Autoimmune Screen

<Pathology>

<Index>

 

 

-  Disease Reference

 

-  Connective Tissue

-  Gastrointestinal

-  Endocrine

-  Neurological

-  Renal

-  Vascular

-  Other

 

 

 

Connective Tissue

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-  ANA

-  ENA

-  RF

-  Antiphospholipid

 

Anti-Nuclear Abs (ANA) <Up>

-  > 90% pts w SLE

-  Also found in juvenile chronic arthritis, Sjogrens syndrome, fibrosing alveolitis, chronic active hepatitis, viral infections (EBV, CMV..), drug reactions

-  Low titre present in normals (increases w age)

-  Anti-Nuclear Ab (Speckled Pattern)

-  SLE, Mixed Connective Tissue Disease (MCTD), Sjogrens syndrome, Scleroderma

-  Anti-Ribosomal Ab

-  RA, 10-15% SLE

-  Levels correlate w disease activity

-  Anti-Nucleolar Ab

-  Scleroderma, SLE, polymyositis

-  Anti-dsDNA Ab

-  40-70% pts w SLE

-  Anti-Centromere Ab

-  Found in CREST variant of scleroderma or limited cutaneous scleroderma

-  12% pts w 1o biliary cirrhosis

-  50% of these have S/S of scleroderma

-  Anti-Histone Ab

-  18-50% w SLE

-  95% drug induced SLE

 

Anti-Extractable Nuclear Antigen Ab (ENA) <Up>

-  Anti-Ribonucleoprotein Ab

-  SLE & Mixed Connective Tissue Disease (MCTD)

-  Anti-sm Ab

-  Specific for SLE

-  Anti-Ro (SS-A) Ab

-  1o Sjogrens syndrome

-  Subacute cutaneous lupus, neonatal lupus, congenital complete heart block (babies born to SLE mothers), SLE with interstitial pneumonitis

-  Anti-La (SS-B) Ab

-  1o Sjogrens syndrome & SLE

-  Anti-Jo-1 (Histidyl tRNA synthetase) Ab

-  20-40% polymyositis usually w interstitial lung disease & arthralgia

-  Anti-Scl-70 (toposiomerase-1) Ab

-  20-40% progressive systemic sclerosis

-  20% pts w limited scleroderma

 

Rheumatoid Factor (RF) <Up>

-  High titre: RA

-  Low titre: connective tissue diseases (WG), AID, SLE, scleroderma, infection

 

Anti-Phospholipid Ab <Up>

-  Anti-Cardiolipin (Beta 2 glycoprotein 1)

-  Anti-Phospholipid Syndrome: 1o or SLE

 

 

 

Gastrointestinal

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Anti-Tissue Transglutaminase (TTG) Ab

-  Coeliac disease

 

Anti-Endomysial (EMA) Ab

-  90-100% specific in untreated Coeliac disease or dermatitis herpeteformis

 

Anti-Gliadin Ab

-  Coeliac disease (low specificity), dermatitis herpeteformis & gluten sensitivity

 

Anti-Gastric Parietal Cell (GPC) Ab

-  95% pts w pernicious anaemia or atrophic gastritis (Type A)

-  Associated w autoimmune thyroid disease

 

Anti-Intrinsic Factor (IF) Ab

-  75% pts w pernicious anaemia

 

Anti-Smooth Muscle Ab

-  High titre: autoimmune hepatitis

-  Low titre: after infection

 

Anti-Liver Kidney (LKM) Ab

-  LKM-1: Type 2a & 2b autoimmune hepatitis

-  LKM-2: Drug induced hepatitis

-  LKM-3: Hepatitis D

 

Anti-Mitochondrial Ab

-  M1: Positive syphilis serology

-  M2: 95% w 1o biliary cirrhosis, also chronic active hepatitis, autoimmune thyroiditis, Sjogrens syndrome

-  M5: SLE & antiphospholipid syndrome

 

 

 

Endocrine

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Anti-Adrenal Ab

-  60-70% pts w idiopathic Addisons disease

 

Anti-Islet Cell Ab

-  Early in course of DM Type 2, then dissappear

 

Anti-Steroid Cell Ab

-  React w CYP450 in adrenals, ovaries, testies & anterior pituitary

-  Also found in Type 1 autoimmune polyendocrinopathy syndrome & premature gonadal & ovarian failure

 

Thyroid

-  Anti-Thyroperoxidase (TPO) Ab

-  1o hypothyroidism, Hashimotos thyroidits, Graves disease

-  Also Pernicious anaemia, Addisons disease

-  Anti-TSH Receptor Ab

-  Graves disease

 

 

 

Neurological

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Anti-Acetyl Cholin Receptor (AChR) Ab

-  85-95% Myasthenia Gravis

 

