Page last updated: 2024-09-27

Cogan Syndrome

A condition consisting of inflammatory eye disease usually presenting as interstitial KERATITIS, vestibuloauditory dysfunction, and large- to medium-vessel vasculitis.

Synonyms(3)

Synonym
Cogans Syndrome
Cogan's Syndrome
Cogan Syndrome

Research Excerpts

Overview

ExcerptReference
"Cogan's syndrome is a rare autoimmune disorder characterized by systemic vasculitis as well as eye and ear inflammation."( Blackstone, J; Cartin, A; Currie, C; Pinette, MG; Wax, JR, 2009)
"Cogan's syndrome is a rare chronic inflammatory disorder which typically associates an ophthalmological and vestibulocochlear involvement, with a risk of systemic symptoms in 50-70% of cases."( Garabedian, EN; Hautefort, C; Loundon, N; Marlin, S; Montchilova, M; Ulinski, T, 2009)
"Cogan's syndrome is a rare idiopathic chronic inflammatory disease of the eye and the inner ear in young adults."( Conraads, V; Haine, S; Weyn, T, 2009)
"Cogan syndrome is a syndrome of non-syphilitic interstitial keratitis associated with vestibuloauditory deficits."( Chakarbarty, B; Jain, R; Juneja, M, 2011)
"Cogan's syndrome is characterized by interstitial keratitis, vestibular impairment and hearing loss, commonly bilateral."( Karakostas, E; Stratoudaki, R; Vasileiadis, I, 2012)
"Cogan's syndrome is a relatively rare inflammatory disorder presenting with a combination of ocular and vestibuloauditory symptoms."( Knapp, C; Mukherjee, R; Shahid, FL, 2013)
"Cogan syndrome is a systemic disease manifesting interstitial keratitis, sensorineural hearing loss, tinnitus, and rotatory vertigo."( Enya, T; Izu, A; Miyazawa, T; Nishi, H; Okada, M; Sugimoto, K; Takemura, T, 2014)
"Cogan's syndrome is a rare chronic vasculitis, characterized in its typical form by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis."( Jančatová, D; Komínek, P; Matoušek, P; Zeleník, K, 2015)
"Cogans syndrome is a rare systemic inflammatory disease characterized by a combination of audiovestibular and ocular symptoms."( Carlsson, PI; Hedin, E; Lyckberg, H, 2016)
"Cogan syndrome is a rare inflammatory condition that mainly affects adults and is characterised by inflammation of various ocular structures and by audiovestibular symptoms such as hearing loss and vertigo."( Rösken, GRHJ; Stroomer, JWG; van Oosterhout, WRPJ, 2022)
"Cogan syndrome is a rare inflammatory condition that warrants a multidisciplinary approach by an ophthalmologist, neurologist, ENT-physician, and rheumatologist / immunologist for swift diagnosis and treatment with immunosuppressive medication."( Rösken, GRHJ; Stroomer, JWG; van Oosterhout, WRPJ, 2022)

Treatment

ExcerptReference
"A 33-year-old G1 P0 with Cogan's syndrome experienced an uneventful pregnancy while being treated with hydroxychloroquine and prednisone."( Blackstone, J; Cartin, A; Currie, C; Pinette, MG; Wax, JR, 2009)

Research

Studies (33)

TimeframeStudies, This Condition (%)All Conditions %
pre-19901 (3.03)23.3326
1990's0 (0.00)12.5806
2000's6 (18.18)18.1394
2010's23 (69.70)28.8240
2020's3 (9.09)9.53
DrugIndicatedRelationship StrengthStudiesTrials
aspirin0low20
azathioprine0low40
hydroxychloroquine0low10
lamotrigine0low10
leflunomide0low10
prednisolone0low100
prednisone0low20
fluprednisolone0low10
methylprednisolone0low80
isoxazoles0low10
adenosine diphosphate ribose0low10
propiconazole0low10
dexamethasone sulfate ester0low10
fluorodeoxyglucose f180low10
methotrexate0low30
cortisone0low10
fibrin0low10
difluprednate0low10
tacrolimus0low10
mycophenolic acid0low10
cardiovascular agents0low20
cyclosporine0low20
clozapine0low10
eye0low10

