ObjectiveA very few studies describe the epidemiology of primary Sjögren's syndrome (pSS). The reported frequency of pulmonary involvement in pSS varies widely depending on the detection method employed, and consists mainly of various forms of airways disease. We aimed to evaluate the incidence and mortality of pSS and of lung disease in pSS, focusing on interstitial lung disease (ILD).MethodsA population-based incidence cohort of patients diagnosed with pSS in 1976–2005 was assembled. Diagnosis was based on the 2002 American-European Consensus Group criteria for pSS. Cumulative incidence adjusted for the competing risk of death was estimated. A Cox model with a time-dependent covariate was used to determine the incidence and the standardised mortality HR of pSS.Results85 patients with pSS were identified (mean age 59.9 years; 91% women). The annual incidence of pSS was 4.2, 95% CI (3.3 to 5.1)/100 000 population and it increased with higher age at pSS diagnosis (18–44 years: 2.1/100 000 vs ≥75 years: 12.3/100 000). Standardised mortality ratio in pSS compared with the general population was 0.92, 95% CI (0.57 to 1.41). A total of 105 patients with pSS and ILD were identified (mean age 58.1 years; 91% women). Among patients with pSS without prior ILD, the cumulative incidence of ILD in patients with pSS was 10% (±3%) at 1 year after diagnosis of pSS and increased to 20% (±4%) by 5 years after pSS. The development of lung disease in pSS was associated with poor survival (HR 2.16; 95% CI 0.99 to 4.74).ConclusionspSS incidence seems to be almost the same as was reported in a previous study conducted among Olmsted County Minnesota population. Survival among patients with pSS and general population does not differ substantially. However, patients with pSS who have ILD likely have increased premature mortality.

Objectives To assess the occurrence, risk factors, morbidity and mortality associated with lower extremity (LE) ulcers in patients with rheumatoid arthritis (RA). Methods Retrospective review of Olmsted County, Minnesota residents who first fulfilled 1987 American College of Rheumatology criteria for RA in 1980–2007 with follow-up to death, migration or April 2012. Only LE ulcers that developed after the diagnosis of RA were included. Results The study included 813 patients with 9771 total person-years of follow-up. 125 patients developed LE ulcers (total of 171 episodes), corresponding to a rate of occurrence of 1.8 episodes per 100 person-years (95% confidence interval [CI]: 1.5, 2.0 per 100 person-years). The cumulative incidence of first LE ulcers was 4.8% at 5 years after diagnosis of RA and increased to 26.2% by 25 years. Median time for the LE ulcer to heal was 30 days. Ten of 171 (6%) episodes led to amputation. LE ulcers in RA were associated with increased mortality (HR 2.42; 95% CI: 1.71, 3.42) adjusted for age, sex and calendar year. Risk factors for LE ulcers included age (HR 1.73 per 10 year increase; 95% CI: 1.47, 2.04); rheumatoid factor positivity (HR 1.63; 95% CI: 1.05, 2.53); presence of rheumatoid nodules (HR 2.14; 95% CI: 1.39, 3.31); and venous thromboembolism (HR 2.16; 95% CI: 1.07, 4.36). Conclusions LE ulcers are common among patients with RA. The cumulative incidence increased by 1% per year. A significant number require amputation. Patients with RA who have LE ulcers are at two-fold risk for premature mortality.

Aim It has been a popular belief that gout does not typically occur in patients with rheumatoid arthritis (RA). Our aim was to assess the occurrence, prevalence, clinical presentation and possible risk factors for gout in patients with RA. Patients & methods We retrospectively reviewed a population-based incidence cohort of patients who fulfilled 1987 ACR criteria for RA between 1980 and 2007. The cumulative prevalence of gout in RA adjusted for the competing risk of death was estimated. Results Among the 813 patients with RA, six were diagnosed with gout prior to RA incidence and 22 patients developed gout during a total of 9771 person-years of follow-up. Nine out of 22 patients had crystal-proven gout. The 25-year cumulative prevalence of gout diagnosed by clinical criteria in patients with RA was 5.3%. Conclusion Gout does occur in patients with rheumatoid arthritis, however, at a lower rate than in the general population.

Autoimmune pancreatitis (AIP) is an uncommon cause of chronic pancreatitis with an estimated prevalence rate of 0.82/100,000 in Japan [1][2][3]. Exact incidence and prevalence in the United States is unknown. AIP is generally divided into two types: Type 1 (Lymphoplasmacytic sclerosing pancreatitis/IgG4 related) and Type 2 (Idiopathic ductcentric pancreatitis) [4,5]. Systemic glucocorticoids remain the mainstay of treatment for AIP. However, more than 50% of patients with AIP treated with steroids experience relapse with the steroid taper [6]. Remission maintenance in many patients requires chronic steroid use, which poses risk of adverse effects [7]. In order to minimize risk of chronic steroid use, drugs such as azathioprine (AZA), 6-mercaptopurine (6-MP) and rituximab (RTX) have been used in AIP [7]. Thiopurine Methyl Transferase (TPMT) activity is reduced in about 11% of general population, who therefore may not tolerate AZA or 6-MP [8]. Also, AZA is associated with drug-induced pancreatitis, which may mimic AIP flare and cause therapeutic confusion. Disadvantages of RTX include its requirement for intravenous infusion and its expense. Hence there is a need for alternative cheaper, effective and safer oral steroid-sparing agents for AIP. Mycophenolate mofetil (MMF), a powerful inhibitor of lymphocyte proliferation, is a commonly used immunosuppressive agent by rheumatologists. It is available in generic form and also the safety profile is known over few decades. It may be better tolerated than AZA or 6-MP in some patients. However data regarding efficacy and safety of MMF in AIP are lacking. As of 4-2016, less than 15 such cases have been reported in Englishlanguage literature [9][10][11][12]. AIP patients were identified by Rheumatology and Gastroenterology providers at the University of Minnesota (UMN), a tertiary referral center for patients with pancreatic diseases. On the basis of previous case series [13], UMN providers have used mycophenolate for AIP treatment in recent years. Here we present one center's experience with mycophenolate mofetil in 4 patients with AIP.Patient A is a 23-year-old female with history of celiac disease, type 1 diabetes mellitus, and chronic urticaria who presented with recurrent abdominal pain. Her abdominal pain was initially thought to be due to sphincter of Oddi dysfunction, but failed to respond to biliary and pancreatic sphincterotomies by endoscopic retrograde cholangio-pancreatography (ERCP). She was started on prednisone 40mg daily for chronic urticaria of unclear etiology (skin biopsy was non-specific) and the prednisone was quickly tapered off. Surprisingly, her abdominal pain improved dramatically. However, she was not a candidate for long-term prednisone therapy because of underlying type 1 diabetes mellitus and anxiety. MMF (750 mg twice daily) was started for her urticaria. However both urticarial rash and abdominal pain improved. When MMF dose was tapered down, her abdominal pain worsened. EUS showed hyper-echoic foci and lobularity in the pancreas. Ampullary ...