An atypical case of lymphoproliferative pulmonary involvement in a patient with Sjögren’s syndrome: a case report

BMC Research Notes

Jun-ichi Kadota²  Chie Yasuda²  Hisako Kushima²  Kosaku Komiya²  Hiroshi Ishii¹  Hiroaki Oka² 

[1] Department of Respiratory Medicine, Faculty of Medicine, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-0180, Japan;Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine, Oita, Japan

关键词: T-cells;    Lymphoproliferative pulmonary disease;    Sjögren’s syndrome;   

Others  :  1141683
DOI  :  10.1186/1756-0500-6-367

 received in 2013-04-02, accepted in 2013-09-10,  发布年份 2013

【 摘 要 】

Background

Sjögren’s syndrome is characterized by lymphocytic infiltration of the exocrine glands, together with polyclonal B-cell activation, and lung diseases are well-known complications of the disease. Therefore, in most cases associated with Sjögren’s syndrome, infiltrating lymphocytes in the lung specimen exhibit the features of B-cells. We herein report an atypical case of lymphoproliferative pulmonary involvement in a patient with Sjögren’s syndrome.

Case presentation

A 46-year-old female was admitted to our hospital because of an abnormal chest roentgenogram finding on a medical checkup. Chest computed tomography showed randomly-distributed micronodules and patchy ground-glass opacities. A surgical biopsied specimen showed an atypical pattern of interstitial pneumonia with numerous lymphoid follicles. Among the infiltrating lymphocytes in the lung, only the monoclonality of the T-cells was proven by a gene rearrangement analysis, but there was no cytological atypicality or genetic disorder revealed by testing the bone marrow aspirate. A diagnosis of Sjögren’s syndrome was made based on the patient’s other symptoms and these negative findings. The patient’s pulmonary lesions have been successfully treated and remission has been maintained for over three years with corticosteroid treatment alone.

Conclusion

The present patient was an atypical case of lymphoproliferative pulmonary involvement in a patient with Sjögren’s syndrome. Although monoclonality of the infiltrating T-cells was proven, the clinical course and the findings of the imaging and laboratory examinations were inconsistent with the previously-reported cases of primary pulmonary T-cell lymphoma. This suggests that the monoclonality of lymphocytes does not always define malignancy. The diagnosis of malignant lymphoma or lymphoproliferative diseases should be made clinically, pathologically and cytogenetically to rule out other similar diseases.

【 授权许可】

   
2013 Oka et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

【 参考文献 】

• [1]Cornec D, Devauchelle-Pensec V, Tobon GJ, Pers JO, Jousse-Joulin S, Saraux A: B cells in Sjogren’s syndrome: from pathophysiology to diagnosis and treatment. J Autoimmun 2012, 39:161-167.
• [2]Ito I, Nagai S, Kitaichi M, Nicholson AG, Johkoh T, Noma S, Kim DS, Handa T, Izumi T, Mishima M: Pulmonary manifestations of primary Sjogren’s syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med 2005, 171:632-638.
• [3]Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH: Interstitial lung disease in primary Sjogren syndrome. Chest 2006, 130:1489-1495.
• [4]Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH: Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002, 61:554-558.
• [5]Constantopoulos SH, Papadimitriou CS, Moutsopoulos HM: Respiratory manifestations in primary Sjogren’s syndrome. A clinical, functional, and histologic study. Chest 1985, 88:226-229.
• [6]Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM: Malignant lymphoma in primary Sjogren’s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjogren’s Syndrome. Arthritis Rheum 1999, 42:1765-1772.
• [7]Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, Costa J, Decker JL, Chused TM: Increased risk of lymphoma in sicca syndrome. Ann Intern Med 1978, 89:888-892.
• [8]Freeman C, Berg JW, Cutler SJ: Occurrence and prognosis of extranodal lymphomas. Cancer 1972, 29:252-260.
• [9]Hare SS, Souza CA, Bain G, Seely JM, Frcpc , Gomes MM, Quigley M: The radiological spectrum of pulmonary lymphoproliferative disease. Br J Radiol 2012, 85:848-864.
• [10]Travis WDCT, Koss MN, Rosado-de-Christenson ML, Muller NL, King JTE: Non-neoplastic disorders of the lower respiratory tract. Atlas of nontumor pathology 2002., Volume 2
• [11]Laohaburanakit P, Hardin KA: NK/T cell lymphoma of the lung: a case report and review of literature. Thorax 2006, 61:267-270.
• [12]Hanawa T, Chiba W, Fujimoto T, Wazawa H, Yamashita N, Yasuda Y, Matsubara Y, Hatakenaka R, Funatsu T, Ikeda S: [T cell lymphoma presenting as recurrent bilateral pulmonary infiltrates over five years]. Nihon Kyobu Shikkan Gakkai Zasshi 1996, 34:363-368.
• [13]Choi TS, Doh KS, Kim SH, Jang MS, Suh KS, Kim ST: Clinicopathological and genotypic aspects of anticonvulsant-induced pseudolymphoma syndrome. Br J Dermatol 2003, 148:730-736.