Protein Targets (466)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
TDP1 protein130013
Transient receptor potential cation channel subfamily M member 20011
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE5005
Chain A, TYROSYL-DNA PHOSPHODIESTERASE5005
Chain A, Phospholipase A2 isoform 30011
Chain A, HADH2 protein6006
Chain B, HADH2 protein6006
Chain A, 2-oxoglutarate Oxygenase3003
GTP-binding protein (rab7)0011
ras protein, partial0011
interleukin 82002
thioredoxin reductase7007
GLS protein4004
AR protein140014
aldehyde dehydrogenase 1 family, member A15005
thyroid stimulating hormone receptor2002
hypothetical protein, conserved1001
cytochrome P450 family 3 subfamily A polypeptide 44004
EWS/FLI fusion protein7007
glucocorticoid receptor [Homo sapiens]130013
retinoic acid nuclear receptor alpha variant 19009
estrogen-related nuclear receptor alpha110011
estrogen nuclear receptor alpha140014
cytochrome P450 2D64004
glucocerebrosidase2002
peroxisome proliferator-activated receptor delta8008
Rac1 protein0011
IDH17007
cell division cycle 42 (GTP binding protein, 25kDa), partial0011
euchromatic histone-lysine N-methyltransferase 26006
Bloom syndrome protein isoform 16006
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 13003
DNA polymerase iota isoform a (long)6006
DNA polymerase kappa isoform 13003
muscarinic acetylcholine receptor M16006
lethal factor (plasmid)3003
Prostaglandin-H2 D-isomerase0101
ATP-binding cassette sub-family C member 30808
Multidrug resistance-associated protein 40809
Solute carrier family 22 member 60101
Prostaglandin G/H synthase 1 0101
Prostaglandin G/H synthase 2 0101
Bile salt export pump010010
Epidermal growth factor receptor0101
Carbonic anhydrase 10202
Carbonic anhydrase 20213
Fatty acid-binding protein, liver0202
Integrin beta-32103
Myeloperoxidase0101
Prostaglandin G/H synthase 10101
Seed linoleate 13S-lipoxygenase-10101
Integrin alpha-IIb2103
Glutathione hydrolase 1 proenzyme0101
Prostaglandin G/H synthase 10202
Adenosine receptor A10202
Adenosine receptor A2a0101
Substance-P receptor0101
Prostaglandin G/H synthase 20303
Urotensin-2 receptor0101
4-aminobutyrate aminotransferase, mitochondrial0101
Nuclear receptor ROR-gamma3003
Ras-related protein Rab-2A0011
Gamma-aminobutyric acid receptor subunit alpha-15207
Prostaglandin G/H synthase 20101
Rho-associated protein kinase 20101
Nicotinate phosphoribosyltransferase0101
Solute carrier family 22 member 200202
Solute carrier family 22 member 60202
Canalicular multispecific organic anion transporter 10808
Sigma non-opioid intracellular receptor 10213
Sigma non-opioid intracellular receptor 10213
Chain A, Ferritin light chain5005
acetylcholinesterase5005
phosphopantetheinyl transferase4004
RAR-related orphan receptor gamma8008
SMAD family member 23003
ATAD5 protein, partial4004
Fumarate hydratase3003
SMAD family member 33003
GLI family zinc finger 38008
Microtubule-associated protein tau3003
thioredoxin glutathione reductase2002
nuclear receptor subfamily 1, group I, member 37007
retinoid X nuclear receptor alpha7007
farnesoid X nuclear receptor5005
pregnane X nuclear receptor7007
G4004
polyprotein3003
P532002
peroxisome proliferator activated receptor gamma6006
arylsulfatase A2002
aryl hydrocarbon receptor6006
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a100010
thyroid stimulating hormone receptor1001
activating transcription factor 63003
