Cogan Syndrome
Cogan's syndrome with left main coronary artery occlusion. (1/12)
Cogan's syndrome is a rare idiopathic chronic inflammatory disease of the eye and the inner ear in young adults. Cogan's syndrome can be associated with large vessel vasculitis. We report a young female, with a history of interstitial keratitis and audiovestibular disease, who presents with large vessel vasculitis with left main coronary artery occlusion and develops heart failure. Cogan's syndrome was diagnosed on the basis of the presence of large vessel vasculitis with the typical inner ear and ocular involvement. (+info)A shifty diagnosis: Cogan's syndrome. A case report and review of the literature. (2/12)
Cogan's syndrome is defined as a chronic inflammatory disease of unknown origin, an autoimmune disease, characterized by bilateral sensorineural hearing loss, vestibular symptoms, inflammatory ocular manifestations with variable risk of developing into a systemic disease. The onset of disease is variable but is often characterized by isolated ocular symptoms or acute ear and/or vestibular manifestations, variably associated. The diagnosis of Cogan's syndrome can be a challenge as is evident in the case described here since it is based only on the association between bilateral ocular and vestibuloauditory symptoms with no specific diagnostic tests available. (+info)Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report. (3/12)
(+info)Aortic dissection associated with Cogans's syndrome: deleterious loss of vascular structural integrity is associated with GM-CSF overstimulation in macrophages and smooth muscle cells. (4/12)
(+info)The ciliopathies in neuronal development: a clinical approach to investigation of Joubert syndrome and Joubert syndrome-related disorders. (5/12)
(+info)Atypical Cogan's syndrome associated with coronary disease. (6/12)
Cogan's syndrome (CS) is a rare inflammatory disorder characterized by interstitial keratitis and vestibuloauditory abnormalities often associated with various systemic manifestations. Involvement of cardiovascular system resembling systemic vasculitis may lead to severe complications and death. The present report describes a case of a female patient with atypical Cogan's syndrome presented with systemic manifestations and severe coronary and femoral artery stenosis. Despite the clinical improvement after glucocorticoids and cyclophosphamide, the patient required double aortocoronal bypass grafting one year letter. During three years follow-up, she was in stable condition, without stenocardial symptoms and claudication and her inflammatory parameters remain normal. This case highlights the rare involvement of coronary arteries without associated large-vessel vasculitis of the aortic arch in CS. (+info)Otolaryngologic manifestations of systemic vasculitis. (7/12)
Systemic vasculitis is a heterogeneous group of diseases of various aetiologies and manifestations. In general, the clinical results derive from ischemia caused by vascular inflammation, which depends on the organ affected. Such vasculitis cases are classified according to the classification of the Chapel Hill conference. They can present with relative frequency as ENT manifestations in both their debut and throughout their evolution. Consequently, the ENT specialist should include them in the differential diagnosis in patients with ENT manifestations that are difficult to control or of atypical presentation. Our objective was to review the most common ENT clinical signs and symptoms in each of these diseases. (+info)Cogan's syndrome: an autoimmune inner ear disease. (8/12)
(+info)The exact cause of Cogan syndrome is not known, but it is believed to be an autoimmune disorder, where the immune system mistakenly attacks the healthy cells in the inner ear. It can also be associated with other autoimmune disorders such as rheumatoid arthritis, thyroiditis, and type 1 diabetes.
Symptoms of Cogan syndrome include:
* Progressive hearing loss, usually starting in one ear and gradually affecting the other ear
* Tinnitus (ringing in the ears)
* Unsteadiness or vertigo (dizziness)
* Nausea and vomiting
* Loss of balance and coordination
* Fatigue and fever
Diagnosis of Cogan syndrome is based on a combination of clinical examination, hearing tests, and imaging studies such as CT or MRI scans. There is no cure for Cogan syndrome, but treatment options include:
* Hearing aids or cochlear implants to improve hearing
* Medications to control tinnitus and vertigo
* Physical therapy to improve balance and coordination
* Corticosteroids to reduce inflammation in the inner ear
Prognosis for Cogan syndrome varies, but early diagnosis and treatment can help slow down the progression of the disease. In some cases, the hearing loss may be reversible, while in others it may be permanent. It is important to seek medical attention if you experience any symptoms of Cogan syndrome to receive an accurate diagnosis and appropriate treatment.
Examples of syndromes include:
1. Down syndrome: A genetic disorder caused by an extra copy of chromosome 21 that affects intellectual and physical development.
2. Turner syndrome: A genetic disorder caused by a missing or partially deleted X chromosome that affects physical growth and development in females.
