Objectives To analyse the predictive values of inflammatory back pain (IBP), positive HLA B27 antigen, increased C-reactive protein (CRP), Spondyloarthritis (SpA) features, familial history (FH), magnetic resonance sacroiliac joints (MRI-SIJ) imaging and its weight in early SpA diagnosis. Methods 133 patients with back pain, aged <50, duration of the pain <2 years were included. Data such as IBP, HLA B27, increased CRP, SpA features, FH, SIJ´s radiography and MRI were collected for each patient. STIR sequences were classified as strongly positive bone morrow oedema (SPBME ≥2), clearly present and easily recognisable as positive according to the ASAS criterion, weakly positive (WPBME ≥2), suggestive, but not easily recognisable and, clearly negative none of those features. T1-weighted sequences were assessed as positive/negative for erosion, fat metaplasia, backfill and sclerosis, if ≥1, for each lesion was present. MRI images were read by three blinded readers. Results The average age was 38.9 years. 47 (35.3%) patients received SpA diagnosis according to the clinical opinion. IBP was highly specific, 0.81 and sensitive, 0.83. HLA B27 was positive in a half of the SpA patients. SPBME ≥2 provided a great specificity, 0.94 and an acceptable sensitivity, 0.79. Erosion was significantly more frequent in SpA patients (72% vs 7%), specificity 0.93. The addition of erosion ≥1 to the WPBME ≥2 noticeably improved specificity, 0.98, although slightly decreased sensitivity, 0.64. Fat metaplasia and backfill were highly specific, but poorly sensitive. Factors forecasting positive diagnosis were IBP, followed by SpA features and increased CRP. Conclusions At the onset, IBP might be a good marker for selecting patients with suspicion of SpA. The addition of erosion to the ASAS criterion might be helpful for early diagnosis, especially in patients with doubtful STIR imaging where BME is present but it is hard to determinate whether the ASAS “highly suggestive” criterion is met.

BackgroundDiagnosis of Spondyloarthritis remains challenging in the daily practise. Inflammatory back pain might be a good tool for early diagnosis.ObjectivesTo analyse sensitivity, specificity and predictive values of inflammatory back pain (IBP), positive HLA B27 antigen, increased C-reactive protein (CRP), positive sacroiliac joints (SI) magnetic resonance (MRI) imaging, additional features (AF) such as peripheral arthritis, dactylitis, psoriasis, uveitis, inflammatory bowel disease (IBD) and familiar history (FH) and assesse probabilities to develop SPA.MethodsWe prospectively collected and follow up 82 patients referred to our department with suspicion of SPA from September 2014 to December 2016. Data such as IBP, HLA B27, additional features, familiar history of SPA, increased CRP, sacroiliac x-Rays and sacroiliac MRI imaging was performed for each patient. Each MRI image was separately and independently evaluated by rheumatologist and radiologist.ResultsThe average age in our study was 39.8 years with male/female ratio 0.4/1. 37 (45.1%) patients were diagnosed with axial SPA. Radiographic sacroiliitis had only 5 (6.1%) patients. AF had 21 (25.6%) patients. IBP was found in 36 (43.9%) patients, positive HLA B 27 antigen in 24 (29.3%) and increased CRP in 22 (26.8%). Sacroiliac joints (SI) MRI images were assessed as clearly positive if patients had more than 2 highly specific for SPA bone marrow oedema (BME) lesions, at least 3 fatty lesions and more than 1 erosion, positive MRI image if patients had at least 2 highly specific BME lesions, and clearly negative MRI images if patients had not got any of those features. We found 83.78% sensitivity and 88.89% specificity for IBP, 37.84% sensitivity and 80.95% specificity for positive HLA B27 antigen, 43.24% sensitivity and 88.1% specificity for increased CRP. AF such as such as peripheral arthritis, dactylitis, psoriasis, uveitis and IBD, evaluated together reached sensitivity 37.84% and specificity 84.44%. Positive FH only contributed to the diagnosis with 13.51% sensitivity, but showed higher specificity (84.44%). Sensitivity for positive SIJ MRI imaging were poor (51.35%) but reached excellent specificity (100%). Predictive values in our study were as follows: 86.11% predictive positive values (PPV) and 86.96% predictive negative value (PNV) for IBP, 63.64% PPV and 59.65% PNV for HLA B27, 76.19% PPV and 63.79% PNV for increased CRP, 66.67% PPV and 62.30% PNV for AF. Positive FH contributed to the diagnosis with 66.67% PPV and 62.30% PNV. Positive MRI reached 100% PPV and showed high PNV −71.43. Multivariate analysis displayed 81.8% likelihood to be diagnosed for SPA if only IBP without AF at the onset of the diagnosis and 94.8% if both IBP and AF were presented.ConclusionsAt the onset, IBP may be a good indicator for SPA with high sensitivity and acceptable specificity. Additional feature such as peripheral arthritis, dactylitis, psoriasis, uveitis and IBD might increase the possibility of SPA. HLA B27 antigen. increased CRP and FH brings low sensitivity for ...

