Immunoglobulin G4-related disease involving the bladder wall and urethra
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UROLOGY - LETTER TO THE EDITOR
Immunoglobulin G4‑related disease involving the bladder wall and urethra Zaizhu Zhang1 · Wei Yu1 · Wenmin Guan1 · Qiang Lin1 Received: 3 March 2020 / Accepted: 15 April 2020 © Springer Nature B.V. 2020
Editor, Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition associated with fibroinflammatory lesions that can occur at nearly any anatomic site [1]. However, vesical and urethral involvement is much less common and often misdiagnosed [2]. Thus, urologists should perform preliminary assessments to avoid inappropriate or unnecessary treatments [2]. Herein, we present a 75-year-old woman with definite IgG4-RD affecting the urinary bladder and urethra. A 75-year-old woman was admitted with a chief complaint of urinary retention without dysuria, urinary frequency and hematuria for one month. On admission, pelvic magnetic resonance imaging (MRI) revealed a well-circumscribed, nodular mass at the right anterior bladder wall with a low signal intensity on T2-weighted image and another hypointense mass encased the urethra, extending from the distal urethra to the bladder neck (Fig. 1a, b). Under a clinical suspected diagnosis of bladder cancer, a transurethral resection of the bladder mass was carried out to allow for definitive tissue sampling. Pathological findings demonstrated that dense lymphoplasmacytic and eosinophilic infiltration in submucosa with phlebitis. On immunohistochemical studies, CD138-positive, CD38-positive, CD20-positive, CD3-positive and IgG4-positive plasma cells were scattered, and the IgG4/IgG ratio was more than 40%. Serum IgG4 was elevated to 3670 mg/dl (reference range 8–140 mg/dl). IgG4-RD was diagnosed based on the clinical and histopathologic findings [3].
* Qiang Lin [email protected] 1
Given definitive diagnosis of IgG4-RD, the patient was treated with oral prednisolone, 40 mg daily, for 2 weeks. The dose was tapered by 5 mg each week for 6 weeks to reach a daily target of 10 mg, which was maintained for 12 weeks. After 20 months, the follow-up pelvic MRI showed that the bladder mass disappeared and the periurethral lesion markedly decreased in size (Fig. 1c, d). Serum IgG4 was reduced to 357 mg/dl. Only few cases of vesical or urethral involvement with IgG4-related disease were reported [4–10]. To our knowledge, this is the first report of IgG4-RD involving both urinary bladder and urethra. The bladder mass was transurethrally resected without recurrence in a 20-month follow-up. Our case suggests that complete surgical removal of the lesions might give the patient a disease-free status, which was similar to the cases reported by Park et al. [5] and Kufukihara et al. [8]. In light of the recurrence of the bladder mass without surgical resection reported by Gehring et al. [7], the operation with or without steroid treatment might be a preferable choice. The periurethral mass markedly decreased in size and urine retention disappeared after treatment of prednisolone, which indicated the lesion had effective response to corticosteroid
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