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1 online ML Comm Case Report Korean J Otorhinolaryngol-Head Neck Surg 2015;58(8): / pissn / eissn A Case of Multiple Sialolithiasis in the Parotid Gland with Sjögren s Syndrome Yeong Joon Kim 1, Hyoung Shin Lee 1, Seon Yoon Choi 2, and Sung Won Kim 1 1 Departments of Otolaryngology-Head and Neck Surgery, 2 Internal Medicine, Kosin University College of Medicine, Busan, Korea 쇼그렌증후군과동반된다발성이하선타석증 1 예 김영준 1 이형신 1 최선윤 2 김성원 1 고신대학교의과대학이비인후과학교실, 1 내과학교실 2 Received August 1, 2014 Revised October 23, 2014 Accepted October 28, 2014 Address for correspondence Sung Won Kim, MD Department of Otolaryngology- Head and Neck Surgery, Kosin University College of Medicine, 262 Gamcheon-ro, Seo-gu, Busan , Korea Tel Fax swforyou@gmail.com Multiple calcification in the major salivary glands is very rare. Sjögren s syndrome is characterized by tissue damage due to chronic lymphocyte infiltration of exocrine glands, and the involvement of the major salivary glands is followed by typical symptoms such as multiple formation of sialolith, blockage of salivary duct, and edema of the parenchyme. When multiple calcification is found in the parenchyme of parotid gland on the computed tomography imaging, Sjögren syndrome should be considered, where the primary solution is conservative treatment and preventing recurrent inflammation. Korean J Otorhinolaryngol-Head Neck Surg 2015;58(8): Key WordsZZParotid gland ㆍ Sialolithiasis ㆍ Sjögren s syndrome. 서론 쇼그렌증후군 (Sjögren s syndrome) 은침샘과눈물샘에만성림프구침윤으로인한조직손상을특징으로하는질환으로, 타액선의실질과관내의타석이다발성으로생기는경우가존재한다. 1,2) 쇼그렌증후군으로생기는타석은주로이하선에서생기고, 본증례와같이이하선의실질내에다발성의석회화소견을보이는것이특징이다. 3,4) 쇼그렌증후군에서생기는다발성타석은매우드물기때문에 3,5) 증례와문헌보고를리뷰하여임상적인도움을주고자한다. 증례 64세여자환자가 1년간좌측이하선의반복되는이하선부위의종창과통증을주소로내원하였다. 환자는내원 1년전부터지속적인구강건조및안구건조증상이동반되었다. 이런주증상은주로식사를할때갑자기생기는전형적인폐 쇄성이하선염의소견을보였고, 이하선부위를마사지하면이하선관의개구부에서농성분비물이배액되면서증상이다소개선되었다. 경부전산화단층촬영 (computed tomography, CT) 에서좌측이하선실질의종창과실질내다발성의석회화소견이관찰되었다 (Fig. 1). 쇼그렌증후군등의원인질환을감별하기위해류마티스내과및안과와협진을진행하였다. 혈액검사결과백혈구 (white blood cell) 는 4200/10 3 L로림프구 (lymphocyte) 비율은 52.8% 였다. 항핵항체양성, 항-Ro 항체및항-La 항체강양성이었으며, 적혈구침강반응 (erythrocyte sedimentation rate, ESR) 은 102 mm/hr였다. 타액선신티그래피검사 (salivary scintigraphy) 에서는양측이하선부위의흡수감소가관찰되었다 (Fig. 2). 쉬르머검사 (Schirmer s test) 결과, 좌안, 우안각각 3 mm, 4 mm로분비감소소견을보였다. 쇼그렌증후군의진단기준 6개중 5개에해당하며객관적기준 4개중 3개에충족하여쇼그렌증후군으로진단가능하여조직검사는시행하지않았다 (Table 1 and 2). 1) 치료는수분공급을충분히하면서이하선부위를마사지하 Copyright 2015 Korean Society of Otorhinolaryngology-Head and Neck Surgery 567

Korean J Otorhinolaryngol-Head Neck Surg 2015;58(8):567-71 여타석이잘배출될수있게보존적치료를하였다. 약물치료로는타액분비촉진을위해 pilocarpine을사용하였고, 염증완화를위해스테로이드 (triamcinolone) 를병용하였다.

