Background Immunoglobulin 4-related Mikulicz'sdisease (IgG4-MD) is a subtype of IgG4-related diseases with the future of swollen bilateral salivary glands and highly responsive to gluccocorticoids (GCs) therapy. It has the similar ultrasonography (US) features as primary sjogren's syndrome (pSS) including multiply hypoechoic zone, hyperechogenic reflections, inhomogeity and echogeneity appearance of the parotid and submandibular glands. Our study tried to distinct IgG4-MD from pSS by US.
Objectives Our study tried to distinct IgG4-MD from pSS by US.
Methods 7 cases of IgG4-MD with parotid involvement and 28 cases of pSS first diagnosed without treatment before were observed by ultrasonography (US) of bilateral parotid and submandibular glands. Parameters includes parotid gland depth, submandibular size were recorded, and the extent of nodal hypoechoic areas, hyperchogenic reflections, inhomogeity, echogeneity, border clearness were scored according to the pSS US scoring system designed by Hocevar. Afterward, 7 IgG4-MD and 28 pSS patients were given 16mg Methylprednisolone and then tapered 4mg per month and followed up by US after 3 months.
Results 1) The parotid gland depth of IgG4-MD were significantly larger than of pSS (38±7mm: 11±5mm, p<0.01).
2) The scores of hypoechoic areas, hyperchogenic reflections, inhomogeity of IgG4-MD cases by Hocevar's method were significantly higher than of pSS cases repectively (mean scores=9.1: 6.3, 11.1: 3.6, 11.8: 5.9, p<0.01), border clearness and echogeneity were not significantly difference.
3) It is shown depth of bilateral parotid glands (38±7mm: 14±3mm) and the score of hypoechoic areas (9.1: 3.7, p<0.01) in IgG4-MD cases remarkably decreased after 3 month follow-up, however, no too much difference of the depth of bilateral parotid glands (11±5mm: 10±6mm) and the score of hypoechoic areas (6.3: 5.3) in pSS cases.
Conclusions Ultrasound could be used for distinguish IgG4-MD salivary involvement from pSS for monitoring the depth of parotid and scoring hypoechoic areas especially during the GCs therapy.
Disclosure of Interest None declared
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