Clinical Immunological Laboratory Am Sonnenberg 9 Prof. Dr. Winfried Stöcker D-23627 Groß Grönau (Germany) Clinical Pathologist Telephone +49 451 58 55 100 Fax 5855 101

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Systemic Autoantibodies against P A G M BASIC SPECTRUM P A G M SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) P A G M SHARP SYNDROME MCTD ANA (cell nuclei) IF global testing ANA (cell nuclei) IF global testing U1-nRNP (70K, A, C) ANA profile for differentiation Nucleosomes ANA (cell nuclei) IF global testing dsDNA NcX ELISA dsDNA NcX ELISA ssDNA dsDNA IFT SLE-specific dsDNA IFT (C. luciliae) ENA-ProfilePlus 1 ELISA dsDNA RIA P A G M POLYMYOSITIS, nRNP/Sm, Sm, SS-A, SS-B, Scl-70, Jo-1 ENA PoolPlus ELISA DERMATOMYOSITIS SLE Profile ELISA (dsDNA, histones, U1-nRNP (70K, A, C) ANA (cell nuclei) IF global testing nucleosomes, nRNP/Sm, Sm, SS-A, SS-B, Sm Myopathy Profile EUROLINE Scl-70) SS-A (Ro) 60 kDa: native (Mi-2 alpha, Mi-2 beta, TIF1 gamma, AMA (mitochondria) Ro-52: recombinant MDA5, NXP2, SAE1, Ku, PM100, PM75, ASMA (smooth muscle) SS-B (La) Jo-1, SRP, PL-7, PL-12, EJ, OJ, Ro-52, cN-1A) cANCA 1 (granulocytes) Ribosomal P-protein Jo-1 pANCA 1 (granulocytes) Ku PL-7 Autoantibody profile 30 IF substrates Cyclin I (PCNA) PL-12 Histones (global) OJ P A G M ANA DIAGNOSTICS ssDNA (single-stranded DNA) EJ EUROLINE (nucleosomes, dsDNA, histones, pANCA 1 (granulocytes) PM-Scl75, PM-Scl100 SS-A, Ro-52, SS-B, nRNP/Sm, Sm, Mi-2 SRP (signal recognition particle) alpha, Mi-2 beta, Ku, CENP A, CENP B, P A G M SJÖGREN’S SYNDROME Ro-52: recombinant Sp100, PML, Scl-70, PM-Scl100, PM-Scl75, ANA (cell nuclei) IF global testing Ku RP11, RP155, gp210, PCNA, DFS70) SS-A (Ro) 60 kDa: native Mi-2 EUROLINE Cytoplasm Profile (AMA M2, Ro-52: recombinant cN-1A (Mup44, NT5C1A) M2-3E, rib. P-prot., Jo-1, SRP, PL-7, PL-12, EJ, SS-B (La) OJ, Ro-52) ssDNA P A G M (RHEUMATOID) ARTHRITIS CCP (cyclic citrullinated peptide) P A G M VARIOUS AUTOANTIBODIES P A G M ANTI-PHOSPHOLIPID Sa DFS70 SYNDROME (APS) CEP-1 Centrioles RF (classic rheumatoid factor) MSA-1 (NuMa, spindle fibres) ß-2-glycoprotein 1 (RA keratin) MSA-2 (HsEg5, spindle fibres) Phosphatidylserine GS-ANA (granulocyte-specific ANA) MSA-3 (chromosome-associated antigen) ANA (cell nuclei) IF global testing F protein (CENP-F, mitosin) P A G M SYSTEMIC SCLEROSIS Histones Midbody (DIFFUSE + LIMITED FORM) RANA (rheumatoid arthritis nuclear antigen) Ribosomes ANA (cell nuclei) IF global testing pANCA 1 (granulocytes) Golgi apparatus Systemic Sclerosis Profile EUROLINE Cartilage Lysosomes (Scl-70, CENP A, CENP B, RP11, RP155, Cytoskeleton fibrillarin, NOR90, Th/To, PM-Scl100, Therapy monitoring PM-Scl75, Ku, PDGFR, Ro-52) MabTrack Level Adalimumab Vimentin Scl-70 (DNA I) MabTrack Anti-Drug Antibody Adalimumab Cytokeratin PM-Scl75 (75 kDa) MabTrack Level Infliximab Tropomyosin PM-Scl100 (100 kDa) MabTrack Anti-Drug Antibody Infliximab Vinculin Desmin Centromere A protein (recombinant) Laminin (basement membrane) Centromere B protein (recombinant) P A G M IMMUNE HAEMATOLOGY Elastin U3-nRNP (fibrillarin) Lymphocytes Vessel endothelium RNA polymerase III Thrombocytes, indirect (free antibodies) 7-2-RNP (Th/To) Thrombocytes, direct (bound antibodies) 3 CIRCULATING IMMUNE COMPLEXES NOR (nucleolus organisator region) PCA (parietal cells) CIC-C1q ELISA (antigen detection) Ku H+/K+-ATPase ELISA (PCA) C1q ELISA

