track. PAN is classified in to subacute, acute and chronic stages. It’s a similar to many diseases like Hepatitis B and C infections, Churg-strauss syndrome, cogan’s syndrome and kawaski disease.

 

Panel Test:

 

References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  2. Ribi C, Cohen P, Pagnoux C, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: A prospective randomized study of one hundred twenty-four patients. Arthritis Rheum 2010; 62:1186.
  3. Puéchal X, Pagnoux C, Baron G, et al. Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial. Arthritis Rheumatol 2017; 69:2175.

 

  • Systemic vasculiits

 

Definition:

Vasculitis is a sort of rare inflammatory disorders and immune-mediated in blood vessels.  Vasculitis can lead to reduced blood flow to tissues like nerves, skin and lungs. These changes can result serious damage in the critical organs. Vasculitis is often serious disease and it can be sometimes fatal. Vasculitis is a systemic disease so, patients have fever, weight loss, fatigue and pain.

 

Panel Test:

 

References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  2. American Society of Clinical Pathologists (ASCP): Addendum to the international consensus statement on testing and reporting of antineutrophil cytoplasmic antibodies − Quality control guidelines, comments, and recommendations for testing in other autoimmune diseases (2003)

 

 

Definition:

Takayasu arteritis is a rare type of granulomatous vasculitis in large vessel such as aorta and its major branches. Inflammation lead to wall thicknening, occlusive lesions and thrombosis. The disease can lead to arm or chest pain, high blood pressure and heart failure. Commonly woman younger than age 50 years are affected and the first onset is usually between 10 and 40 years.

 

Panel Test:

 

 

References

  1. Kerr GS. Takayasu’s arteritis. Rheum Dis Clin North Am 1995; 21:1041.
  2. Misra R, Danda D, Rajappa SM, et al. Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010). Rheumatology (Oxford) 2013; 52:1795.
  3. Hata A, Numano F. Magnetic resonance imaging of vascular changes in Takayasu arteritis. Int J Cardiol 1995; 52:45.

 

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:

 

References

  1. Lim W. Antiphospholipid syndrome. Hematology Am Soc Hematol Educ Program 2013; 2013:675.

 

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

  1. 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
  2. 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 2010; 51(6): 2193-213. PubMed
  3. International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. 2008; 48(1): 169-76. PubMed
  4. 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. 2010; 51(2): 660-78. PubMed
  5. Gleeson D, Heneghan MA, British society of Gastroenterology. British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. 2011; 60(12): 1611-29. PubMed
  6. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis.J Hepatol. 2015; 63(4): 971-1004. PubMed

 

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:

 

References

  1. 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.

 

 

 

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,
  • 1 antibody.

 

 

References:

  1. Chaudhry HM, Mauermann ML, Rajkumar V. Monoclonal Gammopathy-Associated Peripheral Neuropathy: Diagnosis and Management.Mayo Clin Proc. 2017; 92(5): 838-850. PubMed

 

 

 

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

  1. Pearce EN, Farwell AP, Braverman LE. Thyroiditis. N Engl J Med 2003; 348:2646.
  2. 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.
  3. Paes JE, Burman KD, Cohen J, et al. Acute bacterial suppurative thyroiditis: a clinical review and expert opinion. Thyroid 2010; 20:247.
  4. McLaughlin SA, Smith SL, Meek SE. Acute suppurative thyroiditis caused by Pasteurella multocida and associated with thyrotoxicosis. Thyroid 2006; 16:307.
  5. 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)

o   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

 

 

References

  1. 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. 2017; 153(3): 827-834. PubMed
  2. World Gastroenterology Organisation Global Guidelines – Celiac Disease. World Gastroenterology Organisation. Milwaukee, WI [Accessed: Jan 2018]

 

 

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:

 

References

  1. Berentsen S. How I manage cold agglutinin disease. Br J Haematol 2011; 153:309.
  2. 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.
  3. Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013; 122:1114.

 

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:

 

 

References

  1. Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012; 379:348.

 

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 cerevisiaeantibody (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

  1. 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.

 

 

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

 

Reference:

 

  1. 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

 

 

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:

 

 

 

  • Immunogenetic:
  • HLA-A1, B8, DR3, HLA-Bw35/DR1 (induced by penicillamine)

 

Reference:

  1. Gilhus NE. Myasthenia Gravis.N Engl J Med. 2016; 375(26): 2570-2581. PubMed
  1. Li Y, Arora Y, Levin K: Myasthenia gravis: Newer therapies offer sustained improvement. Cleve Clin J Med 2013 Nov;80(11):711-721.
  2. 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.

 

 

 

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:

  1. 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

 

 

 

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:

References

  1. Curtis BR. Recent progress in understanding the pathogenesis of fetal and neonatal alloimmune thrombocytopenia. Br J Haematol 2015; 171:671.

 

 

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:

 

 

 

References

  1. 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.
  2. Sellner J, Boggild M, Clanet M, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol 2010; 17:1019.
  3. Bennett JL, Lam C, Kalluri SR, et al. Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. Ann Neurol 2009; 66:617.
  4. Papadopoulos MC, Verkman AS. Aquaporin 4 and neuromyelitis optica. Lancet Neurol 2012; 11:535.
  5. Hinson SR, McKeon A, Lennon VA. Neurological autoimmunity targeting aquaporin-4. Neuroscience 2010; 168:1009.

 

 

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:

 

  • 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

 

 

 

References

  1. 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. 2019; 69(1): 394-419. PubMed
  2. 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. 2018; 67(9): 1568-1594. PubMed
  3. 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
  4. Bowlus CL, Gershwin E. The diagnosis of primary biliary cirrhosis.Autoimmun Rev. 2014; 13(4-5): 441-4. PubMed
  5. 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
  6. Kwo PY, Cohen SM, Lim JK. ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries.Am J Gastroenterol. 2017; 112(1): 18-35. PubMed

 

 

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:

 

References

  1. Fogo AB, Lusco MA, Najafian B, Alpers CE. AJKD Atlas of Renal Pathology: Membranous Nephropathy. Am J Kidney Dis 2015; 66:e15.
  2. Debiec H, Ronco P. Immunopathogenesis of membranous nephropathy: an update. Semin Immunopathol 2014; 36:381.
  3. Cattran DC, Brenchley PE. Membranous nephropathy: integrating basic science into improved clinical management. Kidney Int 2017; 91:566.
  4. 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.

دیدگاهتان را بنویسید

نشانی ایمیل شما منتشر نخواهد شد. بخش‌های موردنیاز علامت‌گذاری شده‌اند *

تمامی حقوق مادی و معنوی این تارنما متعلق به آزمایشگاه ملل است.