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Objectives: Patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) have increased IL-1β levels. IL-1β and other pro-inflammatory cytokines have a modulating activity on cardiac ion channels and have been associated with increased arrhythmic risk in rheumatoid arthritis patients. Likewise, adult patients with connective tissue diseases (CTDs) may have prolonged QTc intervals associated with the presence of anti-Ro/SSA antibodies. Our objective was to evaluate the presence of serum IL-1β in subjects with CTDs, in relation to the presence of anti-Ro/SSA antibodies and QTc interval duration., Methods: 12-lead electrocardiograms (ECG) were performed and blood was withdrawn, measuring electrolytes, IL-1β anti-Ro/SSA antibodies by ELISA in 73 patients with CTDs., Results: 55 patients were anti-Ro/SSA positive and 18 were anti-Ro/SSA negative. Patients with anti-Ro/SSA positive antibodies had a significantly greater median IL-1β serum level: 7.29 (range: 0.17-17.3 pg/ml) compared to patients with anti-Ro/SSA negative antibodies whose median was: 1.67 (range 0.55-4.12 pg/ml) p<0.001. The mean QTc interval values obtained in both groups were not significantly different (417.7±23.1 vs. 414.7±21.2, p=0.63). The QTc interval was prolonged in 11 (20%) patients, who were all anti-Ro/SSA positive versus 0 (0 %) in anti-Ro/SSA negative patients p=0.05. Median IL-1β levels were: 8.7 (range: 2.69-15.1 pg/ml) in patients with prolonged QTc interval versus median: 5.0 (range: 0.17-17.3 pg/ml) in those with normal QTc interval values (<440ms) p=0.006., Conclusions: IL-1β is elevated in patients with CTDs that have both anti-Ro/SSA antibodies and prolonged QTc intervals.

A 55-year-old man with granulomatosis with polyangiitis (GPA) developed continuous parietal headache, malaise, nasal crusting and dry cough. Neurological exam revealed only left hand hypoesthesia in 4th and 5th finger. Brain MRI showed enlarged right choroid plexus, hyperintense periventricular white matter, thalami and right side of corpus callosum. The suspected diagnosis was ependimoplexitis due to GPA, the patient received three 500 mg methylprednisolone pulses followed by 1 mg/kg of meprednisone with gradual tapering and was switched to oral cyclophosphamide. He had complete resolution of headache. An MRI following this treatment for relapse revealed only minimal ependimal changes.

Objective: Skin disease can be one of the most refractory clinical manifestations of systemic lupus erythematosus (SLE). The standard therapy consists of sunscreens, topical corticosteroids and antimalarials. However in difficult cases a variety of other drugs have been tried. Here we describe our clinical experience with mycophenolate mofetil (MMF) in patients with cutaneous manifestations of SLE., Methods: Seven patients with SLE and skin involvement (including acute cutaneous lupus, subacute cutaneous lupus, discoid lupus erythematosus, vasculitis, urticarial rash and chilblain lupus) who had received treatment with MMF were included. The clinical characteristics, serologicalfindings and response to treatment were recalledfrom retrospective review of the files., Results: Our results showed no response in 5 patients, partial response in 1 patient and initial response but skin flare whilst on MMF in 1 patient. The median dose of MMF was 2 g (range 2-3 g). Adverse events on MMF were mild, mainly gastrointestinal and occurred in 5 patients. No patients discontinued MMF due to adverse events., Conclusions: MMF appears not to be particularly effective in the treatment of skin disease in SLE. It should be noted that our group of patients had previously failed to respond to a median of 4 (range 2-10) different drugs used to treat SLE skin disease. Thus, the patients in the study could be considered at the severe end of skin disease spectrum.