Observation on the therapeutic effect of intraorbital injection of compound betamethasone on the treatment of IgG4-related ophthalmic disease

doi:10.3969/j.issn.1674-8115.2023.02.011

Abstract

Abstract:

Objective ·To investigate the effect of intraorbital injection of compound betamethasone on IgG4-related ophthalmic disease (IgG4-ROD). Methods ·Patients with IgG4-ROD who received intraorbital injection of compound betamethasone in the Department of Ophthalmology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from September 2016 to August 2021 were included. The enrolled patients met the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease (IgG4-RD) and all had lacrimal gland involvement. The intraorbital administration regimen was 0.5 mL compound betamethasone (containing betamethasone propionate 5 mg/mL, and betamethasone sodium phosphate 2 mg/mL) into the affected orbit once a week for 6 weeks, and then once a month for 3 months. The drug was injected into the lacrimal gland and its surrounding superficial orbital tissue. For patients with extraocular muscle and optic nerve involvement, periocular injection was added. Before and after treatment, the IgG4-RD responder index (IgG4-RD RI) was recorded according to the clinical manifestations, serum IgG4 level and imaging findings. If recurrence was determined according to the IgG4-RD RI, maintenance treatment was adopted. Maintenance treatment regimen was once a month for 6 months, and then changed to bimonthly according to the doctor's judgment. Results ·According to the IgG4-RD RI before and after treatment, all patients achieved complete remission after initial treatment with remission periods of 4, 4, 6, and 5 months in case 1, 2, 3 and 4, respectively, but relapse occurred. Despite recurrence, case 1, 3, and 4 remained in remission with maintenance therapy in the follow-up of 50, 45, and 27 months, respectively. Due to the low total dosage of glucocorticoid, no obvious systemic and local side effects occurred during treatment. Also no serious injection-related side effects occurred. Case 2 refused maintenance therapy and the lacrimal gland returned to its original size after discontinuation, but did not aggravate further in the 47 months follow-up. Conclusion ·Intraorbital injection of compound betamethasone may be a useful treatment option for IgG4-ROD. However, patients require repeat injections due to relapse.

Key words: IgG4-related ophthalmic disease (IgG4-ROD), betamethasone, intraorbital injection, treatment response

CLC Number:

R777.5

LIU Xiaohong, WANG Yanuo, JIAO Qin, CHENG Yu. Observation on the therapeutic effect of intraorbital injection of compound betamethasone on the treatment of IgG4-related ophthalmic disease[J]. Journal of Shanghai Jiao Tong University (Medical Science), 2023, 43(2): 215-221.

Figures/Tables 5

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References 17

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Item	Case 1	Case 2	Case 3	Case 4
Gender	Male	Male	Female	Male
Age/year	76	71	44	58
Brief history of illness	Progressive exophthalmos for 1 year, suspected of thyroid-associated ophthalmopathy	Swelling of the left upper eyelid for 5 months, slowly worsened	Bilateral upper eyelids swelling for 1 year, slowly worsened	Bilateral upper eyelids swelling for half a year （mainly the right）, slowly worsened
Sites of orbital involvement	Bilateral lacrimal glands, some extraocular muscles, optic nerve	Bilateral lacrimal glands （mainly the left）	Bilateral lacrimal glands	Bilateral lacrimal glands （mainly the right）
Extraocular sites of involvement	Suspected submandibular gland lesions （resected in other hospital）	Submandibular gland enlargement, tonsillitis	Submandibular lymph nodes enlargement, pharyngitis	Sinusitis, left parotid gland lesion （partial resection）
Pathology	No	No	No	IgG4-RD confirmed by another hospital
Corrected visual acuity^① （R/L）	0.6/0.4	0.9/0.8	1.2/1.2	1.0/1.0
NCT （R/L）/ mmHg	20.1/23.4	17.6/18.2	13.3/16.4	15.2/14.8
Conjunctiva	Hyperaemia （＋＋）, chemosis （＋）	Hyperaemia （-）	Hyperaemia （-）	Hyperaemia （-）
Anterior segment	Punctate epithelial erosions in the left, bilateral mild nuclear sclerosis	Bilateral mild nuclear sclerosis	（-）	（-）
Optic disc	Swelling	（-）	（-）	（-）
Macula	（-）	（-）	（-）	（-）
Exophthalmus （R/L）/mm	21/23	15/16	18/18	17/15
Serum IgG4/（g·L^-1）^②	24.10	28.90	4.10	14.40
Serum IgG/（g·L^-1）^③	29.00	18.10	12.20	26.70
IgG4-RD RI	10	6	6	6

