Localized amyloidosis involving parotid and sub mandibular glands associated to Sjogren syndrome: Rare association

Abstract

Localized amyloidosis involving the major salivary gland is extremely rare. AA amyloidosis was usually shown with systemic diseases such as rheumatoid arthritis, ankylosing spondylitis and others. Its association with Sjogren syndrome was exceptionnal. We present a rare association: localized amyloidosis in major salivary gland co-existing with Sjogren syndrome. It was about a 64-year-old woman with bilateral asymmetric and recurrent swelling of major salivary gland. She suffered from ocular and oral dryness. Physical examination found high blood pressure at 170/90 mm Hg. She had no palpable cervical lymphadenopathy. Specialized ophthalmological examination objectified sicca syndrome. Blood count was normal. Serum level biochemistries was within normal levels. Chest X-ray was normal. Magnetic Resonance Imaging-parotid found parotid and sub-mandibular enlargement gland with no cervical lymph nodes. Salivary biopsy revealed stage IV sialdenitis Chisholm. Anti nuclear antibodies were positive at 1/160. Parotid resection was practiced on February 2016 confirming amyloidosis with inflammatory signs. She received corticosteroids (0.5 mg/kg/day) for 1 month without favorable outcomes. Maxillectomy was performed. Histological findings showed again maxillary AA amyloidosis. Decision was to stop steroids. Localized amyloidosis in head and neck is uncommon. Most of localized amyloidosis affect the skin, kidney, tracheobronchial tract, tongue, breast, salivary gland and vocal cord. Involvement of major salivary gland is very rare. Localized or systemic amyloidosis might coexist with primary SS. Most of localized forms corresponded to AL type, whereas AA amyloidosis was uncommon. Some cases of localized AL amyloidosis have been associated with Sjögren's syndrome in literature. It followed the SS onset in the majority of patients (1–25 years). Diagnosis of amyloidosis was based on histopathological findings. Amyloid deposition in head and neck area can be the first symptom of systemic amyloidosis. Therefore, additional investigations are necessary to exclude systemic involvement. Treatment of choice of localized amyloidosis is local resection of the lesion.