We herein report two cases of thrombotic thrombocytopenic purpura (TTP) complicated by other autoimmune disorders, autoimmune hepatitis and immune thrombocytopenia, respectively. In both cases, corticosteroids were continuously administered for the treatment of preceding autoimmune disorders. However, a sufficient objective response for TTP was not obtained by plasma exchange and corticosteroid treatment. Once a week rituximab (375 mg/m 2 ) treatment for 4 times was initiated within 2 weeks from the diagnosis. Both patients achieved a sufficient response, and have never had any recurrence as of the last follow-up dates. The early introduction of rituximab could be an effective treatment option in TTP patients complicated with other autoimmune disorders.

A 78-year-old Japanese woman with Sjögren's syndrome complicating immune-mediated aplastic anaemia is described. A diagnosis of aplastic anaemia was made from severe pancytopenia with hypoplastic marrow. Laboratory studies suggested an association of bone marrow suppressive T-lymphocytes with the pathogenesis of aplastic anaemia. Following the administration of mepithiostan and prednisolone, pancytopenia improved gradually. Two years after the onset of aplastic anaemia, Raynaud's phenomenon developed and examinations revealed the existence of keratoconjunctivitis sicca and anti-SSA/Ro and anti-SSB/La antibodies.

Background Esophageal achalasia is a rare disease characterized by degeneration of Auerbach's plexus. Although its presumed etiologies include autoimmune process, its precise mechanisms remain uncertain. Recently, it has reported that patients with achalasia are more likely to have autoimmune diseases. Objectives To elucidate the significance of achalasia in patients with autoimmune diseases Methods The computerized medical records system of our medical center were screened for patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM)/dermatomyositis (DM), and achalasia for recent 30 years (between 1982 and 2012). Then the details of picked up patients were investigated. Results Twelve patients with achalasia were identified. Among them, 1 patient was also with SLE and 2 patients with SSc (table). All of them experienced nocturnal coughing with regurgitation of ingested food without gastric acid and suffered from aspiration pneumonia. Interestingly, all these 3 patients had autoantibodies relating to SSc (scl-70 or centromere), although patient 1 had no sign indicating SSc. The prevalence of such autoimmune diseases in achalasia patients was 25% (3/12). Inversely, the prevalence of achalasia in SLE patients was estimated at 0.19% (1/539), while that in SSc patients 0.67% (2/297). These patients are 19 times and 67 times more likely to suffer from achalasia compared with general population (1/10000), respectively. None of the 98 patients with PM and of 55 patients with DM had achalasia. The onset of achalasia could be before, at the same time as, or after that of autoimmune diseases. Conclusions The prevalence of achalasia among patients with autoimmune diseases (especially SSc) is much higher than that in general population. When patients with autoimmune diseases present recurrent regurgitation of food without gastric acid, esophageal achalasia must be ruled out, considering it might be underdiagnosed. Patients with SSc can exhibit similar symptoms, having reflux esophagitis.However, their regurgitations are usually with gastric acid. This is an important point for differential diagnosis. Further study is required to reveal its nature and clinical significance. References Booy JD. Et al. Dis Esophagus. 2012;25:209-13. Katada N. et al. Ann Thorac Cardiovasc Surg 2012; 18: 420–428. Gockel I. et al. Dtsch Arztebl Int. 2012;109:209-14. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4910

Background: The number of solid organ transplantation (SOT) is increasing. While the remarkable improvement in SOT procedures provides recipients with a good chance of long-term survival, the prolonged immunosuppression has led toa variety of malignancies in solid organ transplant (SOT) recipients; post-transplant lymphoproliferative disorder (PTLD) is a life-threating complication among them. However, there remain some controversies regarding its clinical features. Method: We retrospectively surveyed the patients who had visited both departments: hematology and urology, or renal surgery in kidney center at Tokyo Women's Medical University Hospital from 2003 to 2017. We identified 61 patients diagnosed with hematological disorder in kidney-transplant recipients. Among them, PTLD were analyzed. Continuous variables were compared using Student's t-tests. Overall survival rate (OS) was calculated using the Kaplan-Meier method. P values < 0.05 were considered significant. Statistical analyses were performed using JMP Pro 14 (SAS Institute Inc., Cary, NC, USA) and GraphPadPrism version 7.0 (GraphPad Software, San Diego, USA). This study was conducted in accordance with the Declaration of Helsinki and approved by the institutional review boards. Results: During the survey period, 3,133 patients had visited both departments: hematology and urology, or surgery in Kidney Center. Among them, 61 kidney-transplant recipients were diagnosed with hematological disorder; they comprised 27 PTLD, 10 plasmacytic disorder, 6 idiopathic thrombocytopenic purpura, 5 myelodysplastic syndromes, 4 acute leukemia, 3 polycythemia, 6 other diseases. Among the patients with PTLD (n = 27), 18 patients (66.7%) were male.Median patient ages at the diagnosis of PTLD and kidney transplant were 54 (25-79) and 43 (16-75) years, respectively. Eighteen and nine patients had received kidney-transplant from a living donor (15 related and three unrelated donors) and a cadaveric donor, respectively. The median time from kidney transplant to diagnosis of PTLD was 7.8 years; there was a significant difference in the time between the kidney-transplant recipients from a cadaveric donor and those from a living donor (Figure 1A, 11.3 and 5.6 years, P = 0.02). With regard to the histological subtypes of PTLD, they comprised 17 diffuse large B-cell lymphoma (63.0%), 2 Burkitt lymphoma (7.4%), 4 unclassified B-cell lymphoma (14.8%), 2 T-cell lymphoma (7.4%), and 2 unknown cases (7.4%). 24 of 27 patients had extranodal involvement: 9 central nervous system (33.3%), 7 gastrointestinal tract (25.9%) and other sites. First-line treatment procedure which all patients received was the modification of immunosupressants; complete response (CR) rate was 37.0%. After the failure of first-line therapy, 15 of 27 patients received chemotherapy: 4 rituximab monotherapy (30.8%), 6 CHOP-like regimens (46.2%), and other regimens (33.3%). Among them, five patients experienced relapse. They received salvage chemotherapy, and 2 and one patients achieved CR and partial response, respectively. Rest of them died of lymphoma and infection. The estimated five-year OS from the diagnosis of PTLD and the initiation of chemotherapy was 88.7% and 86.7%, respectively. During the survey period, 5 patients died; their causes of death were 3 lymphomas, 1 infection, and 1 heart failure. There were no deaths in kidney-transplant recipients from a cadaveric donor (Figure 1B). Discussion: While our cohort had more extranodal PTLD recipients than those reported previously, the modification of immunosupressants improved them in more than one-third of cases, which showed the usefulness of it as first-line treatment procedure for PTLD. Nearly half of cases were identified in recipients from a cadaveric donor; the results suggest that the incidence o PTLD among them is higher than those from a living donor because the number of kidney-transplant from a living donor was 10 times higher than those from a cadaveric donor. This study has several limitations due to its retrospective nature and small sample size. In addition, the estimated five-year OS might be better than actual because some patients became lost to follow-up. Further large-scale studies are warranted to validate the findings of this study. Disclosures Hagiwara: Bristol Myers Squibb: Membership on an entity's Board of Directors or advisory committees, Research Funding; Celgene: Membership on an entity's Board of Directors or advisory committees. Tanaka:Bristol-Myers Squibb: Research Funding.