Acquired haemophilia A (AHA) is a rare autoimmune disorder caused by an autoantibody against any circulating coagulation factor, especially factor VIII (FVIII). The lack of awareness of this condition suggests that diagnosis is a challenge and usually delayed, which leads to suboptimal treatment. Consequently, early diagnosis is mandatory to prevent potentially life-threatening bleeding complications. We present the case of an 85-year-old woman admitted to hospital with symptoms of respiratory infection who 12 hours later developed haematuria which required transfusion. Laboratory assays showed an isolated prolonged aPTT, a moderately reduced FVIII and a high inhibitor titre. Influenza A and Escherichia coli were also identified. Antivirals, antibiotics, immunosuppressive drugs and haemostatic agents were started. Two weeks later, the inhibitor was not detected, and bleeding and symptoms of infection had resolved. Immunosuppressive drugs were stopped on day 45 and there has been no recurrence since then. To date, no FVIII inhibitors have been reported in concomitant infection with influenza A and urinary E. coli. The identification of conditions potentially associated with AHA is essential to achieve complete remission.

Background:Graft-versus-host disease (GVHD) is a commonly severe multiorgan complication in patients undergoing allogeneic transplantation of hematopoietic progenitors. Its chronic form reflects a complex immune response with different degrees of inflammation, immune dysregulation and fibrosis. In some chronic graft-versus-host disease (cGVHD) patients, positive antibodies have been detected, which represent the presence of immune activity and suggest the possible involvement of B lymphocytes in the disease etiopathogenesis, but their clinical utility is controversial.Objectives:To describe the clinical characteristics of a group of cGVHD patients with positive autoimmunity treated in a multidisciplinary consultation of Rheumatology-Dermatology- Hematology of GVHD.Methods:Observational and retrospective study to describe the clinical characteristics of the patients with positive autoimmunity collected in the database of the multidisciplinary consultation of GVHD. The variables reviewed for this study, in addition to the demographic ones, were type of antibody, disease causing the transplant, presentation, severity and type of involvement. The statistical analysis was done with Epi-info 7.2.2.6.Results:Only 16 (16%) of the 100 patients included in the database had positive autoimmunity. Twelve (75%) tested positive to ANA, although 5 (31.25%) in a lower titer (1/80). The most common immunofluorescence pattern was the nucleolar in 88.89% (66.67% nucleolar and 22.22% nucleolar + cytoplasmic). Other antibodies detected were: 6 anti-Ro52, 2 anti-dsDNA, 1 anti-RP155, 1 anti-Fibrillarin, 1 anti-SAE1, 1 p-ANCA and 1 anti-NOR-90. The mean of age was 51.31±14.03 years. As for sex 4 (25%) were female and 12 (75%) were men. The most frequent disease that caused the transplant was acute myeloid leukemia (58.3%). Ten (62.5%) patients presented de novo cGVHD, 1 (6.25%) progressive and 5 (31.25%) quiescent. The time since receiving the transplant until the first visit was 14 to 79 months. Ten (62.5%) patients had nonspecific symptoms (arthralgia and myalgia), 2 (12.5%) edema, 8 (50%) contractures, 8 (50%) fasciitis and 6 (37.5%) eosinophilia. Eight (50%) patients had ocular involvement and 6 (37.5%) of the oral mucosa in the form of dry syndrome (Sjögren-like syndrome). Ten (62.5%) patients had limitation of joint mobility detected by the range of motion scale (ROM), of which 6 were mild and 4 moderate. Only 5 (31.25%) patients had general condition impairment. As for the skin involvement 10 (62.5%) patients had sclerodermiform involvement (8 of them being eosinophilic fasciitis- like), 2 (12.5%) lichenoid, and 3 (18.5%) mixed (sclerodermiform + lichenoid). Only 1 patient didn´t meet diagnostic criteria for GVHD. The sclerodermiform was the most common type of involvement in the positive ANA patients. Regarding the severity according to the of the American National Institute of Health (NIH) classification: 8 (50%) had serious affectation, 5 (31.25%) moderate and 2 (12.5%) mild, with 4 (25%) exitus.Conclusion:In our cohort of patients with cGVHD, serum detection of autoantibodies is uncommon, being the ANA with nucleolar pattern the most frequent. Although the small sample size does not allow correlations with the clinical variables it´s worth highlighting a greater positivity of autoantibodies in the sclerodermiform skin forms.References:[1]Kuzmina Z et al. Clinical significance of autoantibodies in a large cohort of patients with chronic graft-versus-host disease defined by NIH criteria. Am J Hematol. 2015 February; 90(2): 114–119.[2]Rhoades R, Gaballa S. The Role of B Cell Targeting in Chronic Graft-Versus-Host Disease, Biomedicines 2017, 5, 61: 2-10Disclosure of Interests:Maria Elisa Acosta: None declared, Luis Gómez-Lechón: None declared, Olga Compán: None declared, Sonia Pastor: None declared, Carlos A. Montilla-Morales: None declared, Olga Martínez González: None declared, Ana Isabel Turrión: None declared, Javier del Pino Grant/research support from: Roche, Bristol, Consultant of: Gedeon, Cristina Hidalgo: None declared