1년이지난뒤에외래방문하였을때는증상이많이개선되었고 CT에서도타석의개수가많이감소한것을확인할수있었다 (Fig. 3). 1년이지난현재까지호전된상태를유지하고있으며경과관찰중이다. 고 찰 Fig. 1. Axial view of neck CT scan taken when the patient first visited.

2 Korean J Otorhinolaryngol-Head Neck Surg 2015;58(8): 여타석이잘배출될수있게보존적치료를하였다. 약물치료로는타액분비촉진을위해 pilocarpine을사용하였고, 염증완화를위해스테로이드 (triamcinolone) 를병용하였다. Triamcinolone 2 mg 및 pilocarpine 5 mg를하루 2회 15일간복용하였고폐쇄성이하선염이었지만타액배출폐색으로인한악화없이증상호전을보여 triamcinolone 2 mg을하루 1 회로줄이고 pilocarpine 용법을유지하여 2개월사용후중단하였다. 항생제는사용하지않았다. 1년이지난뒤에외래방문하였을때는증상이많이개선되었고 CT에서도타석의개수가많이감소한것을확인할수있었다 (Fig. 3). 1년이지난현재까지호전된상태를유지하고있으며경과관찰중이다. 고 찰 Fig. 1. Axial view of neck CT scan taken when the patient first visited. Multiple calcification was found in the parenchyme of the left parotid gland. 쇼그렌증후군은침샘과눈물샘에만성림프구침윤으로인한조직손상을특징으로하는질환이다. 전형적인초기증상은반복되는구강과안구의건조증인데, 비특이적인염증을유발하고, 6) 타액선의실질과관내의타석이다발성으로생기면서타액선관의폐색과타액선실질의부종과같은증상이먼저생기는경우도있다. 1,2) 일반적인타석은주로악하 Fig. 2. Salivary scintigraphy result shows reduced uptake in both parotid and submandibular salivary glands, suggesting decreased function. 568

3 A Case of Multiple Sialolithiasis in the Parotid Gland with Sjögren s Syndrome Kim YJ, et al. Table 1. Revised international classification criteria for Sjögren s syndrome Symptoms Table 2. Revised rules for classification of Sjögren s syndrome Primary SS Secondary SS Exclusion criteria Criterias I. Ocular symptoms At least one of: - Daily, persistent, troublesome dry eyes for more than 3 months - Recurrent sensation of sand or gravel in the eyes - Usage of tear substitutes more than 3 times a day II. Oral symptoms Objective criteria items III. Ocular signs IV. Histopathology SS: Sjögren s Syndrome At least one of: - Daily feeling of dry mouth for more than 3 months - Recurrently or persistently swollen salivary glands as an adult - Frequently drink liquids to aid in swallowing dry food At least one of: 1. Schirmer s I test, performed without anaesthesia (<5 mm in 5 minutes) 2. Rose bengal score or other ocular dye score (>4 according to van Bijsterveld s scoring system) In minor salivary glands (obtained through normal-appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score >1, defined as a number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm 2 of glandular tissue V. Sailvary gland involvement At least one of: 1. Unstimulated whole salivary flow (<1.5 ml in 15 minutes) 2. Parotid sialography showing the presence of diffuse sialectasias (punctate, cavitary or destructive pattern), without evidence of obstruction in the major ducts 3. Salivary scintigraphy showing delayed uptake, reduced concentration and/or delayed excretion of tracer VI. Autoantibodies In the serum of the following autoantibodies: - Antibodies to Ro (SSA) or La (SSB) antigens, or both In patients without any potentially associated disease, primary SS may be defined as follows: a) The presence of any 4 of the 6 items is indicative of primary SS, as long as either item IV (histopathology) or VI (serology) is positive b) The presence of any 3 of the 4 objective criteria items (that is, items III, IV, V, VI) c) The classification tree procedure represents a valid alternative method for classification, although it should be more properly used in clinical-epidemiological survey In patients with a potentially associated disease (for instance, another well defined connective tissue disease), the presence of item I or item II plus any 2 from among items III, IV, and V may be considered as indicative of secondary SS Past head and neck radiation treatment Hepatitis C infection Acquired immunodeficiency disease (AIDS) Pre-existing lymphoma Sarcoidosis Graft versus host disease Use of anticholinergic drugs (since a time shorter than 4-fold the half life of the drug) 선에서생기지만, 쇼그렌증후군과관련된타석은주로이하선에서생기며원인은알려져있지않다. 6) 중증의쇼그렌증후군에서는타석이본증례와같이다발성으로생기는것이특징이다. 3) 또한이전에타액선염에대한병력이없었고, 반복되는타액선염으로인한결석이없었던과거력도자세한문진을통해배제해야한다. 4) 본증례에서는약 1년전부터지속적인구강건조및안구건조증상이있었으며, 동위원소검사, 자가항체검사및쉬르머검사를통해서쇼그렌증후군으로진단되었으며, 반복적인이하선의염증소견및이하 선의다발성타석을발견할수있었다. 쇼그렌증후군에서생기는타석에대한유병률을조사한문헌보고는없지만, 이하선에서다발성타석에대한보고는매우드물다. 3,5) 또한일반적으로타석이생기는기전은타액의흐름이줄어들고, 타액에칼슘과염분이고농도로있으면서유기적인기질성분과혼합되어생성된다고알려져있지만, 7) 쇼그렌증후군에서타석이다발성으로생기는이유가명확하게설명된보고는없다. 6) 외분비선에서조직검사를해보면특징적인분비샘의림프구 569

