A systematic research of articles published in PubMed/MEDLINE, Web of Science, LILACS, and SciELO, registered from 1966 to August 2020, was performed. All the researched articles are based on the following DESH terms "Sjögren's syndrome" and "thrombotic thrombocytopenic purpura” without language limitation. Furthermore, a detailed case report on a patient with TTP subsequently diagnosed with SS is herein reported. The following parameters were screened for the published cases of SS and TTP association: demographic characteristics (gender, age), clinical features (clinical presentation of TTP, SS, and SS-related antibodies detection, the onset of symptoms and progression), therapy provided, and the response to this therapy. This article followed the PRISMA guidelines . Informed consent was obtained from the patient.
A 30-year-old Caucasian and black mixed ethnicity female patient with a past medical history of convulsion since in her childhood has been treated until now with carbamazepine. She was admitted in April of 2014 with petechiae over her legs and dark urine, and thrombocytopenia (platelets 5000/mm3). The patient received methylprednisolone pulse therapy 1 g daily for 3 days, and the platelets increased to 20,000/mm3. During the hospital stay, she had three absence episodes and severe hypotension and was transferred to the intensive care unit (ICU). In that place, severe hemolytic anemia (hemoglobin 5 g/dl), thrombocytopenia, renal impairment, and oscillations of conscience level were verified with schizocytes. A diagnosis of thrombotic thrombocytopenic purpura was determined, and plasmapheresis was started. She received 15 sessions of plasmapheresis, and 1 month later, rituximab was infused. After this approach, the TTP improved, and the patient recovered from this condition. Due to antinuclear antibody positivity with a titer of 1:640, a diagnosis of lupus was considered, and hydroxychloroquine 400 mg/day was prescribed. However, she did not have any evidence of skin lesions, serositis, glomerulonephritis, or polyarthritis. She has a cousin with lupus and an aunt with rheumatoid arthritis. After discharge, she went to our private clinic, and her physical examination was unremarkable. No sign of carbamazepine intoxication was found, such as gingival hypertrophy. Laboratory tests demonstrated positive antinuclear antibodies with a titer of 1:640, and a speckled pattern on indirect immunofluorescence in HEp2 cells. Anti-Ro/SS-A, anti-La/SS-B, anti-RNP, anti-Sm, anti-dsDNA, lupus anticoagulant, IgG, IgM, and IgA anticardiolipin, anti-thyroperoxidase, ANCA, anti-thyroglobulin; IgA and IgG anti-gliadin, anti-endomysium and anti-tissue transglutaminase antibodies and cryoglobulins were not detected. Complement levels were within normal range (CH50 97 U/ml, C3 150 mg/dl, and C4 30 mg/dl). Blood cell count, creatinine, and urine spot were normal. Tests for thrombophilia were all negative or within normal range: factor V Leiden, mutant prothrombin, antithrombin III, proteins C and S, and homocysteine. Serology for infectious diseases was negative: HIV 1 and 2, HTLV I and II, syphilis, cytomegalovirus, and hepatitis B and C viruses. Hemolysis test was negative at that moment, with a negative Coombs test, haptoglobin 240 mg/dl (25–190 mg/dl), normal bilirubin, and negative for schistocytes. ADAMTS 13 activity was less than 2% (> 50%) and ADAMTS13 antigen was 0.1 (0.6–1.6). She complained of xerostomia and xerophthalmia. Salivary gland scintigraphy showed a decrease in salivary production bilaterally on submandibular and parotid glands. Schirmer test was < 5 mm in 5 min in both eyes, and Bengal rose tests were positive with a score > 5. A minor salivary gland biopsy was positive with focal lymphocytic sialadenitis and a focus score of 1 foci/4 mm. A diagnosis of primary Sjögren's syndrome was then determined. Hydroxychloroquine was kept, and ocular lubricants were added. She is currently asymptomatic; under HCQ and lubricants, routine laboratory tests are within the normal range.