Objectives: To compare the presence of psycho-affective disorders and obesity-associated factors between ankylosing spondylitis (AS) and psoriatic arthritis (PsA) patients and to corelate these comorbidities with PsA disease activity.Methods: This prospective observational longitudinal study included 216 PsA patients and 114 AS patients. Anxiety and depression were compared between both patient groups using the Hospital Anxiety and Depression Scale (HADS) questionnaire. We compared the waist/hip ratio, body mass index (BMI) and laboratory variables including apolipoprotein A, apolipoprotein B, lipoprotein A, the ApoB/ApoA ratio, peptide C, insulin, resistance to insulin (HOMA-IR) and leptin. PsA activity was evaluated by the Disease Activity Index for Psoriatic Arthritis (DAPSA) score every 4 months for a year. Patients were divided into two groups: those with moderate or high activity and those with low activity or remission. We compared anxiety-, depression- and obesity-related factors between these two groups.Results: There were no differences in baseline characteristics between AS and PsA patients. Among the studied factors, only serum leptin was significantly higher in PsA patients than in AS patients (18.49±19.01 vs 11.5±10.05, p<0.01). Initial serum leptin, obesity and depressive behaviour were poor prognostic factors for persistent low disease activity. The leptin level was correlated with the visual analogue scale (VAS) pain score (R=0.21, p<0.01), VAS activity score (R=0.26, p<0.01), swollen joint count (SJC) (R=0.23, p<0.01) and tender joint count (TJC) (R=0.26, p<0.01).Conclusion: Obesity alone or concurrent with increased leptin secretion and depression may influence persistent PsA activity. Leptin levels differentiated PsA patients from AS patients.

Background:Graft versus host disease is the most frequent complication after allogeneic transplantation of hematopoietic progenitors. Its chronic form usually involves a multisystemic syndrome that reflects a complex immune response with varying degrees of inflammation, immune dysregulation and fibrosis, responsible for the characteristic clinical manifestations of the disease. Joint, muscular and fascial involvement represents one of the areas, often unnoticed or poorly evaluated, that negatively impacts the physical function and quality of life of these patients.Objectives:Describe the presence of musculoskeletal manifestations and their clinical characteristics in patients with chronic GVHD (cGVHD) evaluated in a multidisciplinary consultationMethods:Descriptive and retrospective observational study to detail the initial presence and during the follow-up of diagnostic and nonspecific musculoskeletal manifestations of cGVHD in a cohort of 103 patients included in the database. The clinical characteristics of 68 patients with a defined diagnosis of sclerotic cGVHD are described. Demographic variables are collected along with clinical conditions about transplant and in a systematic way the assessment according to diagnostic criteria of the American National Institute of Health 2015 highlighting: range of motion scale and photographic range of motion (P-ROM) scale in shoulders, elbows, hands and ankles; and laboratory data: presence of eosinophilia and autoantibodies. Descriptive and frequency statistical analysis was done using Microsoft Office Excel 2007.Results:Sixty-eight(66%)patients meet diagnostic criteria for sclerotic cGVHD during follow-up. Forty-five(66.2%) women and 23(33.8%) men, with a mean age of 54.5 years (range 10-78), Acute myeloid leukemia was the reason for transplant in 20(29, 4%) followed by non-Hodgkin lymphoma in 15 (16.2%). In 40(58.7%) patients it was performed from a related donor and with reduced intensity conditioning in 43(63.2%). Only in one patient the source of hematopoietic progenitors was bone marrow (rest peripheral blood). The average time from transplant to the first visit was 29.5 months (range 4 -168). Twelve(17.64%) patients presented isolated joint/fascial involvement without objective skin involvement (Table 