Cogan syndrome H16.3; H90.3;H93.1; H81.3

David Glendering Cogan (1908-1993), US ophthalmologist.

Autoimmune vasculitis with unknown pathogenesis, with infestation of large and small vessels (Iliescu DA et al. 2015).

The exact cause of the disease is not yet known; infections?

Average age at first diagnosis: 33 years; (5-69). (Mora P et al. 2017); m:w=1:1;

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).

BSG↑; CRP↑; Antineutrophil cytoplasmic antibodies (ANCA)↑; Rheumatoid factor positive.

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.

Keratitis, episcleritis and anterior uveitis can usually be treated with 1% topical prednisolone acetate 4 times daily.

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).

If left untreated, the disease can lead to corneal scarring and loss of vision and in 60-80% of patients to permanent hearing loss.

1. 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
2. Espinoza GM et al (2017) Cogan's syndrome and other ocular vasculitides. Curr Rheumatol Rep 17:24.
3. Iliescu DA et al. (2015) COGAN'S SYNDROME.Rome J Ophthalmol 59:6-13.
4. 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.
5. 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.
6. Padoan R et al (2019) Cogan's syndrome: new therapeutic approaches in the biological era. Expert Opinion Biol Ther 6:1-8.