Korean J Otorhinolaryngol-Head Neck Surg 2015;58(8):567-71 Fig. 3. Axial view of neck CT scan 1 year after the first visit showed decreased number of the sialoliths in the parotid glands.

4 Korean J Otorhinolaryngol-Head Neck Surg 2015;58(8): Fig. 3. Axial view of neck CT scan 1 year after the first visit showed decreased number of the sialoliths in the parotid glands. 침윤이있으며, 혈청감마글로불린증가, 적혈구침강률 (ESR) 증 가, SS- 항원 A 와 B 에대한항체 (anti-ss antigen A and B), 항핵항체 (antinuclear antibody), 류마티스인자 (rheumatoid factor) 등이양성이다. 1) 쇼그렌증후군에서드물지만이하선 종양으로발생하는점막관련림프조직종양 [mucosa-assisted lymphoid tissue(malt) lymphoma] 이생길가능성이증가 되기때문에주의하여관찰하여야한다. 8) CT 검사를하면다발성으로석회화된타석과급성이하선 염을쉽게확인할수있고, MALT 림프종이나가성종양이있 는지도감별할수있어서유용하다고알려져있으며, 6) 본증 례에서도초음파및 CT 를통해종양은배제할수있었으며, 다발성타석을확인할수있었다. 침샘염의급성기에는검, 사탕등의타액분비촉진제 (sialogogues) 가도움이되고, 수분공급, 온열압박마사지가도움이 된다. 특히이하선을잘촉지하고, 마사지를잘해서타석이 이하선관개구부를통해구강으로빠져나오는것을확인하 여증상이빨리개선되었다는보고가있다. 6) 본케이스의경 우마사지를교육하여시행하였지만타석이나오지는않았 다. 약물치료는급성기에는항생제, 항염증제, 진통제등을 사용하고, 장기적으로는쇼그렌증후군에대한치료를하여 야한다. 6) 대증요법에대한치료가효과가없다면, 수술적치료를하 기도한다. 최근에는수술적치료로침샘관내시경이나침샘 조영술을하면서바스켓 (basket) 을이용해타석을제거하는 방법이소개되고있는데, 9,10) 결석이이하선관이나문 (hilum) 근처에있는경우라면결석을제거하는데유용하지만, 결석이 이하선의실질에있는경우에는적용되기어렵고, 결절의크기 가 5 mm 이상이면이하선관을통해결석을제거하기어렵다 는제한이있다. 또다른방법은초음파를하면서충격파쇄 석술을하는것인데, 11) 결석의위치, 크기, 개수에상관없이유 용하게사용될수있고, 반복시술이가능하다는것도장점이 다. 하지만, 충격파쇄석술을하면증상이완화되는경우는 80% 정도이지만, 결석이제거되는경우는 50% 정도로낮다 는점도고려하여야하고, 충격파가이하선의실질에손상을 주어섬유화를유발할수있다는단점이있다. 12) 그리고, 고실 신경절단술등을하기도한다. 13) 침샘을제거하는수술이근 본적인치료이긴하지만, 중요한신경손상등의침샘제거수 술로인한심각한합병증이있을수있으므로마지막에선택 되는술식이며일측의이하선의결석이 3 개이상있고, 반복되 는이하선염의병력과침샘관내시경이나충격파쇄석술로치 료되지않는경우에고려해볼수있다. 12) 동물모델에서고칼 슘혈증이타석생성의원인일수있다는보고도있지만, 14) 인 간에서는증명된바가없어서예방적으로칼슘이많이함유 된음식을주의할필요는없다. 6) 본증례와같이이하선종창이있고, CT 검사에서다발성 석회화소견이관찰되면, 쇼그렌증후군을감별진단해야한 다. 그리고, 수술적치료보다는대증적인치료와반복되는염 증을예방해주는것이필요하다. REFERENCES 1) Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren s syndrome: a revised version of the European criteria proposed by the American- European Consensus Group. Ann Rheum Dis 2002;61(6): ) Nahlieli O, London D, Zagury A, Eliav E. Combined approach to impacted parotid stones. J Oral Maxillofac Surg 2002;60(12): ) Shimizu M, Yoshiura K, Nakayama E, Kanda S, Nakamura S, Ohyama Y, et al. Multiple sialolithiasis in the parotid gland with Sjögren s syndrome and its sonographic findings--report