Israel Medical Association Journal

Sjögren’s syndrome (SS), a chronic systemic autoimmune inflammatory condition involving the exocrine glands, has been suggested to be part of the spectrum of the “Autoimmune/inflammatory Syndrome Induced by Adjuvants” (ASIA). ASIA incorporates an umbrella of clinical conditions including siliconosis, macrophage myofasciitis syndrome, and post-vaccination phenomena that occur after the exposure to a substance, namely the adjuvant. Interestingly, SS and ASIA share several common features. Firstly, a shared pathogenic mechanism involving a disruption of the immune system balance, with B cell proliferation, cytokine production and tissue infiltration, have been proposed. Patients with ASIA often present clinical features resembling those of SS; dry mouth and dry eyes have also been included in the proposed classification criteria for ASIA. Finally, several case reports have suggested that both vaccines and silicone may trigger the development of SS. Unveiling these common pathways will contribute considerably to our understanding and managing of both conditions.

There is growing interest in the contribution of vitamin D deficiency to autoimmunity. Several studies have shown an association between low levels of vitamin D and autoimmune disorders, including multiple sclerosis, rheumatoid arthritis, type 1 diabetes, autoimmune thyroid diseases, celiac disease, and systemic lupus erythematosus (SLE). Vitamin D receptor ligands can mediate immunosuppressive effects. It has been suggested that low levels of this hormone contribute to the immune activation in lupus and other autoimmune diseases. This review updates and summarizes the literature on the association between vitamin D and SLE, and discusses the various correlations between vitamin D and SLE activity, clinical expressions, serology, and gene polymorphisms of vitamin D receptors.

Sarcoidosis is a chronic multisystem disease with variable course resulting from the interaction between environmental factors and the immune system of individuals genetically predisposed. The evidence linking sarcoidosis with environmental triggers such as metals is increasing. We describe the case of a 44 year old female with a history of smoking since age 30 and previous mercury dental filling who presented at physical examination with numerous subcutaneous nodules. Laboratory data showed accelerated erythrocyte sedimentation rate and high titer of anti-U1 ribonucleoprotein antibodies (U1-RNP). Skin biopsy and chest X-ray suggested the diagnosis of sarcoidosis. In this report we illustrate the different causes involved in the onset of sarcoidosis.

Background: Androgen deprivation therapy (ADT) added to radiation therapy (RT) in intermediate to high risk prostate cancer negatively impacts quality of life. 

Objectives: To compare health-related quality of life (HR-QOL) in patients receiving combined RT with and without ADT 

Methods: The study population comprised patients treated with definitive RT for prostate cancer who completed the Expanded Prostate Cancer Index Composite-26 form between 3 and 24 months after completing RT. Covariance and a stepwise backward logistic regression model was used. 

Results: Data were available for 143 patients who received RT+ADT and 70 who received RT alone. The sexual function and hormonal vitality scores of patients receiving RT+ADT were significantly lower than those receiving RT alone (P < 0.0001). Patients with only compulsory school education had significantly lower sexual function scores than patients with university level education (P ≤ 0.005). Patients with depression had significantly lower hormonal vitality scores than those without depression (P ≤ 0.0001). 

Conclusions: The addition of ADT to RT is responsible for decrements in quality of life in the sexual and hormonal vitality domains, which is further compounded by lack of education and depression. This underlines the need to improve education, identify and treat depression, and develop strategies to improve the quality of life of patients receiving combination therapy. 

 

Background: Left cardiac sympathetic denervation (LCSD) was reported to be effective in patients with intractable ryanodine receptor mutation-associated catecholaminergic polymorphic ventricular tachycardia (CPVT). 

Objectives: To report our experience with LCSD in calsequestrin (CASQ2) mutation-associated CPVT. 

Methods: LCSD was performed in three patients with CASQ2 mutation-associated CPVT with symptoms and exercise-induced ventricular arrhythmia despite high dose beta-blocker. 

Results: None of them experienced symptoms or exercise-induced ventricular arrhythmia after LCSD. However, all had recurrence of symptoms and/or exercise-induced ventricular arrhythmia after 6 months (6–18 months).

Conclusions: LCSD conferred short-term suppression but less than optimal long-term suppression of exercise-induced ventricular arrhythmia among CASQ2-associated CPVT patients.

 

Background: Trauma patients diagnosed with pancreatic duct injury (PDI) have a high complication rate and prolonged hospital stay. The role of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of PDI remains unclear. During the last decade, our trauma unit incorporated ERCP into the management protocol for suspected PDI cases. 

Objectives: To determine whether ERCP is a sensitive tool to detect PDI. 

Methods: This retrospective trauma patient series study assessed the diagnostic yield of ERCP in trauma cases with suspected PDI on computed tomography (CT) or intraoperatively. Between 1 January 2004 and 31 December 2011, 13 patients admitted to our medical center underwent ERCP for suspected PDI. Patient demographics, mechanism of injury, Injury Severity Score (ISS), time from injury to ERCP, and ERCP-related complications were documented and assessed. 

Results: Of the 13 patients included in the analysis, 8 stable patients with suspected PDI on CT had no leak from the main pancreatic duct on ERCP. Two of them underwent surgery for suspected pancreatic transection. ERCP confirmed a main pancreatic duct leak in three patients. Two patients underwent ERCP for suspected PDI after “damage control” surgery. No leak from the pancreatic ducts was diagnosed. No pancreas-related complications or ERCP-related complications were observed.

Conclusions: ERCP is a sensitive and relatively safe tool for the diagnosis of PDI, and its use might prevent unnecessary surgical interventions in selected trauma cases.

 

Fungal diseases affect a large proportion of the population, ranging in severity from mild superficial infections to life-threatening invasive diseases. Estimates of disease burden are needed to inform public health policies. We estimated the incidence of fungal diseases in Israel based on published surveillance data and risk-based calculations using previously established models. Deaths associated with fungal diseases were estimated from local surveillance data (candidemia) and published reports (invasive aspergillosis). Candidemia was estimated in 649 persons/year and invasive aspergillosis in 254 persons/year; the associated mortality was 2.75 and 0.96 per 100,000 population/year, respectively. Recurrent Candida vulvovaginitis occurs in 130,440 women annually. National incidence rates of cryptococcosis, pneumocystis pneumonia and mucormycosis could not be reliably estimated. Single-center data-derived estimates yielded 24, 26 and 20 cases/year, respectively. Allergic bronchopulmonary aspergillosis, asthma with fungal sensitization and allergic fungal sinusitis affect 8297 (range 2323–11,615), 14,372 (14,372–17,965) and 39,922 (15,969–183,643) persons, respectively. In Israel, candidemia and invasive aspergillosis rank high among infection-related causes of mortality. Allergic fungal diseases cause chronic or recurrent symptoms in a large population and may contribute to asthma-related hospitalization and death. These general estimates should serve as a primer for future efforts to study fungal epidemiology.