1 Ann Rheum Dis Month 2019 Vol 0 No 0 Could we use salivary gland ultrasonography as a prognostic marker in Sjogren’s syndrome? Response to: ‘Ultrasonographic damages of major salivary glands are associated with cryoglobulinemic vasculitis and lymphoma in primary Sjogren’s syndrome: are the ultrasonographic features of the salivary glands new prognostic markers in Sjogren's syndrome?’ by Coiffier et al We thank Dr Coiffier and colleagues for their interesting letter in which they suggest that damages of major salivary glands (SG) might be associated with cryoglobulinemic vasculitis and lymphoma in primary Sjögren’s syndrome. The authors raise the question whether the ultrasonographic (US) features of the salivary glands [our proposed novel ultrasound scoring system] could be used as the new prognostic markers for cryoglob- ulinemic vasculitis and lymphoma in patients with Sjögren’s syndrome. 1 In their recent study of different US scoring systems in 97 sicca syndrome patients, Coiffier et al found three B-cell lymphomas in the primary Sjogren’s syndrome (pSS) group and three, two, and one patient with cryoglobulinemic vascu- litis in the pSS, secondary Sjogren’s syndrome (sSS) and control groups, respectively. 2 The authors concluded that the detection of B-cell lymphomas or cryoglobulinemic vasculitis was associated with pathological US findings regardless of the scoring used. The reported US pathological features of SG were either numerous cystic lesions without healthy paren- chyma or fibrous glands scored as a grade 3 according to the new semi quantitative scoring system described by the SG sub group of the OMERACT US working group. 1 Although their findings may suggest specific ultrasound features as a risk factor for developing lymphoma or cryoglobulinemic vascu- litis, we think at this point in time it is premature to draw such a conclusion. 3 Ultrasound may reveal predisposing factors, but these are not proven to be pathognomonic of lymphoma. Indeed, several predictors of lymphoma in pSS such as epide- miological, clinical (permanent swelling of the SG, palpable purpura, organomegaly), biological (cryoglobulinaemia, or low complement levels) and histopathological findings should also be taken into account. 4–9 Large sample and longitudinal studies assessing these clinical and biological predictors of lymphoma with US are currently ongoing and will probably shed more light on this challenging issue. Furthermore, pSS disease activity, for example, assessed by the EULAR SS index, can be used as a clinical predictor of lymphoma development with a dose effect. 10 SG enlargement (eg, clinical aspects: unilateral, fixed and hard parotid glands) is regarded as the most dominant clinical symptom for lymphoma in patients with pSS. 6 SGUS performed by well-trained ultrasonographers can provide a precise struc- tural assessment of the glands’ surface compared with clin- ical examination. 11 In case of SGUS grade 3, that is, complete destruction of the gland, with numerous hypo-echoic or hyper- echoic bands, the detection of abnormal lymph nodes should raise awareness of possible lymphoma development. Suspicion of abnormal lymph nodes can be confirmed during a long term monitoring of pSS patients especially those with high risk of lymphoma development. In addition, Doppler assessment of gland’s vascularisation in pSS might be of help to detect at risk ultrasound lesions. To this end, the forthcoming results of a longitudinal study for development of consensual Doppler US scoring of gland’s vascularisation in pSS can be quite helpful. Sandrine Jousse-Joulin , 1 Maria Antonietta D'Agostino, 2 Alojzija Hočevar , 3 Esperanza Naredo, 4 Lene Terslev, 5 Sarah Ohrndorf, 6 Annamaria Iagnocco, 7 Wolfgang A Schmidt, 8 Stephanie Finzel, 9 Zarrin Alavi, 10 George A W Bruyn, 11,12 on behalf the Salivary Gland subgroup of the OMERACT US working group 1 Rheumatology, Brest University Hospital, Brest, France 2 Rheumatology Department, INSERM U1173, Laboratoire d’Excellenc, Paris, France 3 Rheumatology, Universitiy Medical Centre Ljubljana, Ljubljana, Slovenia 4 Rheumatology, Severo Ochoa Hospital, Madrid, Spain 5 Rheumatology, Glostrup University Hospital, Copenhagen, Denmark 6 Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany 7 Scienze Cliniche e Biologiche, Università degli Studi di Torino, Rome, Italy 8 Rheumatology, Medical Centre for Rheumatology Berlin Buch, Berlin, Germany 9 Rheumatology and Clinical Immunology, University Medical Center Freiburg, Freiburg, Germany 10 INSERM, CIC 1412, Brest University Hospital, Brest, France 11 MC Group Hospitals, Lelystad, The Netherlands 12 Reumakliniek Flevoland, Lelystad, The Netherlands Correspondence to Dr Sandrine Jousse-Joulin, Rheumatology Department, Cavale Blanche Hospital and Brest Occidentale University, Brest EA 2216, ERI 29, France; sandrine.jousse-joulin@chu-brest.fr Handling editor Josef S Smolen Contributors All co-authors participated in the writing and reading of this correspondence letter. Funding The authors have not declared a specifc grant for this research from any funding agency in the public, commercial or not-for-proft sectors. Competing interests None declared. Patient consent for publication Not required. Provenance and peer review Commissioned; internally peer reviewed. © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ. To cite Jousse-Joulin S, D’Agostino MA, Hočevar A, et al. Ann Rheum Dis Epub ahead of print: [please include Day Month Year]. doi:10.1136/ annrheumdis-2019-216327 Received 16 September 2019 Accepted 18 September 2019 ► http://dx.doi.org/10.1136/annrheumdis-2019-216122 Ann Rheum Dis 2019;0:1–2. doi:10.1136/annrheumdis-2019-216327 ORCID iDs Sandrine Jousse-Joulin http://orcid.org/0000-0002-5479-5887 Alojzija Hočevar http://orcid.org/0000-0002-7361-6549 REFERENCES 1 Jousse-Joulin S, D’Agostino MA, Nicolas C, et al. Video clip assessment of a salivary gland ultrasound scoring system in Sjögren’s syndrome using consensual defnitions: an OMERACT ultrasound Working group reliability exercise. Ann Rheum Dis 2019;78:967–73. 2 Coiffer G, Martel A, Albert J-D, et al. Ultrasonographic damages of major salivary glands are associated with cryoglobulinemic vasculitis and lymphoma in primary Sjogren’s syndrome: are the ultrasonographic features of the salivary glands new prognostic markers in Sjogren’s syndrome? Ann Rheum Dis 2019. doi:10.1136/ annrheumdis-2019-216122. [Epub ahead of print: 16 Aug 2019]. 3 Theander E, Vasaitis L, Baecklund E, et al. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjogren’s syndrome. Ann Rheum Dis 2011;70:1363–8. Correspondence response on June 12, 2020 by guest. Protected by copyright. http://ard.bmj.com/ Ann Rheum Dis: first published as 10.1136/annrheumdis-2019-216327 on 10 October 2019. 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