Cogan syndromeH16.3; H90.3;H93.1; H81.3
Synonym(s)
HistoryThis section has been translated automatically.
David Glendering Cogan (1908-1993), US ophthalmologist.
DefinitionThis section has been translated automatically.
Autoimmune vasculitis with unknown pathogenesis, with infestation of large and small vessels (Iliescu DA et al. 2015).
EtiopathogenesisThis section has been translated automatically.
The exact cause of the disease is not yet known; infections?
ManifestationThis section has been translated automatically.
Average age at first diagnosis: 33 years; (5-69). (Mora P et al. 2017); m:w=1:1;
Clinical featuresThis section has been translated automatically.
The occurrence and the course of the disease are very different. The symptoms can occur simultaneously or consecutively:
- Ocular symptoms (38%):
- Bilateral interstitial keratitis or other stromal keratitis
- Episcleritis or scleritis
- Uveitis
- Papillitis
- Other orbital inflammations (e.g. vitritis, choroiditis)
Vestibulocochlear symptoms (46%): sensorineural hearing loss (25-50%) up to the rare total hearing loss Also: Tinnitus and vertigo.
After 5 months, 75% of patients have both ocular and vestibulocochlear symptoms. Non-specific symptoms are fever, headache, joint pain and myalgia.
Vasculitic symptoms (10-30%): infestation of the major arteries possible. Occurrence of Takayasu arteritis possible (Kawasaki Y et al. 2018). Furthermore, inflammation of the aorta (aneurysm), the aortic and mitral valves (valvulitis), the vessels of the extremities (intermittent claudication) are possible (Beltagy A et al. 2019).
Skin symptoms: In rare cases skin symptoms in the form of "small vessel vasculitis" as well as signs of panniculitis may occur (Kawasaki Y et al. 2018).
LaboratoryThis section has been translated automatically.
BSG↑; CRP↑; Antineutrophil cytoplasmic antibodies (ANCA)↑; Rheumatoid factor positive.
DiagnosisThis section has been translated automatically.
The diagnosis is based on clinical findings and the exclusion of other causes (e.g. syphilis, Lyme borreliosis, Epstein-Barr virus infection) by means of appropriate serological tests.
External therapyThis section has been translated automatically.
Keratitis, episcleritis and anterior uveitis can usually be treated with 1% topical prednisolone acetate 4 times daily.
Internal therapyThis section has been translated automatically.
Systemic: depending on the acuteity of the symptoms prednisone 1 mg/kg p.o. once/day for 2-6 months continued In case of therapy resistance or recurrence additionally: cyclophosphamide, methotrexate or ciclosporin (Padoan R et al. 2019).
Note: The use of immunomodulating biologicals is discussed (Mora P et al.2017). Results for Infliximab are available. With regard to the efficiency of rituximab and tocilizumab further study results are necessary (Padoan R et al. 2019).
Progression/forecastThis section has been translated automatically.
If left untreated, the disease can lead to corneal scarring and loss of vision and in 60-80% of patients to permanent hearing loss.
LiteratureThis section has been translated automatically.
- Beltagy A et al (2019) Echocardiography. Aortic valve perforation in the setting of Cogan's syndrome.doi: 10.1111/echo.14428 https://www.ncbi.nlm.nih.gov/pubmed/31246322
- Espinoza GM et al (2017) Cogan's syndrome and other ocular vasculitides. Curr Rheumatol Rep 17:24.
- Iliescu DA et al. (2015) COGAN'S SYNDROME.Rome J Ophthalmol 59:6-13.
- Kawasaki Y et al. (2018) Cutaneous vasculitis in Cogan's syndrome: A Report of Two Cases Associated withChlamydia Infection.J Nippon Med Sch 85:172-177.
- Mora P et al (2017) Cogan's syndrome: State of the art of systemic immunosuppressive treatment in adult and pediatric patients.Autoimmune Rev 16:385-390.
- Padoan R et al (2019) Cogan's syndrome: new therapeutic approaches in the biological era. Expert Opinion Biol Ther 6:1-8.