v-jun sarcoma virus 17 oncogene homolog (avian)5005
Histone H2A.x4004
peripheral myelin protein 22 isoform 15005
hemoglobin subunit beta1001
cellular tumor antigen p53 isoform a3003
thyroid hormone receptor beta isoform 28008
heat shock protein beta-13003
nuclear factor erythroid 2-related factor 2 isoform 18008
geminin9009
peripheral myelin protein 226006
survival motor neuron protein isoform d6006
cytochrome P450 3A4 isoform 15005
M-phase phosphoprotein 84004
muscleblind-like protein 1 isoform 13003
lamin isoform A-delta107007
Gamma-aminobutyric acid receptor subunit pi5106
Bile salt export pump0404
Voltage-dependent calcium channel gamma-2 subunit8008
Interferon beta6006
HLA class I histocompatibility antigen, B alpha chain 4004
Cellular tumor antigen p538008
Gamma-aminobutyric acid receptor subunit beta-15106
Gamma-aminobutyric acid receptor subunit delta5106
Gamma-aminobutyric acid receptor subunit gamma-25106
Glutamate receptor 28109
Gamma-aminobutyric acid receptor subunit alpha-55106
Gamma-aminobutyric acid receptor subunit alpha-35106
Gamma-aminobutyric acid receptor subunit gamma-15106
Gamma-aminobutyric acid receptor subunit alpha-25106
Gamma-aminobutyric acid receptor subunit alpha-45106
Gamma-aminobutyric acid receptor subunit gamma-35106
Gamma-aminobutyric acid receptor subunit alpha-65106
Gamma-aminobutyric acid receptor subunit beta-35106
Gamma-aminobutyric acid receptor subunit beta-25106
GABA theta subunit5106
Inositol hexakisphosphate kinase 14004
ATPase family AAA domain-containing protein 59009
Ataxin-29009
Gamma-aminobutyric acid receptor subunit epsilon5106
Protein-arginine deiminase type-40303
cytochrome P450 2C9, partial4004
Chain A, JmjC domain-containing histone demethylation protein 3A1001
5-hydroxytryptamine receptor 3E0101
Luciferase2002
hypoxia-inducible factor 1 alpha subunit4004
NFKB1 protein, partial1001
caspase 7, apoptosis-related cysteine protease2002
estrogen receptor 2 (ER beta)5005
progesterone receptor100010
Beta-1 adrenergic receptor 0101
bromodomain adjacent to zinc finger domain 2B2002
vitamin D (1,25- dihydroxyvitamin D3) receptor6006
caspase-32002
Adenylate cyclase type 1 0101
cytochrome P450 2D6 isoform 12002
cytochrome P450 2C19 precursor2002
cytochrome P450 2C9 precursor2002
D(1A) dopamine receptor2002
chromobox protein homolog 14004
potassium voltage-gated channel subfamily H member 2 isoform d2002
DNA polymerase beta2002
mitogen-activated protein kinase 12002
atrial natriuretic peptide receptor 2 precursor1001
nuclear receptor ROR-gamma isoform 15005
short transient receptor potential channel 6 isoform 10011
transient receptor potential cation channel subfamily V member 11001
Polyunsaturated fatty acid lipoxygenase ALOX15B2002
Voltage-dependent L-type calcium channel subunit alpha-1F0101
5-hydroxytryptamine receptor 3A0101
5-hydroxytryptamine receptor 40202
5-hydroxytryptamine receptor 3B0101
Albumin0011
Aldo-keto reductase family 1 member B10404
Muscarinic acetylcholine receptor M20415
Muscarinic acetylcholine receptor M40314
ATP-dependent translocase ABCB10001
Muscarinic acetylcholine receptor M10202
Muscarinic acetylcholine receptor M30202
Muscarinic acetylcholine receptor M40202
Cytochrome P450 3A40304
5-hydroxytryptamine receptor 1A0202
5-hydroxytryptamine receptor 2C0304
Muscarinic acetylcholine receptor M50202
Muscarinic acetylcholine receptor M50303
Alpha-2A adrenergic receptor0303
