PMC6384422_01
A) On admission. Both upper eyelids (1) and the left dorsum of the hand (2) were swollen. Joint pain and limited range of motion were also present. B) After steroid therapy. Swelling of upper eyelid (1) and left dorsum of the hand (2) were improved. Joint pain had disappeared and range of motion was improved
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Both upper eyelids, the salivary gland, the dorsum of the left hand and both legs were swollen (Fig. 1A-1, 2). After lacrimal gland biopsy, he was diagnosed on MD and oral prednisolone was started at 40 mg/day, and the clinical symptoms (Fig. 1B-1, 2) and EMG findings improved.
A, B) Histopathologic examination of lacrimal gland biopsy revealed lymphoid follicles with germinal center. IgG-positive plasma cells on the lymphoid follicles were visualized by enzyme immunostaining. C, D) Enzyme immunostaining with an anti-IgG4 antibody revealed IgG-positive plasma cells, accounting for about a half of IgG4-positive cells on the lymphoid follicles
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Both upper eyelids, the salivary gland, the dorsum of the left hand and both legs were swollen (Fig. 1A-1, 2). Enzyme immunostaining with anti-IgG4 antibody revealed IgG-positive plasma cells, of which about a half were IgG4-positive, on the lymphoid follicles (Fig. 2). After lacrimal gland biopsy, he was diagnosed on MD and oral prednisolone was started at 40 mg/day, and the clinical symptoms (Fig. 1B-1, 2) and EMG findings improved.
On admission, antibiotic therapy (cefazolin 2 g/day) was started to treat cellulitis. Non-steroidal anti-inflammatory drugs (loxoprofen 180 mg/day) and benzbromarone were also started to treat polyarthritis with hyperuricemia. After a diagnosis of IgG4 related disorder, oral prednisolone was started at 40 mg/day. After starting oral prednisolone, the clinical symptoms and nerve conduction study findings improved. After discharge, the steroid dose was tapered. But at the time of reducing 3 mg/day of prednisolone dose, both lacrimal gland swelling and gait disturbance reappeared. Serum IgG4 was also elevated. The steroid dose was increased to 10 mg/day and these symptoms improved
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The clinical course is shown in Fig. 3.