Gougerot Sjögren Syndrome and Medular Aplasia: A Case Report and Review of the Literature

Abstract

Introduction: Gougerot-Sjögren's syndrome (GSS) is a non-organ specific auto-immune epithelitis that affects essentially exocrine glands characterized by an inflammation of the epithelium, in particular salivary and lacrimal epithelium, leading to oral (xerostomia) and ocular (xerophthalmia) dryness, with the possibility of extra-glandular involvement, which reflects its systemic nature. Its prevalence is estimated between 0.01 and 0.09%. Its association with medullary aplasia (MA) is rare; the latter is of various origins, in particular drug-related, linked to methotrexate. Objective: To present an observation that seems to us remarkable by the exceptional association of primary GougerotSjögren's syndrome (PGS) and bone marrow aplasia in an octogenarian woman (80 years old). Observation: This was an octogenarian woman followed in rheumatology since 1993 for a pGSS based on joint involvement made of persistent bilateral asymmetric dry polyarthritis without deformity associated with ocular and oral dryness, accessory salivary gland biopsy (ASGB) at Chisholm and Masson grade 4 by ACR/EULAR 2016 7-point classification criteria, she was on 15mg methotrexate/semaine, 400mg hydroxychloroquine/day for 23years. She presented at entry with fever (T° 39.5°C), the pallor of integuments and conjunctivae, ecchymosis versus hepatomegaly, splenomegaly and bone pain were absent. Biology finds: Pancytopenia made of aregenerative anemia (Hemoglobin at 7.2g/dl, reticulocyte rate at 5934/mm3), leukopenia at 270/mm3, neutropenia at 70/mm3, lymphopenia at 150/mm3 and Thrombopenia at 9000/mm3. The medullogram concluded that the patient was suffering from bone marrow aplasia with poor cellularity, even deserted, but the renal, hepatic, cardiac and infectious investigations were normal. Thus, bone marrow aplasia in a patient followed for primary GSS under methotrexate for 23 years was retained. The treatment was hospitalization, transfusion of 1200ml of platelet concentrate, tramadol 50mg x 3/d in mini-infusion, Fleming (amoxicillin + clavulanic acid) 1g x2/d and Rovamycin 3 million x 2/d, unfortunately, the continuation was marked by her death at D4 of hospitalization. Conclusion: Medullar aplasia and pGSS are an exceptional association in elderly subjects requiring early diagnosis and management to improve the vital prognosis.