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Table 5 Summary of cases of CMV retinitis in the literature without human immunodeficiency virus infection

From: Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients—case report and comprehensive review of the literature

 

CMV retinitis following intraocular and periocular corticosteroid injectiona

CMV retinitis in the setting of immunodeficiency associated with thymoma (Good syndrome)

CMV retinitis in immunocompetent adults (non-Good syndrome)

Number of cases

n = 21, n = 21 eyes

n = 9, n = 10 eyes

n = 178, n = 248 eyes

Age (years)

Mean, 66.4 years

Mean, 56 years

Mean, 45.7 years

Median, 69 years

Median, 56 years

Median, 48.0 year

Range, 30.0–84.0 year

Range, 48–68 years

Range, 1 week–84 years

Gender

Male, 14/21 (66.6 %)

Male, 4/9 (44.4 %)

Male, 113/173 reported (65.3 %)

Female, 7/21 (33.3 %)

Female, 5/9 (55.5 %)

Female, 60/173 reported (34.7 %)

Male to female ratio, 2:1

Male to female ratio, 0.8:1

Male to female ratio, 1.88:1

% unilateral

21/21 (100 %)

8/9 (88.9 %)

108/178 (60.7 %) unilateral

Indication for corticosteroid

RVO, 7/21 (33.3 %)

N/A

N/A

DME, 8/21 (38.0 %)

Uveitic CME, 7/21 (33.3 %)

CNVM due to AMD, 2/21 (9.5 %)

IRU, 0/21 (0.0 %)

Corticosteroid dose/route

1.5–4 mg IVT, 8/21 (38.0 %)

N/A

N/A

8–20 mg, 5/21 (23.8 %)

40 mg, 2/21 (9.0 %)

FA implant, 2/21 (9.0 %)

Range, 1.5–40 mg

Time from corticosteroid dosing to retinitis (months)

Mean, 4.3 months

N/A

N/A

Median, 4.0 months

Range, 0.25–13.0 months

Timing of CMV retinitis relative to thymoma diagnosis (months)b

N/A

Retinitis diagnosed after thymoma, 8/9 (88.9 %)

N/A

Mean, 31.4 months after thymoma

Median, 24 months after thymoma

Range, 75 months after to 1 month before

Associated systemic diseasesb

N/A

Respiratory infections, 7/9 (77.7 %)

No underlying systemic illness,

9/178 (5.1 %)

Non-ocular CMV, 2/9 (22.2 %)

Organ or bone marrow transplant, 61/178 (34.3 %)

Other opportunistic infections, 3/9 (33.3 %)

Autoimmune disease, 34/178 (19.1 %)

 

Leukemia or lymphoma, 51/178 (28.7 %)

Primary immune deficiency, 10/178 (5.6 %)

Other systemic medical conditions, 24/178 (13.5 %)

Immunosuppressive medication

N/A

N/A

No medication, 55/160 reported (34.4 %)

Using medication, 105/160 reported (65.6 %)

Using multiple immunosuppressive medication, 78/105 (74.3 %)

Using chemotherapy, 51/105 (48.6 %)

Using antimetabolites or leukocyte signaling inhibitors, 49/105 (46.7 %)

Associated autoimmune diseases

N/A

Myasthenia gravis, 2/8 reported (25.0 %)

N/A

Pure red cell aplasia, 2/9 cases (22.2 %)

Associated immunologic laboratory abnormalities

N/A

Generalized lymphopenia: 3/5 reported (60.0 %)

N/A

Low CD3+ T cells (<672 /mL), 1/3 reported (33.3 %)

Low CD4+ T cells (<360/μL), 5/6 reported (83.3 %)

Low CD 8 count (<240 /μL), 0/4 reported (0.0 %)

Low CD4+/CD8+ ratio (<0.6), 5/6 reported (83.3 %)

Low NK cells (<130 /mL), 1/2 reported (50.0 %)

Low serum IgG (<8 g/L), 7/8 reported (87.5 %)

Low serum IgM (<0.5 g/L), 7/8 reported (87.5 %)

Low serum IgA (<1.1 g/L), 8/8 reported (87.5 %)

Panhypogammaglobulinemia, 7/8 reported (87.5 %)

CMV testing

Positive aqueous or vitreous PCR, 20/21 (95.2 %)

