Juan Carlos Barreira

Holter monitoring in ankylosing spondylitis patients during methylprednisolone pulse therapy

SummarySudden death following steroid pulse therapy has been recently reported. Continuous electrocardiographic recording was performed 24 hours before, during, and 24 hours after each one to three high dose intravenous methyl prednisolone pulses administered of five patients with severe ankylosing spondylitis unresponsive to conventional therapy. No increase in supraventricular or ventricular arrythmias was observed. Bradiarrythmias, conduction disturbances or ischemic changes were not found. Cardiovascular symptoms did not occur in any case; there were no significative changes in any of the clinical controls. Transient elevations of serum glucose were observed in all patients. Although a transient increase in potassium and decrease in sodium urinary excretion was noted, serum levels remained within normal values.

Coexistence of diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis

SummaryTo the best of our knowledge, only two patients with concurrent diffuse idiopathic skeletal hyperostosis (DISH) and ankylosing spondylitis (AS) have been reported so far. Here we present 3 patients in whom clinical and radiological findings indicative of DISH and AS coexisted. Two of these cases exhibited HLA B27. Although the presence of sacroiliitis would appear to exclude DISH, calcification and ossification of the anterior common vertebral ligament (ACVL) confirmed diagnosis of the latter disease.

The -2518 A/G polymorphism in the monocyte chemoattractant protein 1 gene is associated with the risk of developing systemic lupus erythematosus in Argentinean patients: a multicenter study

Systemic lupus erythematosus (SLE) is a systemic, autoimmune disorder. Monocyte chemoattractant protein 1 (MCP-1), a chemokine involved in the recruitment and migration of monocytes/macrophages, has been shown to be increased in the plasma of SLE patients. The aim of our study was to evaluate the possible association of the polymorphism -2518 of the MCP-1 gene with the risk of developing SLE, manifesting lupus nephritis (LN) and with other clinical features of SLE in an Argentinean population. A group of 171 SLE patients and 120 control subjects were examined. Genotypic and allelic frequencies of the MCP-1 -2518 A/G polymorphism showed significant differences between the SLE and the control groups (p=0.001 and p=0.01, respectively). However, the polymorphism showed no association with LN or with the other clinical variables studied. Our results suggest that the presence of the MCP-1 -2518 A/G polymorphism might be a risk factor for developing SLE in genetically predisposed individuals, but it does not seem to have a role in the evolution of the disease in the Argentinean population.

Validation of an Argentine version of Lupus Quality of Life questionnaire.

Objective To determine reproducibility and validity of an Argentine version of the Lupus Quality of Life questionnaire (LupusQoL) and to determine cut-off values in the questionnaire. Materials and methods One hundred and forty-seven systemic lupus erythematosus patients (American College of Rheumatology 1982/1997) were assessed from April 2014 to July 2014. Demographic and socioeconomic variables were collected, as well as SELENA/SLEDAI, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index Score, comorbidities and treatment data. Patients completed LupusQoL-Argentine version and European Quality of Life Questionnaire (EuroQoL-5D). Internal consistency and reliability were examined. Convergent validity with EuroQoL-5D was assessed through analysis of latent classes, which established homogeneous categories from the responses of each domain of LupusQoL and for the total. Results Out of 147 patients, 93.2% were female, mean age 36.4 ± 11.1 years, mean disease duration 2.7 ± 9 years, mean SELENA/SLEDAI 2.7 ± 3 points. The cut-off point that defined good or bad quality of life was 0.739 for EuroQoL 5D and 63 for LupusQoL. Cut-off values for each LupusQoL domain were also defined, creating two classes in each of them. There was moderate to high concordance to classify quality of life (Kappa = 0.74, 95% confidence interval = 0.54, 0.95). Conclusion The Argentine version of LupusQoL is a valid, reliable and reproducible instrument to assess quality of life. In this study, cut-off points that allow the classification of patients regarding whether they have good or bad quality of life are established for the first time.