Anti-Ganglioside (GM1, GQ1b) Ab

-  GM1: Guillain Barre syndrome, chronic demyelinating polyneuropathy, multifocal motor neuropathy

-  GQ1b: Miller Fischer variant of Guillain Barre

 

Anti-Glutamic Acid Decarboxylase (GAD) Ab

-  >60% stiff man syndrome, also DM Type 1

 

Anti-Muscle Specific Kinase (MuSK) Ab

-  40% Myasthenia Gravis who are –ve for Anti-AChR Ab

 

Anti-Paraneoplastic Ab

-  Paraneoplastic disease

-  Anti-Yo (PCCA)

-  Anti-Hu (ANNA-1)

-  Anti-Ri (ANNA-2)

 

Anti-Skeletal Muscle Ab

-  80-100% thymomatous Myasthenia Gravis

-  Also may occur hepatitis, acute viral infections or polymyositis

 

Anti-Voltage Gated Calcium Channel (VGCC) Ab

-  Lambert Eaton Myasthenic Syndrome (LEMS)

 

Anti-Voltage Gated Potassium Channel (anti VKCC) Ab

-  Acquired neuromyotonia

 

 

 

Renal

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Anti-C1q Ab

-  Renal disease in SLE

-  High levels in Hypocomplementaemic Urticarial Vasculitis Syndrome (HUVS)

 

C3 Nephritic Factor (C3 Nef)

-  Membrano-proliferative GN (Type II) & partial lipidodystrophy

 

Anti-Glomerular Basement Membrane (GBM) Ab

-  >90% sensitivity for Goodpastures syndrome

 

 

 

Vascular

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Anti-Neutrophil Cytoplasmic (ANCA) Ab

-  Primary systemic vasculitis

-  Cytoplasmic ANCA (c-ANCA) Ab

-  Proteinase-3 (PR-3)

-  97% specific for Wegeners Granulomatosis

-  Also found in microscopic polyangiitis (WG, PAN)

-  Perinuclear ANCA (p-ANCA) Ab

-  Myeloperoxidase (MPO-ANCA)

-  Microscopic polyangiitis: WG, PAN

-  Severe vasculitis

-   Wegeners Granulomatosis

-   Churg Strauss Syndrome

-  Other specifities

-  IBD, sclerosing cholangitis, RA, SLE, chronic active hepatitis

-  Atypical ANCA Ab

 

 

 

Other

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Anti-Cardiac Muscle Ab

-  Dresslers syndrome post AMI

 

Anti-IgA Ab

-  Selective IgA deficiency

 

Skin Reactive Ab

-  Anti-Intercellular Substance/Desmosome Ab

-  Pemphigus

-  Anti-Basement Membrane Zone Ab

-  Bullous pemphigoid

 

 

 

Autoimmune Tests by Disease

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Disease

Tests

Addisons Disease

AAD

Antiphoshpolipid Antibody Syndrome

ACL, B2-GPI

Churg-Strauss Syndrome

ANCA, MPO

Coeliac Disease

EMA, IgA, TTG

CREST Syndrome

ANA

DM Type 1

ICA, GAD

Goodpastures Syndrome

GBM

Microscopic Polyangitis

ANCA, MPO, PR3

Myasthenia Gravis

ASTR, ACHR

Pemphigoid

ASK

Pemphigus

ASK

Pernicous Anaemia

APC

Polymyositis

ANA, ENA

Primary Biliary Cirrhosis

AMC

RA

RF, Anti CCP

Scleroderma

ANA, ENA

Sjogrens Syndrome

ANA, ENA

SLE

ANA, DNA, ENA

Wegners Granulomatosis

ANCA, MPO, PR3

 

-  ENA = Ab to Extracable Nuclear Ag

-  ANCA = Anti Neutrophil Cytoplasmic Ab

-  MPO = Anti Myeloperoxidase Ab

-  PR3 = Anti Proteinase 3 Ab

-  APC = Anti Parietal Cell Ab

-  AMC = Anti Mitochondrial Ab

-  ASM = Anti Smooth Muscle Ab

-  GBM = Anti Glomerular Basement Mebrane Ab

-  ICA = Anti Islet Cell Ab

-  ACL = Anti Cardiolipin Ab

-  ASK = Anti Skin Ab

-  LKM = Anti Liver Kidney Microsomal Ab

-  ASTR = Anti Striated Muscle Ab

-  ACHR = Anti ACH Receptor Ab

-  AAD = Anti Adrenal Ab

-  GAD = Anti Glutamic Acid Decarboxylase Ab

-  EMA = Anti Endomysial Ab

-  B2-GPI = Beta-2 Glycoprotein I Ab

-  RF = Rheumatoid Factor

-  CCP = Anti Cyclic Citrulinninated Peptide Ab

 

 

 

References

 

Western Diagnostic Pathology: Guide to Autoimmune Testing