3. Marfan syndrome: A genetic disorder affecting the body's connective tissue, causing tall stature, long limbs, and cardiovascular problems.
4. Alzheimer's disease: A neurodegenerative disorder characterized by memory loss, confusion, and changes in personality and behavior.
5. Parkinson's disease: A neurological disorder characterized by tremors, rigidity, and difficulty with movement.
6. Klinefelter syndrome: A genetic disorder caused by an extra X chromosome in males, leading to infertility and other physical characteristics.
7. Williams syndrome: A rare genetic disorder caused by a deletion of genetic material on chromosome 7, characterized by cardiovascular problems, developmental delays, and a distinctive facial appearance.
8. Fragile X syndrome: The most common form of inherited intellectual disability, caused by an expansion of a specific gene on the X chromosome.
9. Prader-Willi syndrome: A genetic disorder caused by a defect in the hypothalamus, leading to problems with appetite regulation and obesity.
10. Sjogren's syndrome: An autoimmune disorder that affects the glands that produce tears and saliva, causing dry eyes and mouth.
Syndromes can be diagnosed through a combination of physical examination, medical history, laboratory tests, and imaging studies. Treatment for a syndrome depends on the underlying cause and the specific symptoms and signs presented by the patient.
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Cogan's3
- Cogan's syndrome]. (nih.gov)
- Cogan's syndrome is an autoimmune disorder that mainly affects the eyes and inner ears along with large blood vessels. (nih.gov)
- Cogan's syndrome is a rare disorder characterized by ocular and audiovestibular manifestations in its typical form and caries a wide variety of atypical manifestations. (ucy.ac.cy)
Keratitis2
- Another important association to keep in mind is that patients with interstitial keratitis due to Cogan syndrome are more likely to have a history of systemic vasculitides, and prompt referral to a rheumatologist may be critical. (medscape.com)
- Interstitial keratitis is generally seen in the context of syphilis and, less commonly, in the context of herpes simplex keratitis and Cogan syndrome. (medscape.com)
Herpes1
- Chandler's syndrome (CS) is caused herpes. (articlealley.com)
Salivary glands1
- Abnormal distribution of AQP5 water channel protein in salivary glands from Sjgrens syndrome patients. (hopkinsmedicine.org)
Ocular2
- Joubert syndrome is a multisystem disorder that is associated with a constellation of cyclic ocular motor disturbances. (elsevier.com)
- We describe 2 children with congenital ocular motor apraxia who displayed wheel-rolling torsional eye movements and tonic alternating cyclodeviations of the eyes on retinal examination as a neurodiagnostic phenotype of Joubert syndrome. (elsevier.com)
Iris4
- 15. Optical coherence tomography characteristics of the iris in Cogan-Reese syndrome. (nih.gov)
- Chandler syndrome, the more common of the three, manifests greater corneal changes and edema but fewer iris abnormalities. (articlealley.com)
- Cogan-Reese syndrome presents with iris atrophy, corneal endotheliopathy, corneal edema, and prominent iris nevi. (articlealley.com)
- Irido-corneal endothelial (ICE) syndrome is a rare group of eye related disorders that constitute three different clinical entities: Chandler syndrome (CS), essential / progressive iris atrophy and iris naevus / Cogan-Reese syndrome. (uk.com)
Vasculitis3
- Traditionally, vasculitis syndromes have been categorized according to features that include clinical phenotype, the predominant size of the involved vessels, or the histopathology of the involved vessel. (musculoskeletalkey.com)
- Variable-vessel vasculitis (VVV) involves vessels (arteries, veins, capillaries) of any size and includes Behçet disease and Cogan syndrome. (musculoskeletalkey.com)
- Thus, Wegener granulomatosis, Churg-Strauss syndrome, and Henoch-Schönlein purpura have been replaced, respectively, with the terms granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and IgA vasculitis. (musculoskeletalkey.com)
Acute4
- Pulmonary fibrosis in a dog as a sequela of infection with Severe Acute Respiratory Syndrome Coronavirus 2? (biomedcentral.com)
- Considering the clinical findings, the dog was included in a serological survey for Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection in companion animals, showing positive results. (biomedcentral.com)
- HN - 2010 FX - Polysomnography MH - Acute Chest Syndrome UI - D056586 MN - C8.381.74 MN - C8.618.09 MN - C15.378.71.141.150.150.219 MN - C15.378.420.155.219 MN - C16.320.70.150.219 MN - C16.320.365.155.219 MS - Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. (nih.gov)
