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Pathogenesis and Immunotherapy of Cogan’s Syndrome*
Published in George S. Eisenbarth, Immunotherapy of Diabetes and Selected Autoimmune Diseases, 2019
Barton F. Haynes, Nancy B. Allen, C. Christine Cox, Rex M. McCallum, L. Michael Cobo
Episodes of vestibuloauditory dysfunction in CS are frequently indistinguishable clinically from episodes of Meniere’s disease.2,3 If hearing loss due to CS is treated within 2 weeks of onset of hearing dysfunction with prednisone, hearing acuity can be preserved in approximately 80% of patients.2,3 While most CS syndrome patients do not have life-threatening complications, approximately 10% of patients will develop a large vessel vasculitis syndrome with proximal aortitis and aortic insufficiency, or develop a more distal large vessel vasculitis with involvement of aortic arch, coronary, mesenteric, renal or iliac arteries.2 Moreover, while episodes of interstitial keratitis generally do not lead to vision loss, rarely CS patients can develop vision-threatening inflammatory ocular disease involving the retina and sclera.2
The cornea
Published in Mary E. Shaw, Agnes Lee, Ophthalmic Nursing, 2018
Interstitial keratitis is due to congenital syphilis, manifesting itself when the patient is aged between 5 and 20 years. The disease can also occur as a result of other complications such as leprosy and tuberculosis. Tuberculosis is on the increase in Britain (Public Health England 2015) as the number of immigrants and asylum seekers from countries with a high incidence of tuberculosis rises. Certain viruses, such as the measles virus or the mumps virus, can also cause a type of interstitial keratitis. There is involvement of the deep corneal stromal layer and, if left untreated, the entire cornea develops a ground-glass appearance. The corneal epithelium and endothelium are not involved. There is also invasion of new blood vessels from the limbus.
Connective Tissue Diseases: ENT Complications
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
This is an autoimmune disorder of young people characterized by3: ocular symptoms with non-syphilitic interstitial keratitisaudio-vestibular symptoms similar to those of Meniere’s syndrome (sudden onset of tinnitus and vertigo accompanied by gradual hearing loss)an interval between the onset of the ocular and audio-vestibular features of less than 2 years. Atypical cases occur without any inflammatory ocular manifestations, with interstitial keratitis and with delays of more than 2 years between the eye and ear symptoms. There are often neurological or systemic features of vasculitis with possible infectious or autoimmune triggers. Anti-HSP 70 antibodies may be a marker for the autoimmune nature of the hearing loss. ANCA positivity may occur. Treatment is with corticosteroids followed by immunosuppressive therapy with cyclophosphamide, methotrexate, azathioprine, cyclosporine or anti-TNF blockers such as etanercept and infliximab. Rituximab is also used.
Ocular Involvement Preceded the Onset of Cutaneous Lesions in Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Case Report
Published in Ocular Immunology and Inflammation, 2022
Renpan Zeng, Lixia Du, Danling Wang
A 10-year-old Chinese boy initially presented at our uveitis clinic with a history of recurrent papulovesicular lesions on an erythematous base and hemorrhagic crusts on both cheeks, auricles, and dorsal hands since he was 8 years old (Figure 1). Seasonality was observed, with worsening in summer months. Spontaneous healing occurred when the weather became cold with residual depressed scars (Figure 2). One year before cutaneous lesions, his parents noticed that his eyes were recurrent episodes and self resolving of redness without secretion. The visual acuity of both eyes was not affected. On examination, conjunctival hyperemia and ciliary hyperemia could be seen in both eyes (Figure 3a,b). Cornea was clear and anterior chamber was quiet. Conjunctivitis was diagnosed and transiently treated with topical corticosteroid drops. Five months before cutaneous lesions, he developed photophobia, redness, and epiphora. Ophthalmologic examination revealed bilateral mixed injection with eccentrically located corneal edema extending to the periphery and midstromal infiltrations (Figure 3c,d). Corneal staining with fluorescein green was negative and intraocular pressure was within normal limits. No keratic precipitate or signs of intraocular inflammation was noted on slit lamp examination. Ophthalmic examination was consistent with active interstitial keratitis. After topical corticosteroid drops were used, the corneal edema and infiltrations could gradually subside, but recurred at irregular intervals after discontinuation of drops.
Serpiginous-Like Choroiditis (SLC) – Morphology and Treatment Outcomes
Published in Ocular Immunology and Inflammation, 2020
Ankush Kawali, Bharathi Bavaharan, Srinivasan Sanjay, Ashwin Mohan, Padmamalini Mahendradas, Rohit Shetty
Bilaterality was seen in nine patients in D-SLC and AD-SLC, whereas all patients of P-SLC had unilateral disease. Lesions were asymmetric and in different phases of resolution in bilateral cases. Mean presenting best corrected visual acuity (BCVA) was 20/25, 20/32, and 20/50 in D-SLC, AD-SLC, and P-SLC groups, respectively (Tables 2, 3). Anterior segment was involved in five eyes in the form of interstitial keratitis (Figure 4), granulomatous peripheral keratic precipitates, episcleritis, sclero-keratitis, and chronic non-granulomatous anterior uveitis. Four of them were seen after resolution of SLC lesions (Table 1). Foveal involvement was seen in 12/14, 11/13, and 6/6 eyes in Group D-SLC, AD-SLC, and P-SLC, respectively (Table 1). Overall mantoux was positive (induration ≥ 10mm) in 54.54%, whereas QFN was positive in 55% patients and 35% were positive for both (Table 1). Ocular fluid analysis done in 15 eyes for PCR (vitreous n = 1, aqueous n = 4) yielded positive results only in five eyes (2 HSV, 2 Toxoplasma, and 1 M. TB). (Table 1)
Ocular Syphilis: An Update
Published in Ocular Immunology and Inflammation, 2019
Parthopratim Dutta Majumder, Elizabeth J. Chen, Janika Shah, Dawn Ching Wen Ho, Jyotirmay Biswas, Leo See Yin, Vishali Gupta, Carlos Pavesio, Rupesh Agrawal
Interstitial keratitis is again a rare manifestation of ocular syphilis in the post-antibiotic era. The postulated pathophysiology behind this clinical entity is an immunologic phenomenon directed against treponemal antigens deposited in the cornea. Syphilitic interstitial keratitis usually presents in late childhood or early adulthood, and rarely after 40 years of age. It is classically bilateral, and patients complain of watering and photophobia. In a retrospective study of 97 patients with interstitial keratitis, syphilis was the cause of 48.5% of bilateral inactive interstitial keratitis and 4% of active interstitial keratitis.19 It is to note that, syphilitic interstitial keratitis usually does not respond to penicillin. Instead, steroids have been shown to result in clinical improvement.20 Successful combination treatment of oral steroids with cyclosporine has been reported.21