Calmodulin-10101
Beta-2 adrenergic receptor0202
Cytochrome P450 2D60001
Muscarinic acetylcholine receptor M20202
Muscarinic acetylcholine receptor M10314
Cytochrome P450 2C9 0203
Angiotensin-converting enzyme0202
D(2) dopamine receptor0205
5-hydroxytryptamine receptor 2A0303
Alpha-1B adrenergic receptor0213
Alpha-2B adrenergic receptor0303
Beta-1 adrenergic receptor0101
Alpha-2C adrenergic receptor0303
D0202
D(3) dopamine receptor0202
5-hydroxytryptamine receptor 1A0202
Alpha-2B adrenergic receptor0213
D(2) dopamine receptor0202
Muscarinic acetylcholine receptor M30314
Endothelin receptor type B1102
D(1A) dopamine receptor0203
D(4) dopamine receptor0204
D(1B) dopamine receptor0102
Adenylate cyclase type 30101
Alpha-2C adrenergic receptor0213
UDP-glucuronosyltransferase 1A40002
Alpha-2A adrenergic receptor0213
Alpha-1D adrenergic receptor0213
Sodium-dependent noradrenaline transporter 0404
Sodium-dependent dopamine transporter0202
5-hydroxytryptamine receptor 3A0101
Endothelin receptor type B0101
Histamine H2 receptor0202
Alpha-1D adrenergic receptor0404
D(1B) dopamine receptor0202
Beta-3 adrenergic receptor0101
Adenylate cyclase type 20101
Adenylate cyclase type 40101
5-hydroxytryptamine receptor 2A0204
5-hydroxytryptamine receptor 2C0213
5-hydroxytryptamine receptor 1B0202
5-hydroxytryptamine receptor 1D0202
D(4) dopamine receptor0202
5-hydroxytryptamine receptor 1F0202
5-hydroxytryptamine receptor 2B0213
Histamine H1 receptor0112
Histamine H1 receptor0202
Sodium-dependent serotonin transporter0202
Sodium-dependent serotonin transporter0202
5-hydroxytryptamine receptor 7 0101
Cytochrome P450 2C190001
5-hydroxytryptamine receptor 2C 0101
5-hydroxytryptamine receptor 70101
Alpha-1A adrenergic receptor0101
5-hydroxytryptamine receptor 2A0101
Histamine H1 receptor0226
Alpha-1B adrenergic receptor0101
D(3) dopamine receptor0204
5-hydroxytryptamine receptor 3A0101
Adenylate cyclase type 80101
5-hydroxytryptamine receptor 2B0213
Alpha-1A adrenergic receptor0213
5-hydroxytryptamine receptor 3A0101
Histamine H2 receptor3003
5-hydroxytryptamine receptor 5A0101
5-hydroxytryptamine receptor 60203
Cytochrome P450 2J20203
3-hydroxy-3-methylglutaryl-coenzyme A reductase 0101
D(2) dopamine receptor0101
D(3) dopamine receptor0101
Gastrin/cholecystokinin type B receptor0101
Spike glycoprotein3014
D(2) dopamine receptor0101
D(2) dopamine receptor0202
Calmodulin 0011
Inositol monophosphatase 11001
Voltage-dependent L-type calcium channel subunit alpha-1D 0101
Sodium-dependent dopamine transporter 0303
5-hydroxytryptamine receptor 2B0101
Adenylate cyclase type 60101
Adenylate cyclase type 50101
Potassium voltage-gated channel subfamily H member 20404
5-hydroxytryptamine receptor 40101
Voltage-dependent L-type calcium channel subunit alpha-1S0101
Voltage-dependent L-type calcium channel subunit alpha-1C0202
Transporter0101
Cholecystokinin receptor type A0101
5-hydroxytryptamine receptor 1A0101
Nuclear receptor subfamily 3 group C member 3 0202
5-hydroxytryptamine receptor 3D0101
Adenylyl cyclase 7 0101
5-hydroxytryptamine receptor 3C0101
D0202
Histamine H4 receptor0112
5-hydroxytryptamine receptor 3B0101
Transporter0101
Histamine H3 receptor0101
endonuclease IV1001
dopamine D1 receptor2002
pregnane X receptor3003
regulator of G-protein signaling 41001
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a3003
atrial natriuretic peptide receptor 1 precursor1001