Estudio multicéntrico de prevalencia de anticuerpos antirribosomal P en lupus eritematoso sistémico de comienzo juvenil comparado con lupus eritematoso sistémico del adulto

OBJECTIVE To investigate the prevalence and associations with clinical manifestations of anti- P ribosomal antibodies in patients with juvenile-onset and adult-onset systemic lupus erythematosus (SLE). METHODS Clinical and serological data of 30 patients with juvenile-onset SLE (age at onset younger than 16 years old) were compared with data of 92 patients with adult-onset SLE. Symptoms occurring during the entire disease course were considered. Anti- P ribosomal antibodies were tested by ELISA. RESULTS Anti- P ribosomal antibodies were found significantly more often in pediatric-onset SLE patients (26.7% vs. 6.5%; OR=5.21 [CI95%=1.6-16.5], p=0.003). Alopecia (OR=10.11, CI 95%=1.25-97) and skin rash (non discoid) (OR=4.1, CI 95%=1.25-13.89) were significantly associated with anti- P ribosomal antibodies. CONCLUSION Anti-ribosomal P antibodies are more often found in patients with juvenile SLE. Alopecia and skin rash were the only clinical manifestations associated to anti-ribosomal P antibodies.

Long-term Outcome of Lupus Nephritis Class II in Argentine Patients: An Open Retrospective Analysis.

BackgroundThere is controversy in medical literature over the outcome of patients with lupus nephritis (LN) class II. The aim of this study was to explore the risk of histological transformation (HT) and possible factors related to negative response to treatment in patients with mesangial LN class II. MethodsA retrospective and multicenter study was carried out that includes patients who had received a diagnosis of LN class II on their first renal biopsy. Creatinine, urine sediment, and proteinuria were recorded at the time of the first biopsy, 6 months, and 1, 2, and 5 years after the first biopsy. Response to treatment, HT, and long-term outcome were evaluated. ResultsForty-one patients were included. The manifestation at first biopsy was proteinuria greater than 0.5 g/d in 28 patients (68.29%; 8 [28.57%] of 28 patients had nephrotic syndrome), hematuria in 18 patients (43.90%), and deterioration of renal function in 3 patients (7.31%). During the follow-up (median, 8 years; range, 1–35 years), a new biopsy was performed in 18 patients (43.90%), and in 17 patients (17/18 [94.44%]), there was HT. Median time at rebiopsy was 32 months (range, 11–305 months). Of the 18 patients who had a second biopsy, 10 (55.55%) were on hydroxychloroquine versus 100% (19/19) of patients who did not undergo the procedure (P = 0.001). A year after the first renal biopsy, there are data available from 34 patients; of them, 24 patients (70.58%) had achieved response, and 10 patients (29.41%) had no response (NR) (missing data in 7). A higher 24-hour urinary protein at 6 months was predictor of worse outcome at 1 year, with statistical significance difference for the nonresponder group (median proteinuria, 2.3 g/d [range, 0–4.7 g/d]) compared with responders (median proteinuria, 0.28 g/d [range, 0–1.7 g/d]) (P = 0.0133).In the long-term follow-up (5 years), HT was the main cause of unfavorable outcome and was measured in 78.57% of patients (11/14 patients). ConclusionsThis series shows a high rate of HT in long-term follow-up. Proteinuria at 6 months made it possible to set aside patients who will have an unfavorable outcome in the long term and who will thus benefit from a more aggressive treatment. The results suggest that hydroxychloroquine had a nephroprotective effect.

Reumatismo palindrómico (Enfermedad de Hench Rosemberg): Caso clínico con respuesta a los glucocorticoides

Palindromic rheumatism is characterized by multiple recurrent episodes of arthritis and periarthritis (mono or oligoarticular) that may last hours or days, disappearing without sequels. We report a 69-year-old male with a history of hypertension and a presumptive diagnosis of gout due to recurrent episodes of arthritis and periarthritis in the last thirty years. They involved at least two joints, lasted few days and were self limited. The patient was admitted due to arthritis and periarthritis of both wrists, knees, ankles, elbows and hands. He presented with fever (38-39 degrees C), intense articular pain and anorexia. With a presumptive diagnosis of palindromic rheumatism and the lack of response to non steroidal anti inflammatory drugs, methylprednisolone 20 mg/od per os was started, with an excellent response.

