User:Meghs0/Multifocal choroiditis

Multifocal Choroditis

In 1973, Nozik and Dorsch described 2 patients with bilateral anterior uvetis associated with a chorioretinopathy resembling the presumed ocular histoplasmosis syndrome (POHS)

(1). In 1984, Dreyer and Gass presented their series of 28 patients with uveitis and similar lesions at the level of the retinal pigment epithelium (RPE) and choriocapillaris, and called the syndrome multifocal choroiditis and panuveitis (2). In 1985, Deutsch and Tessler described 28 patients with a similar condition, which they called inflammatory pseudohistoplasmosis, but most of these patients had findings to suggest systemic diseases such as sarcoidosis, syphilis, or tuberculosis (3). Finally, in 1986, Morgan and Schatz reported 11 similar cases of a condition they called recurrent multifocal choroiditis (4). These four reports all describe a condition with fundus lesions that mimics POHS but with the addition of vitritis and, often, an anterior uveitis. MCP is one of the most common white dot chorioretinal inflammatory syndromes (1,2,4)

Clinical features

'''Patient characteristics and symptoms ''' Multifocal choroiditis and panuveitis primarily affects women (75%-100%). The ages have varied from 6 to 69 years, but most patients are in their thirties.

There appears to be no racial predilection. Most patients give no history of living in areas endemic for POHS nor do they have affected family members. Most patients have bilateral involvement (45%-79%) but there may be asymmetric involvement and many of the involved second eyes may be completely asymptomatic.

Patients usually present subacutely with decreased central vision or metamorphpsia. Other less common presenting complaints include paracentral scotomata, floaters, photopsias, mild ocular discomfort, and photophobia. Initial visual acuity is highly variable, ranging from 20/20 to light perception.

Clinical course

MCP tends to be a chronic disorder lasting several months to years. Recurrent bouts of inflammation are the rule and may occur in one or both eyes, either separately or simultaneously. New lesions can occur anywhere in the fundus. CNVM's may be present initially or develop late in the follow-up. Because of the chronic and recurrent nature of MCP, patients need to be followed closely. The visual prognosis in MCP is guarded, mostly due to choroidal CNVM's which occur in about one third of the patients.

Etiology

The cause of MCP is not fully understood. Tiedeman suggested a viral etiology when he found serologic evidence of chronic or persistent Epstein-Barr virus (EBV) infection in 10 patients with MCP which he did not find in 8 control patients(7). A subsequent study by Spaide et al. did not support this hypothesis(8). Moreover, patients with MCP do not have systemic signs of chronic EBV infection. In another recent study 7 cases of MCP were evaluated. The presence of specific antibodies in the aqueous and serum suggested recent infection with herpes zoster in 2 cases and herpes simplex in 2 cases (9). It is possible that an exogenous pathogen(s) whether viral, bacterial or fungal, initially triggers an immune response which can lead to subsequent exacerbations in the absence of the inciting pathogen. This is clearly true in POHS. Additional work is needed to more clearly define the cause of MCP.

Treatment

Periocular or systemic corticosteroids have been used to treat MCP although the best therapy for MCP is still not clear. In Dreyer and Gass's report, 6 of 18 patients had improvement of vision with steroid therapy and 2 additional patients believed that the steroids halted a rapid loss of vision. There was no change in 9 patients (2). Morgan and Schatz reported that all of the patients in their series whom they treated with systemic or periocular steroids responded well and in one patient with a macular CNVM shrinkage of the membrane and improvement in visual acuity was observed (4).Dreyer and Gass treated a similar patient with oral steroids but without any effect on the CNVM (2). Nussenblatt and Palestine observed a moderately good response to steroids but noted that the disease can be stubbornly chronic, and in such cases they recommend consideration of other immunosuppressive drugs,e.g. cyclosporine (12). Clinicians should avoid the mistake of continuing corticosteroids when there appears to be little effect. Extrafoveal membranes can be treated with laser along with oral corticosteroids. Unlike those of POHS, macular CNVM's in MCP have often extensive fibrosis, making the surgical removal of submacular neovascular nets most difficult (13).

REFERENCES

1. Nozik RA, Dorsch W : A new chorioretinopathy associated with anterior uveitis, Am J Ophthalmol 76: 758-762, 1973.

2. Dreyer RF, Gass JDM : Multifocal choroiditis and panuveitis : a syndrome that mimics ocular histoplasmosis, Arch Ophthalmol 102: 1776-1784, 1984.

3. Deutsch TA, Tessler HH : Inflammatory pseudohistoplasmosis, Ann Ophthalmol 17: 461-465, 1985.

4. Morgan CM, Schatz H : Recurrent multifocal choroiditis, Ophthalmology 93: 1138-1147, 1986.

5. Joondeph BC, Tessler HH : Multifocal choroiditis, Int Ophthalmol Clinics 30(4) : 286-290, 1990.

6. Spaide RF, Yannuzzi LA, Freund BK : Linear streaks in multifocal choroiditis and panuveitis, Retina 11(2): 229-231, 1991.

7. Tiedeman JS : Ebstein-Barr viral antibodies in multifocal choroiditis and panuveitis, Am J Ophthalmol 103: 659-663, 1987

8. Spaide RF, Sugin S, Yannuzzi LA, DeRosa JT : Ebstein-Barr virus antibodies in multifocal choroiditis and panuveitis, Am J Ophthalmol 112: 410-413, 1991.

9. Frau E, et all. : The possible role of herpes simplex virus in multifocal choroiditis and panuveitis, Int Ophthalmol 14: 365-369, 1990.

10. Spaide RF, Skerry JE, Yannuzzi LA, DeRosa JT : Lack of the HLA-DR2 specificity in multifocal choroiditis and panuveitis, Br J Ophthalmol 74: 536 537, 1990.

11. Watzke RC, Packer AJ, Folk JC et al. : Puctuate inner choroidopathy, Am J Ophthalmol 98: 572-584, 1984.

12. Nussenblatt RB, Whitcup SM, Palestine AG : Uveitis: fundamentals and clinical practice. Mosby-Year Book, Inc.2nd ed. : 373-375, 1996.

13. Nolle B, Eckardt C : Vitrectomy in multifocal choroiditis, Ger J Ophthalmol 2: 14-19, 1993.

14. Gass DJ. Stereoscopic Atlas of Macular Diseases, 4th ed. Vol.2 Mosby-Year Book, Inc.,1997; 688-693.

15. Reddy CV, Folk JC. Multifocal choroiditis with panuveitis, diffuse subretinal fibrosis, and punctate inner choroidopathy. In: Ryan SJ, Schachat AP, Murphy RP eds. Retina 2nd ed. Mosby-Year Book, Inc.,1994; Vol 2, chap.105.