Teaching File

Institute of Radiology "P. Cignolini" - University of Palermo, Italy

Sicca syndrome

Mario Finazzo, MD
Giuseppe Brancatelli, MD
Tommaso Bartolotta, MD
Gianvincenzo Sparacia, MD

February 27, 1997


Sicca syndrome: MR findings in the parotid gland

Clinical Hystory


A patient had suffered dry eyes, dry mouth and bilateral swelling of the parotid gland for a long time. Saxon's and Schirmer's tests were positive. SS-A and SS-B antibodies were detected. Ultrasonography showed bilateral swelling of the parotid gland. Both the glands were characterized by multiple hypoechoic areas. An MRI was ordered.

Imaging Findings


  • Axial SE T1 image (500/20)
  • Axial Gd-DTPA enhanced SE T1 image
  • Axial SE T2 image (2500/110)

  • Axial SE T1 image shows the swelling of both the glands and the presence of multiple hypointense areas. These areas are hyperintense on axial SE T2 image and do not enhance after intravenous administration of Gd-DTPA.


    Diagnosis

    Sicca syndrome (primary Sjogren syndrome)


    Discussion

    Sjogren syndrome is an autoimmune disease characterized by the triad of xerostomia, keratoconjunctivitis sicca and a systemic autoimmune disease such as rheumatoid arthritis, systemic lupus erithematosus, progressive systemic sclerosis or mixed connective tissue disease.
    The diagnosis of this condition requires the presence of at least two of the three features (1-3).
    Sjogren syndrome takes in two forms: the sicca syndrome (primary form) which includes patients with xerostomia and keratoconjunctivitis sicca without any underlying systemic autoimmune disease and the so called secondary Sjogren syndrome (secondary form) which comprises patients with an underlying systemic autoimmune disease (1).
    There are several methods for the diagnosis of Sjogren syndrome.
    Saxon's and Schirmer's tests are reliable screening methods and the immunoserological tests (rheumatoid factor, SS-A and/or SS-B antibodies) are very helpful, but they do not allow to assess the pathologic changes of the salivary glands (2).
    Histologic examination of the minor salivary gland biopsy specimens is considered the method of choice to confirm the clinical diagnosis of Sjogren syndrome. But it is invasive, painful and has surgical risk. The biopsy results may fail to correlate with clinical and laboratory features. Furthermore the salivary glands abnormalities of elderly people, especially those more than 80 years old, may be similar to those found in the Sjogren syndrome patients (2-3).
    Syalography represents the gold standard in many institutions. However it is invasive, can adversely affect ingestion and taste; furthermore it requires radiation exposure (2).
    MR imaging is noninvasive and requires no radiation. The presence of multiple spots of hypointensity and hyperintensity scattered throughout the parotid gland respectively on SE T1 and T2 images is pathognomonic of Sjogren syndrome, since no parotid disease has got this MR picture (1).
    Some investigators suggested the dilatation of the ductal system is responsible of the hypointense and hyperintese areas on SE T1 and T2 images (1). Our case confirmed this hypothesis, since hypointense spots on T1w images did not enhance after the administration of Gd-DTPA.
    However the role of MR imaging in Sjogren syndrome is still controversial, though this method may be very specific.
    The "salt and pepper" pattern was found in one report in only the 46% of the patients (1). Moreover ultrasonography and CT are as noninvasive as MR imaging and are less expensive.They seems to be better than MR imaging in the diagnosis of inflammatory salivary disease and salivary stones because of their superiority in depicting ductal stones and calcifications within the gland (1).
    On the other hand a recent report asserts that quantitative analysis of MR images can significantly improve sensitivity and specificity in the diagnosis of Sjogren syndrome (3).
    Further investigations are needed to definitely establish the role of MR imaging in Sjogren syndrome.



    References


    1) Takashima S, Takeuchi N, Morimoto S, Tomiyama N, Ikezoe J, Shogen K, Kozuka T, Okumura T. MR imaging of Sjogren syndrome: correlation with syalography and pathology. JCAT 1991; 15 (3):393-400

    2) Vogl TJ, Dresel SHJ, Grevers G, Spath M, Bergman C, Balzer J, Lissner J. Sjogren's syndrome: MR imaging of the parotid gland. Eur Radiol 1996; 6: 46-51

    3) Izumi M, Eguchi K, Ohki M, Uetani M, Hayashi K, Kita M, Nagataki S, Nakamura T. MR imaging of the parotid gland in Sjogren's syndrome: a proposal for new diagnostic criteria. AJR 1996; 166: 1483-1487



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    University of Palermo, Institute of Radiology "P. Cignolini"


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