Positive aqueous PCR, 4/9 (44.4 %)

Positive aqueous PCR, 65/131 reported (49.6 %)

Confirmed by other means, 1/21 (4.8 %)

Positive vitreous PCR, 5/9 (55.5 %)

Positive vitreous PCR, 29/131 reported (22.1 %)

 

Confirmed by other means, 1/9 (11.1 %)

Confirmed by other means, 37/131 reported (28.2 %)

Zone involvedc

Zone I, 4/20 reported (20.0 %)

Zone I, 5/8 reported (62.5 %)

Zone I, 72/97 eyes reported (74.2 %)

Zone II, 17/20 reported (85.0 %)

Zone II, 4/8 reported (50 %)

Zone II, 87/97 eyes reported (89.7 %)

Zone III, 1/20 reported (5.0 %)

Zone III, 2/8 reported (25 %)

Zone III, 39/97 eyes reported (40.2 %)

Vision when retinitis was first diagnosed

Acuity better than 20/40, 2/21 eyes (9.5 %)

Acuity better than 20/40, 0/9 eyes (0.0 %)

Acuity better than 20/40, 61/179 reported eyes (34.1 %)

Acuity between 20/40 and 20/200, 11/21 eyes (52.3 %)

Acuity between 20/40 and 20/200, 7/9 eyes (77.7 %)

Acuity between 20/40 and 20/200, 70/179 reported eyes (39.1 %)

Acuity worse than 20/200, 8/21 eyes (38.0 %)

Acuity worse than 20/200, 2/9 eyes (22.2 %)

Acuity worse than 20/200, 48/179 reported eyes (26.8 %)

Retinitis treatmentd

Intravitreal therapy alone, 5/21 (23.8 %)

Intravitreal therapy alone, 1/9 (11.1 %)

Intravitreal therapy alone, 30/126 reported (23.8 %)

Systemic therapy alone, 5/21 (23.8 %)

Systemic therapy alone, 2/9 (22.2 %)

Systemic therapy alone, 57/126 reported (45.2 %)

Intravitreal and systemic therapy, 12/21 (52.3 %)

Intravitreal and systemic therapy, 6/9 (66.6 %)

Intravitreal and systemic therapy, 39/126 reported (31.0 %)

Follow-up (months)

Mean = 11.8 months

Mean = 4.56 months

Mean = 14.2 months

Median = 5.5 months

Median = 6 months

Median = 6.0 months

Range = 1–84 months

Range = 1.5–7 months

Range = 0–216 months

Vision at the last visit

Acuity better than 20/40, 2/21 eyes (9.5 %)

Acuity better than 20/40, 0/9 eyes (0.0 %)

Acuity better than 20/40, 52/171 reported eyes (30.4 %)

Acuity between 20/40 and 20/200, 8/21 eyes (38.0 %)

Acuity between 20/40 and 20/200, 5/9 eyes (55.5 %)

Acuity between 20/40 and 20/200,:64/171 reported eyes (37.4 %)

Acuity worse than 20/200, 11/21 eyes (52.3 %)

Acuity worse than 20/200, 4/9 eyes (44.4 %)

Acuity worse than 20/200, 55/171 reported eyes (32.2 %)

  1. Abbreviations: AMD age-related macular degeneration, BRVO branch retinal vein occlusion, CRVO central retinal vein occlusion, CME cystoid macular edema, CNVM choroidal neovascular membrane, DME diabetic macular edema, ERM epiretinal membrane, FA fluocinolone acetonide, IRU immune recovery uveitis, IgG immunoglobulin G, CMV cytomegalovirus, NR not reported, PCR polymerase chain reaction, CD cluster designation, NK natural killer, N/A not applicable
  2. aData used from paper done by Takakura et al. [89] currently in peer review “Ocular Immunology and Inflammation”
  3. bAll patients were HIV negative
  4. cZone definitions are as follows: Zone I defined as macula or optic nerve involvement; Zone II defined as mid-periphery; Zone 3 defined as outer periphery. Zone definitions referenced in this paper: Cunningham ET Jr, Hubbard LD, Danis RP, Holland GN. Proportionate topographic areas of retinal zones 1, 2, and 3 for use in describing infectious retinitis. Arch Ophthalmol. 2011;129(11):1507–8 [115]
  5. dDosing with each modality varied widely across studies