Vasculitic Disease
Antiglomerular Basement Membrane Disease – Goodpasture Syndrome
Behçet Syndrome
Cogan Syndrome Vasculitis
Cryoglobulinemia
Eosinophilic Granulomatosis with Polyangiitis – EGPA
Giant Cell Arteritis – Temporal Arteritis
Granulomatosis with Polyangiitis – GPA
IgA Vasculitis – Henoch Schönlein Purpura
Microscopic Polyangiitis – MPA
Polyarteritis Nodosa – PAN
Systemic Vasculitis
Takayasu Arteritis
Vasculitic Disease
Antiglomerular Basement Membrane Disease – Goodpasture Syndrome
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:
Routine tests:
CBC with Automated Differential
BUN and Creatinine
Urine analysis for casts
CRP and ESR
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
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.
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.
Pusey CD. Anti-glomerular basement membrane disease. Kidney Int 2003; 64:1535.
Behçet Syndrome
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.
CBC and Automated Differential
CRP and ESR
C9 level in serum: It’s often increased.
vWF (von Willebrand factor: the level of vWF is increasing
cryoglobulin
Anti-cardiolipin antibody
ANCA or Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
References
EULAR/EUVAS: Recommendations for the management of Behçet disease (2008).
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.
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.
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.
Yazici H, Seyahi E, Yurdakul S. Behçet’s syndrome is not so rare: why do we need to know? Arthritis Rheum 2008; 58:3640.
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.
Mahr A, Maldini C. [Epidemiology of Behçet’s disease]. Rev Med Interne 2014; 35:81.
Cogan Syndrome Vasculitis
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:
CBC with Automated Differential
BUN and Creatinine
Urine analysis for casts
CRP and ESR
ANCA or Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
ANA
RPR
HIV
References
St Clair EW, McCallum RM. Cogan’s syndrome. Curr Opin Rheumatol 1999; 11:47.
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.
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.
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 confirming by molecular test)
Human Immunodeficiency Virus (HIV)
Hepatic Function:
albumin;
ALP; AST; ALT;
bilirubin, direct; and bilirubin, total
protein, total;
Thyroid function
References
Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012; 379:348.
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.
Quartuccio L, Isola M, Corazza L, et al. Validation of the classification criteria for cryoglobulinaemic vasculitis. Rheumatology (Oxford) 2014; 53:2209.
Eosinophilic Granulomatosis with Polyangiitis – EGPA
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:
CBC with Automated Differential
Urine analysis
CRP and ESR
ANCA or Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
References
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.
Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med 2017; 376:1921.
Giant Cell Arteritis – Temporal Arteritis
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:
CBC with Automated Differential
Urine analysis
CRP and ESR
ANCA or Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
Hepatic function
References
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.
Linxweiler M, Hasenfus A, Wolf G, Schick B. Perivascular marginal zone lymphoma mimicking temporal arteritis. Otolaryngol Head Neck Surg 2015; 152:187.
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.
Granulomatosis with Polyangiitis – GPA
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:
CBC with Automated Differential
Urine analysis
CRP and ESR
ANCA or Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
References
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.
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.
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.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
IgA Vasculitis (IgAV) – Henoch Schönlein Purpura (HSP)
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:
CBC with Automated Differential
BUN and Creatinine
Urine analysis for casts
CRP and ESR
ANCA or Anti-Neutrophil Cytoplasmic Antibody
Immunoglobulin A
References
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol 2013; 25:171.
Bonetto C, Trotta F, Felicetti P, et al. Vasculitis as an adverse event following immunization – Systematic literature review. Vaccine 2016; 34:6641.
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.
Microscopic Polyangiitis – MPA
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:
calcium,
carbon dioxide,
chloride,
creatinine,
glucose,
potassium,
sodium,
CBC with Platelet Count and Automated Differential
Urinalysis, Complete
Urea Nitrogen, Serum or Plasma
Creatinine, Serum or Plasma
C-Reactive Protein and Sedimentation Rate, Westergren (ESR)
ANCA or Anti-Neutrophil Cytoplasmic Antibody
Glomerular Basement Membrane Antibody, IgG
Myeloperoxidase (MPO) Antibody
References
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.
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.
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.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
Polyarteritis Nodosa – PAN
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:
CBC with Automated Differential
Urinalysis,
C-Reactive Protein and ESR
MPO/PR3 Antibodies
Hepatitis Virus tests:
hepatitis A virus (HAV) IgM,
hepatitis B virus (HBV) core antibody IgM,
HBV surface antigen (HBsAg), and
hepatitis C virus (HCV) antibody;
Antinuclear Antibodies (ANA), IgG
Glomerular Basement Membrane Antibody, IgG
References
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
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.
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:
ANCA- (antineutrophil cytoplasmic antibody)
MPO antibodies, IgG;
serine PR3, IgG
CBC and Automated Differential
Renal Function tests:
albumin,
calcium,
carbon dioxide,
creatinine,
chloride,
glucose,
phosphorous,
potassium,
sodium,
blood urea nitrogen (BUN),
Urinalysis, Complete
Hepatic Function:
bilirubin, direct; bilirubin, total;
alkaline phosphatase; aspartate aminotransferase; alanine aminotransferase;
protein, total;
albumin (serum or plasma)
CRP and ESR
Cryoglobulin,
Glomerular Basement Membrane Antibody, IgG
Prothrombin Time
Rickettsia rickettsii (Rocky Mountain Spotted Fever) Antibodies, IgG & IgM
Hepatitis tests:
hepatitis A virus (HAV) IgM,
hepatitis B virus (HBV) core antibody IgM,
HBV surface antigen,
hepatitis C virus (HCV) antibody
Human Immunodeficiency Virus (HIV) C
Rapid Plasma Reagin (RPR)
Varicella-Zoster Virus Antibodies, IgG and IgM
Antinuclear Antibody (ANA) with HEp-2 Substrate
Double-Stranded DNA (dsDNA) Antibody, IgG by
Extractable Nuclear Antigen Antibodies (Smith/RNP, Smith, SSA 52, SSA 60, and SSB)
Rheumatoid Arthritis Panel
Beta-2 Glycoprotein 1 Antibodies, IgG and IgM
Cardiolipin Antibodies, IgG and IgM
Blood Smear with Interpretation
Angiotensin Converting Enzyme, Serum
References
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
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)
Takayasu Arteritis
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:
C-Reactive Protein and ESR
Anti-Neutrophil Cytoplasmic Antibody
MPO/PR3 Antibodies
Urinalysis, Complete
CBC with Platelet Count and Automated Differential
References
Kerr GS. Takayasu’s arteritis. Rheum Dis Clin North Am 1995; 21:1041.
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.
Hata A, Numano F. Magnetic resonance imaging of vascular changes in Takayasu arteritis. Int J Cardiol 1995; 52:45.