Objective: To analyse clinical features, triggering diseases, treatment strategies and prognostic factors in patients with secondary haemophagosytic limphohistosytosis (SHLH). Methods: We retrospectively analysed thirty-three patients with positive haemophagocytosis bone marrow biopsies, all collected between 1995 and 2015 from two different hospitals. Results: The average age was 44.39 years with a man/women ratio 1.06/1. The underlying diseases were as follows: Autoimmune diseases (n=11), liver or kidney transplant (n=9), haematological malignancies (n=5) infection (n=5) and solid organ cancer (n=3). The average time from hospitalisation to death was 49.95 days (49.95 ± 39.608). Three different prognostic factors were separately analysed: overall mortality, severe disease (less than two months to death) and extremely severe disease (less than one month to death). Risk factors associated to overall mortality were age >35 years (p<0.011), severe cytopenias such as anaemia (p<0.002), bicytopenia (p<0.007) and pancytopenia (p<0.025), ongoing lung involvement (p<0.012) and sepsis (p<0.044). Risk factors for severe disease were underlying treatment with corticosteroids alone (p<0.013), severe anaemia (p<0.002), neutropenia (p<0.027) and pancytopenia (p<0.016). Severe hypofibrinogenemia (p<0.039), ongoing lung involvement (p<0.022) and a late start to specific treatment (p<0.047) were highly indicative for severe disease, whereas underlying autoimmune disorder (p<0.003) and the simultaneous use of immunosuppressant at the onset (p<0.007) seemed to act as a protective factor for severe disease. Risk factors for extremely severe disease were underlying solid organ cancer (p<0.043), severe cytopenias such as thrombocytopenia (p<0.016), bicytopenia (p<0.016) and pancytopenia (p<0.009), and a late start to specific treatment (p<0.043). Conclusions: Patients with secondary HLH might have a different prognosis according to the triggering disease. Underlying autoimmune disorder might be related to a better prognosis and malignancy might indicate high mortality. Early recognition and specific treatment is essential for the patient's survival whereby tight suspicion is necessary for an attempt at curative therapy to be made.

BackgroundThe secondary hemophagocytic lymphocytosis syndrome (SLHS) is a rare fatal diseasewithout specific treatment. In the present study 32 cases are reported on with SLHS diagnosed by bone marrow biopsy in the period from 1995 to 2013.ObjectivesComparison between patients with Macrophage activation syndrome (MAS) and other causes of secondary hemophagocytic lymphohistiocytosis (HLH). Disease triggers, causes, course, treatment and pronostic factors.MethodsOutcomes analysed were overall survival (from the time of patient's hospitalization until the patient's death) and due to fact that the majorities of the demises were performed in the first 5 weeks, two another variables were chosen: extremely severe disease (demises in the first month) and severe disease (demises in the first two months). The variable age was assessed by T Student. Chi square and cross tabulation were used for analyze categorical variables. Statistical significance was set at P<0.05. Associations were expressed using Odds ratios (ORs) with 95% confidence interval (95% CI).ResultsThe average age of patients was 44.50 years (range 14-78 years), with a ratio 1.13/1 men/women. The associated diseases were: autoimmune illness - 10 (31.3%), Kidney or Liver transplant - 9 (28.1%), hematological disease - 5 (15.6%), infection -5 (15.6%) and solid cancer - 3 (9.4%) patients. The most frequent clinical manifestations were a temperature over 38.5° 32 (100%) patients, splenomegaly, 23 (71.9%) and severe hepatopathy 19 (59.4%). The most prominent analytical data was: anemia less than 8.5 mg/dl in 18 (56.3%) of the patients, neutropenia less than 500 in 13 (40.6%) and thrombocytopenia less than 35,000 in 15 (46.9%) of them. The prognostic factors associated with mortality were: age>47 years (p<0.013), the associated cancer (p<0.049), the previous treatment with glucocorticoid (p<0.011), previous use of mycophenolate mofetil (p<0.032), the presence of anemia (p<0.03), bicytopenia (p<0.005), severe pancititopenia (p<0.020) and affectation of vital organs such as the lung (p<0.017). Significant association was found between the severity of the disease and anemia (p<0.001), neutropenia (p<0.036) thrombocytopenia (p<0.020) and pancytopenia (p<0.020) severe, hypofibrinogenemia lower than 100 mg/dl, the associated cancer and the coincident infection. The non instauration of specific treatment is associated to worse prognosis and higher probability to develop severe illness (p<0.049), for which close monitoring is necessary and more aggressive treatment from the clinical suspicion.ConclusionsThe main differences reside in the better prognosis of the patients with MAS and the lesser proportion associated with severe illness (p<0.005). The presence of autoimmune disease seems to act as a trigger factor per se, and the infection does not appear to play an important role in patients with MAS (p<0.018). The Ferritin levels higher than 5,000 were more typical for patients with previous autoimmune disease MAS (p<0.046).AcknowledgementsAll my colleages.Disclosure of Int...