Item	Case 1	Case 2	Case 3	Case 4
Gender	Male	Male	Female	Male
Age/year	76	71	44	58
Brief history of illness	Progressive exophthalmos for 1 year, suspected of thyroid-associated ophthalmopathy	Swelling of the left upper eyelid for 5 months, slowly worsened	Bilateral upper eyelids swelling for 1 year, slowly worsened	Bilateral upper eyelids swelling for half a year （mainly the right）, slowly worsened
Sites of orbital involvement	Bilateral lacrimal glands, some extraocular muscles, optic nerve	Bilateral lacrimal glands （mainly the left）	Bilateral lacrimal glands	Bilateral lacrimal glands （mainly the right）
Extraocular sites of involvement	Suspected submandibular gland lesions （resected in other hospital）	Submandibular gland enlargement, tonsillitis	Submandibular lymph nodes enlargement, pharyngitis	Sinusitis, left parotid gland lesion （partial resection）
Pathology	No	No	No	IgG4-RD confirmed by another hospital
Corrected visual acuity^① （R/L）	0.6/0.4	0.9/0.8	1.2/1.2	1.0/1.0
NCT （R/L）/ mmHg	20.1/23.4	17.6/18.2	13.3/16.4	15.2/14.8
Conjunctiva	Hyperaemia （＋＋）, chemosis （＋）	Hyperaemia （-）	Hyperaemia （-）	Hyperaemia （-）
Anterior segment	Punctate epithelial erosions in the left, bilateral mild nuclear sclerosis	Bilateral mild nuclear sclerosis	（-）	（-）
Optic disc	Swelling	（-）	（-）	（-）
Macula	（-）	（-）	（-）	（-）
Exophthalmus （R/L）/mm	21/23	15/16	18/18	17/15
Serum IgG4/（g·L^-1）^②	24.10	28.90	4.10	14.40
Serum IgG/（g·L^-1）^③	29.00	18.10	12.20	26.70
IgG4-RD RI	10	6	6	6

Item	Case 1	Case 2	Case 3	Case 4
Corrected visual acuity^① (R/L)	0.7/0.6	0.9/0.8	1.2/1.2	1.0/1.0
NCT (R/L)/mmHg	13.6/12.5	17.6/18.2	14.3/15.4	16.2/14.8
Conjunctiva	Hyperaemia (-), chemosis (-)	Hyperaemia (-)	Hyperaemia (-)	Hyperaemia (-)
Anterior segment	Bilateral mild nuclear sclerosis	Bilateral mild nuclear sclerosis	(-)	(-)
Optic disc	No swelling	(-)	(-)	(-)
Macula	(-)	(-)	(-)	(-)
Exophthalmus (R/L)/mm	14/14	15/14	16/16	15/15

Item	Case 1	Case 2	Case 3	Case 4
Corrected visual acuity^① (R/L)	0.7/0.6	0.9/0.8	1.2/1.2	1.0/1.0
NCT (R/L)/mmHg	13.6/12.5	17.6/18.2	14.3/15.4	16.2/14.8
Conjunctiva	Hyperaemia (-), chemosis (-)	Hyperaemia (-)	Hyperaemia (-)	Hyperaemia (-)
Anterior segment	Bilateral mild nuclear sclerosis	Bilateral mild nuclear sclerosis	(-)	(-)
Optic disc	No swelling	(-)	(-)	(-)
Macula	(-)	(-)	(-)	(-)
Exophthalmus (R/L)/mm	14/14	15/14	16/16	15/15

Item	Case 1	Case 2	Case 3	Case 4
1 month after treatment
Serum IgG4/(g·L^-1)	5.8	8.3	2.5	11.0
IgG4-RD RI	4	3	3	3
3 months after treatment
Serum IgG4/(g·L^-1)	3.8	9.8	1.3	3.7
IgG4-RD RI	2	2	2	3
6 months after treatment
Serum IgG4/(g·L^-1)	8.4	11.9	2.1	4.5
IgG4-RD RI	3	3	3	3
Duration of remission/month	4	4	6	5
The last follow up
Serum IgG4/(g·L^-1)	4.2	42.9	1.1	6.0
IgG4-RD RI	2	3	2	2