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Thrombotic microangiopathy associated to Sjogren’s Syndrome: case report and literature review

Arij Ezzouhour Yahyaoui*, Sameh Sayhi, Bilel Arfaoui, Nour Elhouda Guediche, Faida Ajili, Nadia Ben Abdelhafidh

Introduction: Sjogren’s syndrome (SS) is an autoimmune disease characterized by ocular and oral dryness and a variety of other systemic manifestations. Thrombotic Microangiopathy (TMA) is rarely associated with SS. We report a case that illustrates this association in a female patient, who also presents autoimmune hypophysitis.
Observation: A 65-year-old patient was referred for investigation of an axonal sensorimotor neuropathy that has been evolving for 5 months. Possible infectious and neoplastic causes were ruled out by examination, laboratory tests and imaging. The patient reported ocular and oral dryness. Minor salivary gland biopsy highlighted the presence of focal lymphocytic sialadenitis with a focus score of 1 foci/4 mm2. Schirmer’s test was inferior to 5 mm/5 min in both eyes. Laboratory investigations revealed anterior pituitary failure including secondary adrenal insufficiency, central hypothyroidism and hypogonadotropic hypogonadism. Magnetic resonance imaging of the hypothalamo-pituitary region demonstrated signs of autoimmune hypophysitis. During hospitalization, the patient developed
anemia and thrombocytopenia with schizocytes count at 4%. The diagnosis of TMA associated to SS, without renal failure was made. The patient was commenced on intravenous immunoglobulin, corticosteroids, azathioprine and pregabalin. An improvement was noticed in her neuropathic pain, anemia and thrombocytopenia, schizocytes count was reduced progressively.
Conclusion: Previous systematic reviews highlighted the rarity of SS associated with TMA. Our case also illustrates two manifestations of SS: Axonal neuropathy and autoimmune hypophysitis.