AB1206-HPR Adaptation into spanish of the scleroderma health assesment questionnaire (S-HAQ)

Background The Health Assessment Questionnaire (HAQ) is an instrument administered to patients to self-report functional status originally in rheumatoid arthritis (RA). In Argentina, it has been translated and validated for RA in 2004. For diffuse SSc, HAQ has been associated to morbidity and mortality. Objectives To adapt S-HAQ into Spanish and to assess its validity in SSc patients in Argentina. Methods S-HAQ was translated following a forward-backward translation procedure of the original English version, and transcultural adaptation was performed by a comprehension test reaching the final Spanish version. SSc patients that fulfilled ACR 80 criteria and early Systemic Sclerosis according to Le Roy and Medsger criteria were included. Patients with overlap were excluded. Cronbachs alpha and item-item item-total correlations were used to assess internal consistency. Construction validity was analyzed through factor analysis with Varimax rotation. Continuous variables were compared by t-test, Mann-Whitney or Kruskal-Wallis test, and categorical variables by chi-square or Fishers test. A value of p<0.05 was considered significant. Results l9An adapted Argentine-Spanish version of S-HAQ was developed. One hundred patients were surveyed; 84% were female, mean age 54±12.8 years and disease duration 8.8±9.1 years. Limited SSc was more frequent (63%), followed by diffuse SSc (36%). Serologically, 89% were ANA positive, 27% had anti Scl 70 and 41% had anti centromere antibodies. Median Rodnan score (mRSS) was 9.8 (0–40.5) and median activity measured by EUSTAR was 1.25 (0–6). Median S-HAQ was 0.62 (0–2.5), Cronbachs alpha 0.89, and when removing questions one by one the coefficient decreased. Median VAS (visual analogue scale) was 0.57 (0–2.8). Factor analysis identified two factors for the S-HAQ: factor 1: dressing (0.61), arising (0.68), reach (0.63), and personal hygiene (0.70); factor 2: eating (0.68), grip (0.72), walking (0.49), usual activities (0.62). For questions, three factors were identified through VAS: factor 1: overall disease severity (0.63) and gastro-intestinal symptoms (0.57); factor 2: Raynauds (0.66), digital ulcers (0.56); factor 3: respiratory symptoms (0.43). There was a statistically significant association between higher values of S-HAQ and higher values of mRSS (1.1±0.74 vs. 0.64±0.5 p=0.002) and also with seropositivity for anti-Scl 70 (p=0.003). Higher values of total VAS were associated to female gender (0.75±0.5 vs. 0.49±0.71, p=0.01). There was a significant association between S-HAQ and MEDSGER (p=0.04) and EUSTAR (p=0.03) scores; likewise, between VAS and MEDSGER (p=0.00) and EUSTAR (p=0.00) scores. Conclusions A Spanish version of S-HAQ was developed, showing an acceptable reliability and validity. Disclosure of Interest None declared

AB0451 Clinical and Serological Features of Sjögren's Syndrome Associated with SSB/LA