- HN - 2010 FX - Respiratory Distress Syndrome, Adult MH - Acute Generalized Exanthematous Pustulosis UI - D056150 MN - C17.800.33 MS - A rare skin eruption that is characterized by acute, extensive formation of sterile pustules, fever, and peripheral blood LEUKOCYTOSIS. (nih.gov)
Eosinophilic2
- Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis. (medlineplus.gov)
- Eosinophilic granulomatosis with polyangiitis , also known as Churg-Strauss syndrome, often affects the respiratory tract, gastrointestinal tract, skin, heart and nervous system. (nih.gov)
Infection2
- Cogan syndrome following SARS-COV-2 infection. (bvsalud.org)
- Multiple factors (e.g., infection, and pulmonary FAT EMBOLISM) may contribute to the development of the syndrome. (nih.gov)
Disorder2
- The 17q12 recurrent deletion syndrome is characterized by variable combinations of the three following findings: structural or functional abnormalities of the kidney and urinary tract, maturity-onset diabetes of the young type 5 (MODY5), and neurodevelopmental or neuropsychiatric disorders (e.g., developmental delay, intellectual disability, autism spectrum disorder, schizophrenia, anxiety, and bipolar disorder). (nih.gov)
- Chandler's syndrome (CS) is a rare eye disorder. (articlealley.com)
Patients2
- Efficacy and safety of 0.3% carbomer gel compared to placebo in patients with moderate-to- severe dry eye syndrome. (karabatsas.gr)
- Patients with Chandler's syndrome typically have worse corneal edema than the rest of the group, while secondary glaucoma is more severe in the other Chandler's syndrome affects females more often than males. (articlealley.com)
Rare1
- HN - 2010 MH - Alstrom Syndrome UI - D056769 MN - C10.500.300.99 MN - C10.574.500.495.99 MN - C10.668.829.800.300.99 MN - C11.270.684.249 MN - C16.131.77.80 MN - C16.131.666.300.99 MN - C16.320.290.684.249 MN - C16.320.400.375.99 MS - Rare autosomal recessive disease characterized by multiple organ dysfunction. (nih.gov)
Management3
- If endolymphatic hydrops is attributable to a given disease process-that is, if it is Ménière syndrome rather than Ménière disease-the first-line management is diagnosis and treatment of the primary disease (eg, syphilis, thyroid disease). (medscape.com)
- Management of Chandler's syndrome (CS) is case specific and should be dictated by the degree of corneal edema and severity of the secondary glaucoma. (articlealley.com)
- Topical aqueous suppressants are the medical mainstay for management of glaucoma secondary to Chandler's syndromes. (articlealley.com)
Large1
- Arteritis involving large, medium-sized, and small arteries associated with the mucocutaneous lymph node syndrome. (musculoskeletalkey.com)
Cogan's Syndrome1
- Cogan's syndrome is an autoimmune disorder that mainly affects the eyes and inner ears along with large blood vessels. (nih.gov)
Autoimmune1
- Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. (msdmanuals.com)
Systemic2
- Another important association to keep in mind is that patients with interstitial keratitis due to Cogan syndrome are more likely to have a history of systemic vasculitides, and prompt referral to a rheumatologist may be critical. (medscape.com)
- Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis. (medlineplus.gov)
Diagnosis1
- If endolymphatic hydrops is attributable to a given disease process-that is, if it is Ménière syndrome rather than Ménière disease-the first-line management is diagnosis and treatment of the primary disease (eg, thyroid disease). (medscape.com)
Affects2
- Cogan syndrome affects young adults, with 80% of patients between 14 and 47 years. (msdmanuals.com)
- Eosinophilic granulomatosis with polyangiitis , also known as Churg-Strauss syndrome, often affects the respiratory tract, gastrointestinal tract, skin, heart and nervous system. (nih.gov)
Idiopathic2
- A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. (medlineplus.gov)
- In other words, Ménière syndrome is endolymphatic hydrops caused by a specific condition, and Ménière disease is endolymphatic hydrops of unknown etiology (ie, idiopathic endolymphatic hydrops). (medscape.com)
Respiratory2
- HN - 2010 FX - Polysomnography MH - Acute Chest Syndrome UI - D056586 MN - C8.381.74 MN - C8.618.09 MN - C15.378.71.141.150.150.219 MN - C15.378.420.155.219 MN - C16.320.70.150.219 MN - C16.320.365.155.219 MS - Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. (nih.gov)
- HN - 2010 FX - Respiratory Distress Syndrome, Adult MH - Acute Generalized Exanthematous Pustulosis UI - D056150 MN - C17.800.33 MS - A rare skin eruption that is characterized by acute, extensive formation of sterile pustules, fever, and peripheral blood LEUKOCYTOSIS. (nih.gov)