ObjectivesTo analyse sensitivity, specificity and predictive values of inflammatory back pain (IBP), positive HLA B27 antigen, increased C-reactive protein (CRP), clinical features (CF) such as peripheral arthritis, dactylitis, psoriasis, uveitis, inflammatory bowel disease and enthesitis, familial history (FH) of SPA and assess probabilities to develop SPA. To evaluate the impact of chronic (T1) MRI lesions in early diagnosis of SPAMethodsWe prospectively collected and followed up 133 patients referred to our department with suspicion of SPA from September 2014 to March 2018. Data such as IBP, HLA B27 antigen, increased CRP, CF, FH and sacroiliac X-rays were collected for each patient. STIR and T1 MRI imaging were separately and independently evaluated by a rheumatologist and two radiologists. In case of disagreement, agreement between two readers was regarded as conclusive. MRI STIR sacroiliac joints (SI) images were assessed in a semi-qualitative way as follows: 1) strongly positive MRI imaging(SPMRI): patients with at least 2 highly specific bone marrow oedema(BME) lesions, easily classifiable as SPA. 2) Weakly positive MRI imaging(WPMRI): patients with at least 2 tiny BME lesions, suggestive, but not easily classifiable as SPA. 3) Clearly negative MRI imaging: patients without any of those features. T1 SI MRI images were assessed for erosion, fat metaplasia, backfill and sclerosis in a qualitative way as positive, if there were 1 or more than one, or negative, if there were none of those lesions.ResultsThe average age in our study was 38.9 years. 47(35.3%) patients received diagnosis axial SPA. Radiographic sacroiliitis had 8(17%). Sensitivity (St), specificity (Sf) and predictive positive values (PPV) were found as follows for each item (p<0.001): IBP: 83% St, 81.4% Sf, 71%PPV, positive HLA B27: 49% St, 80% Sf, 57.5% PPV, increased CRP: 40.4% St, 93% Sf, 76% PPV, and CF: 36.2% St, 91% Sf, 68%PPV. Multivariate logistic regression binary analysis applied to the group of patients with non-radiographic SPA showed Odds Ratios for positive diagnosis, 64.8 for IBP (p=0.000), 9.4 for increased CRP (p=0.005) and 11.9 for CF (p=0.006). For the total group of patients the Odds Ratios were as follows: 49.2 for IBP (p=0.000), 10.3 for increased CRP (p=0.002) and 11.3 for CF (p=0.002). STIR MRI imaging assessment proved acceptable sensitivity (79%), excellent specificity (94%), and high PPV (88%) for SPMRI (p<0.001). WPMRI displayed better sensitivity (87%) nonetheless, specificity (67%) and PPV decreased (59%) (p<0.001). T1 MRI examination showed reasonable sensitivity (72%) excellent specificity (97%) and high PPV (85%) for erosion (p<0.001). Sensitivity for backfill was lower (32%), but specificity (100%) and PPV (100%) were excellent (p<0.001). Association, erosion and SPMRI pointed out a highly strong specificity (100%) and PPV (100%), however decreased sensitivity (51%) (p<0.001). Association, erosion and WPMRI significantly increased specificity (98%) and PPV (94%), although slightly decreased sensibility (64%)(p<0...