flap endonuclease 12002
Glucocorticoid receptor08215
Sex hormone-binding globulin0011
Glycine receptor subunit alpha-10808
Corticosteroid-binding globulin0202
Glycine receptor subunit beta0808
Glycine receptor subunit alpha-20808
Glycine receptor subunit alpha-30808
Potassium voltage-gated channel subfamily A member 10011
TAR DNA-binding protein 434004
Multidrug and toxin extrusion protein 10101
Chain A, Beta-lactamase2002
Chain A, Cruzipain1001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)3003
nuclear receptor subfamily 1, group I, member 25005
importin subunit beta-1 isoform 12002
snurportin-12002
GTP-binding nuclear protein Ran isoform 11001
neuropeptide S receptor isoform A2002
Genome polyprotein 0101
Spike glycoprotein0011
Replicase polyprotein 1ab0011
Transmembrane protease serine 20011
Procathepsin L0011
Replicase polyprotein 1a0011
Replicase polyprotein 1ab0011
Replicase polyprotein 1ab0112
Replicase polyprotein 1ab0112
Sigma intracellular receptor 20101
Cyclic GMP-AMP synthase0101
Angiotensin-converting enzyme 2 0011
Toll-like receptor 90101
Toll-like receptor 70101
GALC protein1001
nuclear factor erythroid 2-related factor 2 isoform 27007
Sodium channel protein type 1 subunit alpha0101
Sodium channel protein type 2 subunit alpha0101
Sodium channel protein type 3 subunit alpha0101
Dihydrofolate reductase0426
Potassium voltage-gated channel subfamily E member 10101
Sodium channel protein type 4 subunit alpha0102
Potassium voltage-gated channel subfamily KQT member 10101
Sodium channel protein type 5 subunit alpha0101
Sodium channel protein type 9 subunit alpha0101
Sodium channel protein type 2 subunit alpha0101
Sodium channel protein type 3 subunit alpha0101
Sodium channel protein type 10 subunit alpha0101
glp-1 receptor, partial3003
matrix metalloproteinase 1, partial0011
USP1 protein, partial3003
PPM1D protein2002
Thrombopoietin1001
nonstructural protein 13003
Parkin1001
pyruvate kinase1001
NPC intracellular cholesterol transporter 1 precursor1001
thyroid hormone receptor beta isoform a2002
histone deacetylase 9 isoform 31001
huntingtin isoform 21001
ras-related protein Rab-9A1001
urokinase-type plasminogen activator precursor2002
plasminogen precursor2002
urokinase plasminogen activator surface receptor precursor2002
histone acetyltransferase KAT2A isoform 12002
Dihydroorotate dehydrogenase (quinone), mitochondrial0101
Cytochrome P450 1A20101
Amine oxidase [flavin-containing] A0101
Endothelin-1 receptor1001
Dihydroorotate dehydrogenase (quinone), mitochondrial0101
Dihydroorotate dehydrogenase (quinone), mitochondrial0101
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain B, DIHYDROFOLATE REDUCTASE0011
acid sphingomyelinase1001
ClpP1001
15-lipoxygenase, partial1001
67.9K protein2002
Thymidylate synthase 0101
alpha-galactosidase1001
Thymidylate synthase 0101
polyunsaturated fatty acid lipoxygenase ALOX121001
vitamin D3 receptor isoform VDRA3003
transcriptional regulator ERG isoform 31001
nuclear receptor coactivator 1 isoform 1 [Homo sapiens]0101
peptidyl-prolyl cis-trans isomerase NIMA-interacting 11001
DNA polymerase eta isoform 11001
transactivating tegument protein VP16 [Human herpesvirus 1]0101
caspase-1 isoform alpha precursor1001
nuclear receptor coactivator 3 isoform a0101
Toll-like receptor 40101
Fatty-acid amide hydrolase 10101
ATP-binding cassette sub-family C member 30102
Dihydrofolate reductase0203