Grey boxes: standard analysis. Immunoglobulin classes: P = IgAGM, A = IgA, G = IgG, M = IgM 1) ANCA diagnostics in acute cases within one hour 2) Send frozen sample(s) 3) Use special sampling procedure

SA_0000_I_UK_A14, 03/2020 Page 1/2 PLEASE ENTER AGAIN! Clinical Immunological Laboratory Sample ID of sender/report recipient: Am Sonnenberg 9 Prof. Dr. Winfried Stöcker D-23627 Groß Grönau (Germany) Clinical Pathologist Telephone +49 451 58 55 100 Fax 5855 101

Organ /Tissue-Specific Autoimmunity: Autoantibodies against P A G M THYROID GLAND P A G M KIDNEY, LUNG P A G M ANCA-ASSOCIATED VASCULITIDES TRAb (TSH receptors) cANCA IFT 1 granulocytes (WEGENER’S DIS., MICROSC. ARTERIITIS, TPO (thyroidea peroxidase) pANCA IFT 1 granulocytes CHURG-STRAUSS SYNDROME) TAb (thyroglobulin) Kidney IF global testing cANCA IFT 1 granulocytes MAb (microsomes) GBM ELISA glomerular basement membrane PR3 (proteinase 3) PLA2R BPI (CAP 57) P A G M DIABETES MELLITUS THSD7A pANCA IFT 1 granulocytes ICA (islet cells) ANA (cell nuclei) IF global testing MPO (myeloperoxidase) GAD (glutamic acid decarboxylase) dsDNA IFT Elastase IA2 (tyrosin phosphatase) TBM (tubular basement membrane) Cathepsin G Insulin, human Lung alveolar basement membrane Lactoferrin Glucagon-producing cells ANCA Profile ELISA Zinc transporter 8 PR3, MPO, elastase, cathepsin G, BPI, lactoferrin Lipocytes P A G M NERVOUS SYSTEM ANA (cell nuclei) IF global testing Neural Antigens IFT Elastin P A G M (POLY-)ENDOCRINOPATHY Hu, Ri, ANNA-3, Yo, Tr/DNER, myelin, Vessel endothelium Adrenal cortex Ma/Ta, GAD65, amphiphysin, aquaporin-4, 21-Hydroxylase glutamate receptors (type NMDA, AMPA), Ovary: theka cells GABAA/B receptors, LGI1, CASPR2, ZIC4, P A G M LIVER, BILE DUCTS Ovary: corpus luteum DPPX, glycin receptors, mGluR1, mGluR5, Liver IFT global testing Testis: Leydig cells Rho-GTPase activating protein 26, ITPR1, Autoimmune Liver Diseases Profile Steroid hormone-producing cells Homer 3, MOG, recoverin, neurochondrin, EUROLINE AMA M2, M2-3E, Sp100, Paratyhroid gland GluRD2, flotillin 1/2, IgLON5 PML, gp210, LKM-1, LC-1, SLA/LP, SS-A, ICA (islet cells) Ro-52, Scl-70, CENP A, CENP B, PGDH TPO (thyroidea peroxidase) Paraneoplastic neurological syndromes PCA (parietal cells) Neuronal Antigens Profile Autoimmune hepatitis (AIH) H+/K+-ATPase ELISA (PCA) EUROLINE amphiphysin, CV2, PNMA2 SLA/LP (soluble liver antigen) Pituitary gland: anterior lobe (Ma-2), Ri, Yo, Hu, recoverin, SOX1, F-actin Pituitary gland: posterior lobe titin, Zic4, GAD65, Tr (DNER) ANA (cell nuclei) IF global testing MAb (thyroid microsomes) LGI1 LC-1 (liver cytosol) Adrenal medulla CASPR2 LKM (liver kidney microsomes) Placenta Potassium channel (VGKC) RIA LKM-1 ELISA Vasopressin-producing cells NMDA receptors ASGPR (asialoglycoprotein receptors) AMPA receptors (GluR1, GluR2) ASMA (smooth muscle) P A G M INFERTILITY GABAB receptors LSP (liver-specific protein) Cardiolipin GAD65 LMA (liver cell membrane) Ovary: theka cells, c. luteum, zona pellucida Hu (neurone nuclei; ANNA-1) PGDH Testis: Leydig cells Ri (neurone nuclei; ANNA-2) Spermatozoa