Importance of proper diagnosis for management: multifocal choroiditis mimicking ocular histoplasmosis syndrome

Hatef, Elham; Turkcuoglu, Peykan; Ibrahim, Mohamed; Sepah, Yasir; Shulman, Matthew; Heo, Jangwon; Lee, Jeong Hee; Channa, Roomasa; Khwaja, Afsheen; Rentiya, Zubir; Shah, Syed Mahmood; Do, Diana V.; Nguyen, Quan Dong

doi:10.1007/s12348-010-0016-4

Journal of Ophthalmic Inflammation and Infection

Table 1 Demographic, clinical, and serologic characteristics of the patients

From: Importance of proper diagnosis for management: multifocal choroiditis mimicking ocular histoplasmosis syndrome

Case no./age, yr/race/sex/affected eye	Visual acuity		F/U (mo)	AC/vitreous reaction at initial visit	Funduscopic findings at initial visit	Serologic findings at initial visit	Imaging findings at initial visit	Treatment and clinical course
Case no./age, yr/race/sex/affected eye	Initial	Final	F/U (mo)	AC/vitreous reaction at initial visit	Funduscopic findings at initial visit	Serologic findings at initial visit	Imaging findings at initial visit	Treatment and clinical course
1/47.57/W/F/BE	20/20 RE 20/400 LE	20/16 RE 20/250 LE	14.51	No evidence of iridocyclitis; no inflammatory cells in the vitreous	BE: Peripapillary atrophy; multiple pigmented, chorioretinal lesions scattering in the peripheral retina	Histoplasma Antigen: N	FA: RE: No evidence of any leakage within the foveal area; leakage of two isolated lesions	Past Ocular Treatment
					RE: 2 small yellowish, deep chorioretinal lesions, 1 superonasal to the foveal center, 1 inferotemporal to the foveal center	Histoplasma Antibody: N	LE: staining of the fibrotic scar, no active leakage	Laser photocoagulation and photodynamic therapy for CNV as well as intravitreal injection of bevacizumab
					LE: A large fibrotic scar in macula	Muramidase (Lysozyme): N	OCT: RE; RPE disruption in areas corresponded to the two lesions superonasal and inferotemporal to the fovea	Treatments at the Wilmer Eye Institute
						ACE: N		Methylprednisolone 1,000 mg/IV a day for 3 days. Prednisone 60 mg/PO a day tapered to 12.5 mg/daily over the period of 6 months; mycophenolate mofetil 2,000 mg/PO daily increased to 2,500 mg/PO daily after 4 months. Despite an increase in the dose of mycophenolate mofetil, on repeated OCTs, there were enlargements of the 2 lesions in the right eye compared to previous OCTs
						RPR Screen: NR		The patient was then enrolled in a clinical trial of local treatment of sirolimus, which helped to reduce the size of the lesions and allowed tapering of prednisone
						FTA-ABS-Serum: NR
						ANA Screen: N
						Anti-DNA: N
						RNP Antibody: N
						Smith Antibody: N
						Anti-RO (SS-A): N
						Anti-LA (SS-B): N
						SCL-70: N
						EBV IgG: P
						Lyme Disease Antibody: N
2/31.33/W/F/BE	20/80 RE 20/63 LE	20/40 RE 20/32 LE	3.16	No evidence of iridocyclitis; no inflammatory cells in the vitreous	RE; Several chorioretinal lesions within and outside of arcade, nasal to optic nerves and in the inferonasal quadrants	Histoplasma Antigen: N Histoplasma Antibody: N	FA: No leakage.	Past Ocular Treatment
					LE; Few chorioretinal scars within and outside of arcades, a curvilinear area of chorioretinal lesions in temporal area consistent with MFC	HSV type I IgG: P	Autofluorescence: areas of hyperfluorescence surrounding various lesions in RE	Prednisone 80 mg/daily for RE involvement. Methotrexate was added for 3 months after LE involvement. Methotrexate was discontinued. With a strong suspicion of PIC she was kept on prednisone 80 mg/daily
						HSV type II IgG: N	OCT: RE; Hyper reflective scar tissue involves the foveal center, starting from sub-RPE level extending to the inner plexiform layer	Treatment at the Wilmer Eye Institute Mycophenolate mofetil 1 g/twice a day. Prednisone tapered after 10 days of administration of mycophenolate mofetil. Three months later, prednisone tapered to 20 mg. During the last visit, funduscopic examination revealed stable findings of multiple chorioretinal lesions mainly in the posterior pole. There had been also a decrease in the amount of hyperfluorescence on autofluorescence, and a decrease in the amount of RPE disruption on OCTs in both eyes
						Muramidase (Lysozyme): N
						ACE: N
						RPR Screen: NR
						FTA-ABS-Serum: NR
