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    • glycosylase br Case presentation A year

    2018-11-03


    Case presentation A 25-year-old female visited our outpatient clinic in April 2013 with badly decayed teeth. Careful intraoral and extraoral examinations revealed xerostomia, candidal infection on her tongue, difficulty in swallowing and parotidomegaly (Fig. 1). Familial sociological profile of the patient included preference of consanguineous marriage. No history of drinking or smoking was reported. The ultrasound study showed a bilateral heterogeneous echopattern of the major salivary glands. The glandular parenchyma was atrophic with diffuse cystic cavities (diameter of the largest cyst was less than 0.9 cm) Miliary sporadic calcifications were conspicuous. The overall picturesque was giving the impression of the so-called “cribriform” appearance. (Fig. 2). Cervical glycosylase nodes maintained their normal oval shape, yet mildly enlarged, and their normally appearing hilum. Both thyroid lobes and isthmus displayed a normal echopattern and texture. MRI on the head and neck was run annually in order to assess the status of the salivary glands and cervical lymph nodes (Fig. 3). Serological results revealed an elevated ESR, positive ANA, strongly positive anti-Sjögren\'s syndrome-A (SS-A) and positive anti–Sjögren\'s syndrome-B (SS-B). The rheumatoid factor was, however, negative. Lymphocytopenia, absolute neutropenia, hypochromic microcytic anemic and thrombocytopenia were evident in her deferential CBC. Under local anesthesia, four minor salivary glands from a normally appearing labial mucosa were harvested. The extract was immersed immediately in 10% formalin to be submitted for microscopic examination. The histologic picture viewed a confluence of lymphocytic infiltrate and a few macrophages which replaced most of the glandular parenchyma (greater than fifty lymphocytes in several foci). Acinar degeneration and sporadic epimyoepithelial islands were also observed. No germinal centers were observed (Fig. 4). All in all, a diagnosis of Sjögren\'s syndrome was established. The patient was referred to a rheumatologist and an ophthalmologist for managing her condition but multidisciplinary follow-up was highly recommended. A low-dose corticosteroid was prescribed by her rheumatologit (20- 30 mg of daily Hostacortin). In July 2016, a minor salivary gland was obtained, under local anesthesia, from a normally appearing buccal mucosa and was immersed immediately in 10% formalin to be submitted for microscopic examination. The histologic picture viewed a higher intensity of inflammatory lymphocytic infiltrate which effaced totally architecture of the glandular structures except for maintaining few epimyoepithelial islands (Figs. 5–7). However, the sonographic and MRI pictures demonstrated no signs of malignancy. Running immunohistochemical confirmatory tests, the minor salivary gland specimen was also stained for CD20, C30, BCL6, MUM1 and Pan cytokeratin to exclude any malignant transformation into lymphoma or squamous cell carcinoma. All tests came back negative except for non-specific immunopositivity of epimyoepithelial islands for Pan cytokeratin. The patient was educated about the possibility of evolving low-grade lymphoma ex-SS and was advised to get her minor salivary glands re-biopsied in February 2018. She was also assured about the conservatively successful management of the evolving MALT lymphomas ex-SS if the close follow-up program was continued. The patient was referred back to her rheumatologist for managing the progressive case. Recuriting a therapeutic line, other than corticosterid intake, was recommended because Hostacortin did not prove effective in alleviating the severity of inflammation in the minor salivary glands.
    Discussion Although the triad of xerostomia, xerophthalmia and rheumatoid arthritis was academically considered cardinal signs of SS, this concept has been jettisoned. Furthermore, dozens of other signs and symptoms were included [2–4]. On the one hand, general symptoms comprise fatigue, arthralgia/arthritis, myalgia/myositis, and lymphadenopathy. Internal organ changes include neuropathy, thyroiditis, interstitial lung disease, chronic atrophic gastritis, celiac disease, primary biliary cirrhosis and other liver manifestations, vasculitis, glomerulonephritis, hearing troubles, vaginal dryness, dyspareunia, and interstitial cystitis. Complicating matters, SS is frequently associated with other connective tissue diseases. Oral manifestations, on the other hand, include xerostomia, high caries index, periodontal disease, early tooth loss, gnathic pain, osteomyelitis, difficulty in swallowing and chewing, chronic erythematous mucosa, candidiasis, stomatopyrosis, halitosis, dysgeusia, crusting/numbness of lips, parotidomegaly as well as angular cheilitis [3].