Patients with Sjögren syndrome (SS) have a higher risk of developing malignant lymphoma.1
A 68-year-old woman presented with a 2-month history of swollen parotid glands. The patient had psoriatic arthritis and had been treated with secukinumab for 3 months. At presentation, she had bilateral swollen and ulcerated parotid glands (Figure 1A) with sicca symptoms. Laboratory data showed positivity for anti-SSA and anti-SSB antibodies and elevated C-reactive protein (15.2 mg/L). There was dry keratoconjunctivitis and decreased salivary production on gum testing. Antibiotic therapy was ineffective; secukinumab was stopped, and a left parotid gland biopsy was performed. Pathology demonstrated infiltration of CD20-positive neoplastic lymphocytes and lymphoepithelial lesions (Figure 2A-D). Immunoglobulin heavy chain gene rearrangement was positive. Epstein-Barr virus–encoded RNA in situ hybridization was negative. A diagnosis of extranodal marginal zone lymphoma of mucosaassociated lymphoid tissue (MALT lymphoma) with SS was made. Positron emission tomography/computed tomography (CT) revealed a nodule with fluorodeoxyglucose uptake in the lung, which we classified as stage IV. While waiting for the biopsy result, the patient’s parotid glands shrank spontaneously, and the skin ulcer showed scarring (Figure 1B). The lung nodule also shrank, as seen in the 1-year follow-up CT. Since then, the patient has been followed up without any exacerbation, even 2 years after the diagnosis.
Bilateral extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a patient with Sjögren syndrome. (A) Bilateral swollen parotid glands with ulceration. (B) Spontaneous shrinkage of the parotid glands 1 month after discontinuation of secukinumab.
Pathology of the left swollen parotid gland. (A) The parotid gland demonstrates infiltration of neoplastic lymphocytes. (B) CD20 immunostaining image. Neoplastic lymphocytes are CD20 positive (white arrow) and cause lymphoepithelial lesions (black arrow). (C,D) Demonstration of (C) kappa chains and (D) lambda chains by ISH. Kappa-dominant light chain restriction was revealed by ISH. ISH: in situ hybridization.
In general, MALT lymphoma is clinically indolent.2 Interleukin (IL)-17 both promotes and inhibits tumorigenesis; however, the mechanism remains unclear.3 In this case, the rapid progression and spontaneous shrinkage of parotid gland lymphoma suggested that MALT lymphoma in SS could be improved by discontinuing IL-17 inhibitors.
Footnotes
MK reports grants and/or speaking fees from AbbVie, Asahi-Kasei, Astellas, AstraZeneca, Ayumi, BMS, Chugai, Daiichi Sankyo, Eisai, Eli Lilly, Gilead Sciences, GSK, Janssen, Kowa, Kyocera, Lotte, Nippon Boehringer Ingelheim, Nippon Shinyaku, Mitsubishi Tanabe, Mochida, Pfizer, Taiju Life Social Welfare Foundation, Taisho, Takeda, Terumo, UCB Japan, and Yamazaki Baking, outside the submitted work. TA has received grants, consulting fees, and/or speaking fees from Astellas, Mitsubishi Tanabe, Chugai, Daiichi Sankyo, Pfizer, Teijin, Novartis, Sanofi, GSK, AbbVie, AstraZeneca, Nippon Boehringer Ingelheim, Janssen, Gilead Sciences, Eli Lilly, ONO, Takeda, Alexion, Kyowa Kirin, Amgen, UCB Japan, and Esai, outside the submitted work. The remaining authors declare no conflicts of interest relevant to this article. The patient described in the case provided informed consent. Ethics approval is waived for case reports in Japan.
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