Dihydrofolate reductase0203
Dihydrofolate reductase 0101
Dihydrofolate reductase0101
Dihydrofolate reductase0101
Dihydrofolate reductase0203
Thymidylate synthase0101
Tyrosine-protein kinase ABL10011
Thymidylate synthase0202
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Thymidylate synthase0202
High mobility group protein B10011
Dihydrofolate reductase0101
Thymidylate synthase0101
Glucose-6-phosphate 1-dehydrogenase0101
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Folate receptor beta0101
Cytochrome P450 11B1, mitochondrial 0101
Folate receptor alpha0101
Dihydrofolate reductase0202
Histidine decarboxylase0101
Trifunctional purine biosynthetic protein adenosine-30101
Cyclin-dependent kinase 20011
Bifunctional purine biosynthesis protein ATIC0202
Multidrug resistance-associated protein 1 0001
Delta-type opioid receptor0101
Mu-type opioid receptor0101
Kappa-type opioid receptor0101
Reduced folate transporter0202
Folylpolyglutamate synthase, mitochondrial0103
6-phosphogluconate dehydrogenase, decarboxylating0101
Caspase-71001
Solute carrier organic anion transporter family member 1A30001
Dihydrofolate reductase0101
Pteridine reductase 10101
Folylpolyglutamate synthase, mitochondrial0103
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Solute carrier family 22 member 60001
Canalicular multispecific organic anion transporter 10102
Trifunctional purine biosynthetic protein adenosine-30101
Dihydrofolate reductase 0101
Solute carrier family 22 member 80001
Dihydrofolate reductase0101
ATP-binding cassette sub-family C member 110001
Proton-coupled folate transporter0101
Multidrug resistance associated protein0102
Solute carrier organic anion transporter family member 1B30001
Solute carrier family 22 member 110001
Broad substrate specificity ATP-binding cassette transporter ABCG20102
ATP-dependent phosphofructokinase2002
Nuclear receptor subfamily 1 group I member 20022
Androgen receptor0303
Nuclear receptor subfamily 1 group I member 20011
Chain A, Inosine-5'-Monophosphate Dehydrogenase 20101
UDP-glucuronosyltransferase 1A90001
Inosine-5'-monophosphate dehydrogenase 20202
Glutamate receptor 10101
Glutamate receptor 30101
Glutamate receptor 40101
Inosine-5'-monophosphate dehydrogenase 1 0202
UDP-glucuronosyltransferase 1A1 0001
Peroxisome proliferator-activated receptor gamma0011
Alpha-synuclein1001
Inosine-5'-monophosphate dehydrogenase0102
UDP-glucuronosyltransferase 1A70001
UDP-glucuronosyltransferase 1A100001
UDP-glucuronosyltransferase 1A80001
luciferase0001
Estrogen receptor0202
Progesterone receptor0203
Glucocorticoid receptor0112
Glucocorticoid receptor0011
Mineralocorticoid receptor 0213
Androgen receptor0202
Glutamine synthetase0011
Nociceptin receptor0011
Estrogen receptor beta0202
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)1001
heat shock protein HSP 90-alpha isoform 20001
Guanine nucleotide-binding protein G1001
heat shock protein 90, putative0001
UDP-glucuronosyltransferase 2B70001
Cytochrome P450 3A50101
Serine/threonine-protein kinase mTOR0101
Peptidyl-prolyl cis-trans isomerase FKBP1A0213
Peptidyl-prolyl cis-trans isomerase FKBP1B0011
Serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform0101
Peptidyl-prolyl cis-trans isomerase FKBP50101
Splicing factor 3B subunit 30101
Solute carrier organic anion transporter family member 1B10101
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]