						VZV IgG Antibody: P
						Toxoplasma IgM: N
						Toxoplasma IgG: N
						EBV IgM: N
3/50.14/W/F/BE	20/250 RE 20/25 LE	20/160 RE 20/25 LE	1.18	No evidence of iridocyclitis; no inflammatory cells in the vitreous	BE: Multiple hypopigmented chorioretinal spots in the mid periphery RE: Edema and subretinal fibrosis through the fovea	Histoplasma Antigen: N Histoplasma Antibody: N	FA: RE: Hyperfluorescence and leakage from many hypopigmented spots in the mid periphery as well as mild leakage from some of the vasculature in the peripheral retina; no optic disk leakage or staining, hyperfluorescence in the fovea corresponding to the area of fibrosis	Past Ocular Treatment
					LE: Pigmentary changes without edema through the fovea	ANA Screen: N	LE: Leakage from the hypopigmented peripheral spots; no optic disk leakage or staining, no macular edema	Bevacizumab 4 injections for CNV.
						Anti-DNA: N	OCT: RE: Intraretinal edema through the center of the fovea	Treatment at the Wilmer Eye Institute
						RNP Antibody: N	LE: very mild intraretinal edema	We recommended a consult with her hematologist/oncologist to evaluate for a possible hematologic underlying disease prior to initiation of potential treatment for multifocal choroiditis
						Smith Antibody: N
						Anti-RO (SS-A): N
						Anti-LA (SS-B): N
						HSV type I IgG: N
						HSV type II IgG: N
						VZV IgG Antibody: N
						Toxoplasma IgM: N
						Toxoplasma IgG: N
						EBV IgG: N
4/26.91/W/F/BE	20/200 RE 20/20 LE	20/125 RE 20/20 LE	1.68	No evidence of Iridocyclitis; no inflammatory cells in the vitreous	RE: Multiple small discrete pigmented chorioretinal lesions scattered in posterior pole and throughout the peripheral retina. Atrophic changes and subretinal fibrosis in the macula	Histoplasma Antigen: N Histoplasma Antibody: N	FA: RE: Multiple small lesions scattered in posterior pole and throughout the peripheral retina without leakage. Scar staining noticed at the foveal center	Past Ocular Treatment
					LE: Few scattered chorioretinal lesions limited mainly to nasal area of optic nerve. Ring of pigment next to the foveal center with no subretinal fluid or hemorrhage	Muramidase (Lysozyme): N	LE: Classic CNV filling pattern noticed without leakage at late phase	Bevacizumab2 injections for CNV
						ACE: N	OCT: RE: Multiple lesions involve different layers of retina and sub-RPE in some areas, including foveal center which result in intra retinal edema, more pronounced nasally	Treatment at the Wilmer Eye Institute
						RPR Screen: NR	LE: presence of a PED	The patient was monitored. Immunomodulatory therapy would be started to decrease the risk of inflammations in case of recurrent CNV in LE. Vascular endothelial growth factor antagonist would be employed as well when there is recurrent CNV
						FTA-ABS-Serum: NR
						ANA Screen: N
						HSV type I IgG: N
						HSV type II IgG: N
						EBV IgG: P
						EBV IgM: N
						Toxoplasma IgM: N
						Toxoplasma IgG: N
5/38.01/W/F/BE	20/63 RE 20/20 LE	20/25 RE 20/16 LE	2.83	No evidence of iridocyclitis; no definite inflammatory cells in the vitreous	RE: Multiple chorioretinal lesions, much more in the nasal aspect of retina. Deep chorioretinal scar on the temporal aspect of foveal center with no subretinal fluid and two lesions in the temporal peripheral retina	Histoplasma Antigen: N Histoplasma Antibody: N	FA: RE: chorioretinal anastomosis in foveal center without significant leakage	Past Ocular Treatment
					LE: Chorioretinal lesions in the nasal aspect of the retina. Macula appeared normal		OCT: RE: Scar tissue involved RPE and photoreceptor layer, no intraretinal edema	Photodynamic therapy and intraocular corticosteroid followed by one intravitreal injection of bevacizumab
							AF: RE: hyperfluorescence surrounding several chorioretinal lesions	Treatment at the Wilmer Eye Institute
								Mycophenolate mofetil 1 g twice a day. During the most recent visit, the lesions appeared to be much less active. On FA, there was no evidence of leakage in the macula of either eye
								Also, there had been much decrease in the amount of fluorescence of macular lesions in RE on AF
6/31.96/W/F/BE	20/20 RE 20/80 LE	20/125 RE 20/125 LE	5.69	No evidence of Iridocyclitis; no inflammatory cells in the vitreous	BE: Chorioretinal lesions inferiorly, LE worse than RE	Histoplasma Antigen: N		Past Ocular Treatment