Background Sjögrens syndrome (SS) is an autoimmune disease associated with focal lymphocytic infiltration in the epithelium of exocrine glands, and may develop systemic manifestations in about 80% of the patients. Typical antibodies present in SS are antinuclear antibody (ANA), rheumatoid factor, SSA/Ro and SSB/La. SS classification criteria includes SSA/Ro and SSB/La, and their presence is associated with extra-glandular manifestations. The prevalence of SSA/Ro in SS is 30–70%, and is related to increased frequency of dermatologic symptoms, scleritis and interstitial lung disease. The relationship between SSB/La and clinical manifestations has not been previously well established, though it can be found in 5–50% of SS, and may co-exist with SSA/Ro half of the times. SSB/La is found isolated in a very low percentages of SS. Objectives To evaluate the correlation between the presence of SSB/La antibody with distinct clinical, demographic and serological characteristics of SS. Methods Adult patients evaluated in the Rheumatology Section (Buenos Aires British Hospital) who fulfilled American European Consensus Group (AECG) SS criteria were included. Three groups were compared according to the antibodies present: 1. Positive SSA/Ro and negative SSB/La 2. Both positive 3. Negative SSA/Ro and positive SSB/La. Mann-Whitney and Fisher tests were used according to variables; those with p<0.05 were introduced in a multivariable analysis by logistic regression. Results Among 195 patients with SS, 93.8% (n=183) were female. Mean age was 54.7 years and the mean total disease duration was 68.5 months. We excluded from analysis 16/8.2% patients due to missing data, and 29/14.9% patients that were negative for both antibodies. Two groups were finally compared (Group 3 excluded due to low prevalence, n=3/1.5%): 80/41% patients with positive SSA/Ro and negative SSB/La; and 67/34.3% positive SSA/Ro and SSB/La. In the univariate analysis, positive SSA/Ro and SSB/La was statistically found to be associated with recurrent sialoadenitis and palpable purpura. Serological findings associated with this group included positive rheumatoid factor, hypocomplementemia and hypergammaglobulinemia. Poor prognosis features such as palpable purpura (OR 9.5 IC 95% 1.1–79 p 0.037), recurrent sialoadenitis (OR 3.1 IC 95% 1.2–7.7 p 0.011), hypocomplementemia (OR 3.6 IC 95% 1.4–9.4 P 0.0075) and hypergammaglobulinemia (OR 2.7 IC 95% 1.2–6.1 p 0.01) were also statistically significant in the multivariate analysis. Cryoglobulinemia and leucopenia were not found to be associated in both of the analysis. Conclusions In our population, the presence of SSB/La in patients with positive SSA/Ro was related to recurrent sialoadenitis, palpable purpura, hypocomplementemia, hypergammaglobulinemia and reumathoid factor. Although previous studies are contradictory, our study might justify the systematic search of this antibody due to its clinical relevance and link to poor prognosis factors in SS. Disclosure of Interest None declared

AB0616 Adaptation into spanish of The Hand Mobility in Systemic Sclerosis Test (Hamis) in A Group of Patients with Systemic Sclerosis

Background Hand function in scleroderma patients can be assessed by specific instruments such as the Cochin scale and the Scleroderma Health assessment questionnaire (SHAQ), both self administrated. Sanqvist and Eklun designed the Hand Mobility in Systemic Sclerosis test (HAMIS), which is performed by the evaluation of a health professional. The original instrument was designed in English. Objectives The aim of this study was to adapt into Spanish the HAMIS test in order to assess hand function in patients with systemic sclerosis and to evaluate its reliability and validity. Methods The study included 28 patients that met ACR/EULAR 2013 classification criteria for systemic sclerosis, who attended the Rheumatology Service from March 2014 to March 2015; 21 were diffuse and 7 limited. Patients with overlapping and sclerodermiform syndromes were excluded. Demographic data, clinical manifestations, laboratory studies including ANA by IIF (Hep2) were recorded. Different scores were performed: modified Rodnan, Medsger and Cochin. HAMIS was translated into Spanish and back translated into English. Four translators did not know the original English version of the test. A pilot test was conducted in 5 systemic sclerosis patients and adaptations were performed in order to obtain the final Spanish version. HAMIS test was assessed by an occupational therapist. Two weeks later, the study was repeated in 10 patients chosen randomly to assess stability (test-retest) and concordance according to Bland&Altman. Values of HAMIS and Cochin test were correlated (Spearmans Rank correlation coefficient). Results All 28 patients (27 female) had mean age at diagnosis of 49.6±13 years and median disease duration of 5 years (range=0–25). All patients developed Raynauds phenomenon (mean age at onset of Raynaud of 45±17 years), 3 patients had puffy hands, 11 presented digital ulcers, 9 pitting scars and 23 telangiectasia. Through capillaroscopic evaluation, early pattern was found in 11 patients, active in 7 and late in 8 patients. When analyzing severity by Medsger score, such was normal in 1 patient, 11 presented mild involvement, 13 moderate and 3 severe. Median for Rodnan score was of 6 (range=2–27) and 8 for Cochin (range=0–38). Median for HAMIS was of 6 (range=0–21) with Cronbachs alpha of 0.81; in test-retest: mean of the difference was of 0.091 (range= -5 to +5). Correlation between HAMIS and Cochin was significant (r=0.65; p=0.0002) Conclusions An adapted Spanish version of HAMIS was developed, showing an acceptable reliability (Cronbach and stability) and criterion validity (correlation with Cochin test). Disclosure of Interest None declared