Yo (Purkinje cell cytoplasm; PCA-1) Primary biliary cholangitis (PBC) Pituitary gland: anterior lobe Amphiphysin AMA (mitochondria) Placenta CV2 (CRMP-5) AMA M2-3E (PDH + BPO) Prostate Ma1/Ma2 (neurone nucleoli; Ta) AMA M4 (sulfitoxidase) ANNA-3 AMA M9 (glycogen phosphorylase) P A G M EPIDERMIS PCA-2 (Purkinje cell cytoplasm) Sp100, PML (nuclear dots) Desmosomes Tr (DNER) gp210 (nuclear membrane, lamin) Desmoglein 1 ZIC4 SS-A / SS-B Desmoglein 3 IgLON5 Centromeres Envoplakin AGNA (anti-glia nuclear antigen) Epidermal basement membrane SOX-1 Primary sclerosing cholangitis (PSC) BP180 (NC16A-4X) Recoverin pANCA (granulocytes) BP230 Oral mucosa Further parameters Further antibodies Transitional epithelium (urinary bladder) Aquaporin-4 Bile ducts Epidermal keratin MOG (myelin-oligodendrocyte glycoprotein) Bile canaliculi Endomysium Glycin receptors Coilin; P80 (few nuclear dots) Deamidated gliadin (CD-AGFA, GAF-3X) DPPX Melanocytes ITPR1 Hair follicle CARPVIII P A G M STOMACH, INTESTINE Collagen type VII NC1 Flotillin 1/2 PCA (parietal cells) p200 mGluR1 H+/K+-ATPase (PCA) ELISA mGluR5 Intrinsic Factor ELISA Myelin Gastrin (G) cells P A G M EYE MBP (myelin-basic protein) Pancreas acini Recoverin MAG (myelin-associated glycoprotein) CUZD1 Tunica choroidea GFAP (glial fibrillary acidic protein) GP2 Cornea Astrocytes Pankreas secretion Retina Basal ganglia Saccharomyces cerevisiae Lens oculi Plexus myentericus (non-medullated nerves) Oral mucosa Corpus ciliare Profile Goblet cells, intestinal Eye muscle GM1, GM2, GM3, GD1a, GD1b, GT1b, GQ1b pANCA (granulocytes) cANCA 1 (granulocytes) Individual gangliosides: DNA-bound lactoferrin ANA (cell nuclei) IF global testing GM1 GM2 GM3 GD1a Enterocytes GD1b GT1b GQ1b Endomysium Borrelia burgdorferi (EMA) ELISA P A G M SKELETAL MUSCLE, THYMUS CXCL13 (CSF; antigen detection) Deamidated gliadin (CD-AGFA; GAF-3X) Acetylcholine receptors MuSK Research parameters Calcium channel (VGCC), PQ type AT1A3 Contactin1 Dop. rec. 2 (DRD2) EXOCRINE GLANDS, PANCREATITIS, Potassium channel (VGKC) ERC1 GABA-a receptors P A G M SJÖGREN’S SYNDROME CASPR2 GluRD2 Homer3 KCNA2 Pancreas, exocrine Thymus Neurexin Neurochondrin Pancreas acini Titin Neurofascin (NF155/NF186) Pancreas excretory ducts Skeletal muscle RhoGTpase-activating protein 26 Salivary glands (parotid gland) Sarcolemma Parotid gland acini Myosin CSF Serum Neurodegenerative diseases Parotid gland excretory ducts Beta-amyloid (1-40) (antigen detection) 2 Lacrimal gland Beta-amyloid (1-42) (antigen detection) 2 ANA (cell nuclei) IF global testing P A G M HEART Total tau (antigen detection) 2 SS-A (Ro) AMA M7 (myocard-specific) pTau (antigen detection) 2 SS-B (La) Heart muscle pNf-H neurofilament (ALS; antigen detection) 2 ssDNA (single-stranded DNA) Heart: intercalated disc pNf-L neurofilament (ALS; antigen detection) 2 Prostate Heart: myolemma Mamma

Grey boxes: standard analysis. Immunoglobulin classes: P = IgAGM, A = IgA, G = IgG, M = IgM 1) ANCA diagnostics in acute cases within one hour 2) Send frozen sample(s)

Last updated: 06/2020 Page 2/2