					LE: yellowish lesion inferonasal to foveal center	Histoplasma Antibody: N		Bevacizumab injections for CNV
								Treatment at the Wilmer Eye Institute
								Prednisone 60 mg/daily for 1 month tapered by 10 mg/daily every 3 weeks. Mycophenolate mofetil 1,000 mg twice a day and increased to 2,500 mg daily after 2 months; cyclosporine (4 mg/kg) was also started. During the most recent visit, AF did not provide any new lesion
7/36.16/W/F/BE	20/200 RE 20/25 LE	20/200 RE 20/25 LE	5.23	No evidence of iridocyclitis	RE: Disciform scar in foveal center and curvilinear distribution of multiple chorioretinal lesions in peripheral retina, especially nasally	Histoplasma Antigen: N Histoplasma Antibody: N	FA: There was no leakage on the FA to suggest active CNV	Past Ocular Treatment
				no definite inflammatory cells in the vitreous	LE: curvilinear distribution of multiple chorioretinal lesions in peripheral retina especially nasal aspect	RPR Screen: NR	OCT: RE: Disciform scar in foveal center involved RPE and photoreceptor layer, no intra retinal edema	Photodynamic therapy (3 times) and 4 intravitreal injections of bevacizumab in RE. 1 intra vitreal injection of bevacizumab in LE
						FTA-ABS-Serum: NR	LE: 5 lesions surrounding foveal center with involvement of RPE and disruption of the photoreceptor layer	Treatment at the Wilmer Eye Institute
						ANA Screen: P		Intravenous methyprednisolone daily for 3 days. Prednisone 60 mg/daily for 2 weeks then tapered to 9 mg/daily in a period of 4 months
					There were 5 lesions with blurry borders surrounding the foveal center	Anti-DNA: N
						RNP Antibody: N
						Smith Antibody: N
						Anti-RO (SS-A): N
						Anti-LA (SS-B): N
						Lyme Disease Antibody (ELISA): equivocal for antibody
						Lyme Disease Antibody (Western Blot): N		Mycophenolate mofetil 1,000 mg twice a day for 3 months, then increased to 1, 500 mg twice a day. During the last visit, on AF, there appeared to be decreased hyperfluorescence surrounding the lesion in RE, especially in nasal aspect of the retina. AF inLE appeared to be stable. There was no clear leakage seen on the macula of either eye.
8/69.22/W/F/BE	20/200 RE 20/400 LE	20/200 RE 20/250 LE	2.30	No evidence of iridocyclitis; no inflammatory cells in the vitreous	BE: Peripapillary atrophy, fibrotic scars in macula and multiple chorioretinal lesions in the peripheral retina; no subretinal fluid or heme	Histoplasma Antigen: N Histoplasma Antibody: N	FA: There was no leakage on the FA to suggest active CNV	Past Ocular Treatment
						Muramidase (Lysozyme): N	OCT: BE: Scars in foveal center involved RPE and photoreceptor layer; no intra retinal edema	5–6 intravitreal injections of bevacizumab in LE
						ACE: N		Treatment at the Wilmer Eye Institute
						RPR Screen: NR
						FTA-ABS-Serum: NR		Monitoring the patient and evaluations of tuberculosis. No IMT was initiated
9/61.97/W/M/LE	20/20 RE 20/400 LE	20/20 RE 20/320 LE	12.20	No evidence of iridocyclitis; no inflammatory cells in the vitreous	LE: Pigmentary changes as well as laser scar and atrophic changes within macula	Histoplasma Antigen: N Histoplasma Antibody: N	FA: There was no leakage on FA to suggest active CNV	Past Ocular Treatment
							OCT: BE: Scar in foveal center involved RPE and photoreceptor layer; no intraretinal edema	Laser photocoagulation for CNV in LE
								Treatment at the Wilmer Eye Institute
								The patient was monitored as there was no evidence of active inflammation

Bold text in the table represents clinical findings that were suggestive of active inflammation
F/U follow-up, AC anterior chamber, W white, F female, M male, RE right eye, LE left eye, BE both eyes, Mo month, P positive, N negative, NR nonreactive, ANA antinuclear antibody, HSV Herpes simplex virus, ACE angiotensin-converting enzyme, RPR rapid plasma regain, FTA-ABS fluorescent treponemal antibody absorption, VZV Varicella zoster virus, EBV Epstein–Barr virus, FA fluorescein angiography, AF auto-fluorescein, OCT optical coherence tomography, CNV choroidal neovascularization, IV intravenous, PO per oral, PED pigment epithelial detachment, IMT immunomodulatory therapy

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