of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99(1): ) Zenk J, Gottwald F, Bozzato A, Iro H. [Submandibular sialoliths. Stone removal with organ preservation]. HNO 2005;53(3): ) Wickramasinghe A, Howarth A, Drage NA. Multiple bilateral parotid sialoliths in a patient with mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) of the salivary glands. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99(4): ) Konstantinidis I, Paschaloudi S, Triaridis S, Fyrmpas G, Sechlidis S, Constantinidis J. Bilateral multiple sialolithiasis of the parotid gland in a patient with Sjögren s syndrome. Acta Otorhinolaryngol Ital 2007;27(1): ) Boynton TT, Lieblich SE. Unusual case of a sialolith: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol 2014;117(1):e ) Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren s Syndrome. Arthritis Rheum 1999;42(8): ) Drage NA, Brown JE, Escudier MP, McGurk M. Interventional 570

5 A Case of Multiple Sialolithiasis in the Parotid Gland with Sjögren s Syndrome Kim YJ, et al. radiology in the removal of salivary calculi. Radiology 2000;214 (1): ) Durr ML, George JR, Meyer AK, Rosbe KW. Sialendoscopy for recurrent parotitis associated with Sjogren syndrome: a case report. Int J Pediatr Otorhinolaryngol Extra 2011;6(4): ) Ottaviani F, Capaccio P, Campi M, Ottaviani A. Extracorporeal electromagnetic shock-wave lithotripsy for salivary gland stones. Laryngoscope 1996;106(6): ) Iro H, Schneider HT, Födra C, Waitz G, Nitsche N, Heinritz HH, et al. Shockwave lithotripsy of salivary duct stones. Lancet 1992;339 (8805): ) Hackett AM, Baranano CF, Reed M, Duvvuri U, Smith RJ, Mehta D. Sialoendoscopy for the treatment of pediatric salivary gland disorders. Arch Otolaryngol Head Neck Surg 2012;138(10): ) Westhofen M, Schäfer H, Seifert G. Calcium redistribution, calcification and stone formation in the parotid gland during experimental stimulation and hypercalcaemia. Cytochemical and X-ray microanalytical investigations. Virchows Arch A Pathol Anat Histopathol 1984;402 (4):

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ÀÌ¾Æ¸§ 155 Table 1. CT Findings and Glandular Involvements of Acute Sialadenitis in 50 Child-Adolescent I (n=16) II (n=20) III (n=14) p value Abscess 8 (50.0%) 0 0