Calciphylaxis is a frequent entity in patients with chronic renal failure of diverse etiology. The main pathogenic mechanism of calciphylaxis is impairment of either calcium and phosphate metabolism or plasma levels of parathyroid hormone. There are communications of patients with normal renal function, and in some cases with chronic inflammatory diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE) and antiphospholipid syndrome. We report a patient with SLE and no renal failure or hyperparathyroidism who developed severe calciphylaxis.

Severe liver involvement requiring liver transplantation is a rare complication in systemic lupus erythematosus (SLE), but very few cases have been reported. We describe a 39-year-old woman with SLE who underwent successful liver transplantation due to acute liver failure. The patient persisted without reactivation of SLE and with good long-term survival. Management and diagnosis considerations are reviewed.

BackgroundThe 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.ObjectivesTo adapt S-HAQ into Spanish and to assess its validity in SSc patients in Argentina.MethodsS-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. Cronbach's 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 Fisher's test. A value of p<0.05 was considered significant.Resultsl9An 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), Cronbach's 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: Raynaud's (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.ConclusionsA Spanish version of S-HAQ was developed, showing an acceptable reliability and validity.Disclosure of InterestNone declared

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BackgroundPeripheral neuropathy (PN) is usually is a late onset event in primary Sjögren Syndrome (pSS) associated with purpura, cryoglobulinemia, hypocomplementemia and increased risk of lymphoma.ObjectivesTo describe the frequency of PN in patients with pSS and identify related factors.MethodsAdult patients in the GESSAR database who met 2002 criteria for pSS. Demographic, clinical, laboratory and electromyogram (EMG) findings were recorded. PN was defined with clinic manifestations and EMG. Other causes of PN were excluded. To compare groups, all patients with PN were included (cases) and a random sample of patients without PN (controls) with a 1:4 ratio was used. Mann-Whitney was used for numeric variables and χ2or Fisher's for categorical. An α of 0.05 was considered significant.ResultsOf 368 patients, 95% were female. Mean age at analysis was 55 y/o (21-87) and 50 y/o (20-89) at diagnosis. The frequency of PN was 11.68% (43/368). Sensory PN was found in 63% (28/43), predominantly small fibers involvement in 41.8% (18/43), axonal PN in 20.9% (9/43) and ataxic in 2.3% (1/43). Somatosensory manifestations were found in 37% (16/43) with axonal involvement in 30.2% (13/43) and mononeuritis multiplex in 6.9% (3/43), none had autonomic PN. When comparing groups (43 vs 172 controls) patients with PN had a higher frequency of vasculitis (11.7% vs 1.7%; p=0.002), purpura (23.8% vs 4.7%, p=0.0001), renal tubular acidosis (7.6% vs 1.2%, p=0.020), leucopenia (30.7% vs. 12.1%, p=0.005), low C3 (48.5% vs. 10.3%, p=0.0001) and C4 (66.6% vs. 18.2%, p=0.0001), (+) Anti-Ro/SSA (85.3% vs. 66.6%, p=0.019), (+) RF (72.5% vs. 52.1%, p=0.022), cryoglobulinemia (42.1% vs. 10.9%, p=0.0001) and higher frequency of hypergammaglobulinemia (60.5% vs 44.6%, p=0.09), Raynaud's (27.5% vs 11.6%, p=0.051) and glomerulonephritis (4.6% vs 0.5%, p=0.018), although without statistical significance.ConclusionsThe frequency of PN was 12%, similar to other cohorts. Small fibers and axonal somatosensory PN were the most common. PN was significantly associated with vasculitis, purpura, renal tubular acidosis, cryoglobulinemia, leucopenia, hypocomplementemia and anti-Ro and RF positivity.Disclosure of InterestNone declared