Background:Thrombotic microangiopathy (TMA) is a clinicopathologic diagnosis defined as microangiopathic hemolytic anemia (MAHA) with associated features of thrombocytopenia and end-organ ischemia. Systemic lupus erythematosus, antiphospholipid antibody syndrome, and scleroderma, are within the autoimmune diseases, the more commonly associated with TMA. It has been considered that the association with Sjögren Syndrome (SS) is rare.Objectives:To describe one patient with TMA and SS, and to review all cases reported in the literature.Methods:We notified a clinical case of a patient with Sjögren’s syndrome and TMA. Then, we searched the medical literature finding a total of 17 cases reported with this association until 2019. Before the data obtained were tabulated by trained staff and descriptive, comparing groups and bivariate analysis was performed. The outcome of interest was the death of the patient. Stata 12.0 software was used.Results:A 26-years-old Colombian female presented with a 6-week history of petechiae in lower limbs, gingivorrhagia, menorrhagia and jaundice; and previous history of arthritis and xerostomia. On admission, in the context of severe thrombocytopenia and MAHA, MAT diagnosis was made. During hospitalization we confirm the diagnosis of SS with ANA, antiRo and salivary gland biopsy. The patient was treated with steroids (methylprednisolone 500 mg/day, 3 days), plasma exchange therapy (PLEX) and Cyclophosphamide (750 mg), with recovery of hemoglobin and platelet levels; however, the patient died due to a complication of the PLEX catheter removal procedure.A total of 18 patients diagnosed with de novo or prevalent Sjögren’s syndrome who had hospital admission with a diagnosis of TMA were included. The mean age was 54.55 years (Standard deviation (SD): 12.45) and 83.33% of the patients corresponded to the female gender.At admission, the mean of hemoglobin was 8.45 g/dL (SD: 2.55) and median platelets of 27250/mm3 (interquartile range (IQR) 10500 - 102000) were found. The most frequent clinical manifestations were central nervous system alterations (50%), followed by bleeding in the skin and renal failure (44.44%) and fever (27.78%). The most frequent antibodies found were anti-Ro (100%), anti-nuclear antibodies (80%) and anti-La (75%). The most frequently prescribed treatment was plasma exchange therapy (83.33%), intravenous steroids (61.11%), oral steroids (61.11%) and cyclophosphamide (27.78%). Of the total patients, 38.89% died and 27.7% had some relapse of TMA.In the group comparison analysis, differences were found in intravenous steroid (81.82% in those who lived vs. 28.57% in those who died p=0.039), use of PLEX (100% in those who survived vs. 57.14% in those who died p = 0.043), fever (9.09% in those who survived and 57.14% in those who died, p = 0.047), admission hemoglobin (7.65 g/dL in those who lived vs. 10.22 g/dL in those who died, p = 0.05), final platelets (148,000 in which who lived and 39,000 in those who died p = 0.02). Then, in the logistic regression analysis, an association was found between mortality and use of intravenous steroids (OR: 0.08, 95% CI 0.009 - 0.83, p = 0.35) and fever at admission (OR: 13.33 95% CI: 1.04 - 169.55, p = 0.046).Conclusion:While the association between TMA and SS is uncommon, so far 18 cases have been reported in the world medical literature. It is typically a condition of women age close to 50 years. The most frequent manifestations are neurological. Among the variables evaluated, only the use of endovenous steroids was associated with a decrease in the probability of mortality; on the contrary, the presence of fever at hospital admission increased the probability of death. The results should be evaluated with caution, since, due to the limited availability of information, they may not be generalizable to clinical practice. More information on this should be obtained in the future.Disclosure of Interests:None declared
Plasma exchange therapy for acute necrotizing encephalopathy of childhood