1)Table 1.Clinical features of the sclerotic joint/fascial chronic GVHD cohort (N= 68)Unspecific musculoskeletal symptoms:46 (67.6%)-Artromyalgia39(57.3%)-Edema13(19%)-Stiffness 4(5.8%)Fasciitis/contractures28(41.2%)/43(63,2%)Restricted ROM1-Mild/Moderate/Severe34(50%)/14(20.2%)/4(5.8%)Impaired mobility-Shoulders26(38.2%)-Elbows21(30.8%)-Wrist/fingers29(42.6%)-Ankles21(30.6%)Sclerodermatous involvement-Superficial /deep 6(8.8%)/7(10.3%)-Mixed (sclerodermiform + lichenoid)20(29.4%)-Overlapping skin sclerosis13(19.2)NIH1Global Score-Mild/Moderate/Severe25(36.7%)/7(10.3%)/1(1.5%)Eosinophilia21 (30.8%)Autoantibodies14 (20.6%)1ROM (range of motion)2NIH: National Institute of HealthConclusion:Joint involvement secondary to sclerosis is very common in our cohort, mainly of the dorsal wrist flexion with deleterious repercussion on physical function. It needs to be recognized and evaluated early with validated scales. The search for new biomarkers associated with fibrosis, the use of advanced imaging techniques and the multidisciplinary approach can help improve the prognosis of patients with cGVHD.References:[1]Jagasia MH,Blood Marrow Transplant. 2015;21(3):389–40Disclosure of Interests:None declared

Background:Eosinophilic fasciitis (EF) is an uncommon chronic inflammatory disease characterized by myalgia, soft tissue hardening, peripheral eosinophilia and increased acute phase reactants, often triggered after strenuous physical exercise. Its appearance has been described as a rare complication after allogeneic transplantation of hematopoietic progenitors in the context of chronic graft-versus-host disease (cGVHD). Its etiopathogenesis is not well known and usually the treatment with systemic corticosteroids is effectiveObjectives:Describe the clinical and transplant-related characteristics of a cohort of patients with eosinophilic fasciitis-like in the context of cGVHDMethods:Observational, retrospective and descriptive study of the clinical characteristics of 28 patients affected by EF-like followed in a multidisciplinary consultation of cGVHD, started in March, 2014. Regular demographic variables, clinical characteristics related to the transplant and with the cGVHD, laboratory parameters, rescue therapies and their response were collected. The statistical analysis was done with Microsoft Excel 2007.Results:Seventeen (60.7%) patients were male and 11 (39.35%) were women with a mean age of 48.75 years (range from 10 to 74). Acute myeloid leukemia was the most frequent cause of the transplant in 11 patients (39.3%). Transplant related characteristics are reflected in Table 1 and the clinical manifestations, therapies received and their response in Table 2. Four (14.2%) patients died during their follow-up, being the cause of death in 2 cases due to sepsis, and in 1 case attributable to GVHD.Table 1.Baseline and transplant related characteristics (N = 28)..VariablesN (%)Donor Type (related / not related)13(46.4%)/15(53.6%)Type of conditioning (reduced intensity / myeloablative)18(64.25%)/10(35.7%)Source of cells (Peripheral blood / bone marrow)27(96.4%/1(3.6%)cGVHD type quiescent / de novo / progressive11(39.3%)/13(46.4%)/4(14.3%)Other affected organs (cGVHD score)-Mouth6(21.4%)-Eyes10(35.7%)-Lung2(7.1%)-Liver3(10.7%)-Gastrointestinal tract0(0%)-Genital2(7.1%)-Cutaneous16(57.14%)Global ScoreNIH1(mild / moderate / severe)4(14.2%)/14(50%)/10(35.7%)1NIH: National Institute of Health.Table 2.Clinical manifestations and therapies (N = 28)..VariablesN (%)/Median (range)Prodromic symptoms: yes / no20(71%)/8(29%)- Stiffness2(7.1%)- Artromyalgia17(60.7%)- Edema3(10.7%)Time until first visit31.3 months (range 9-73)Contracture Yes / No18(64.3%)/10(35.7%)Mobility limitation (mild / moderate)13(46.4%)/9(32.1%)ECOG1affected11(39.2%)Eosinophilia17(60.7%)Positive autoantibodies8(28.5%)First line therapies (corticosteroids)28(100%)Extracorporeal photoapheresis19(67.9%)Therapies of 2nd line/ 3rd or more6(21.4%)/12 (42.8%)Physiotherapy14 (50%)Response: complete / sequels10(35.7%)/18(64.2%)1ECOG: Eastern Cooperative Oncology Group scale to assess the quality of lifeConclusion:Nonspecific joint symptoms such as stiffness, edema or arthromyalgia in patients undergoing allogeneic transplantation of hematopoietic progenitors may be factors that predict the development of sclerotic GVHD type eosinophilic fasciitis-like and should be closely monitored in order to be able to perform early stage diagnoses of the disease. It is necessary to deepen the pathogenesis of this entity and the multidisciplinary approach to improve the prognosis of patients with GVHDReferences:[1]Inamoto Y. Arthritis Rheumatol. 2014;66(4):1044–52.Disclosure of Interests:None declared