Israel Medical Association Journal

Lower extremity ulcers are a late complication of connective tissue diseases and occur more commonly in patients with this disease than in the general population. Although these lesions have historically been attributed to vasculitis, it is now recognized that inflammatory vessel injury accounts for fewer than 20% of ulcers in connective tissue disease. The pathogenesis of these lesions is complex, and often several processes act synergistically to initiate and perpetuate tissue injury. We review the evidence for antiphospholipid antibodies and prothrombotic states contributing to a vasculopathy in patients with connective tissue disease, precipitating ulceration and impairing healing.

Background Drug-specific CD8+ TH1 lymphocytes have been found in the peripheral blood and involved skin of patients with drug-induced bullous exanthems.

Objectives To determine whether the interferon-gamma release test can identify culprit drugs in pemphigus patients.

Methods Clinical and laboratory workup for pemphigus was performed in 14 pemphigus vulgaris patients who had been exposed to drugs, and the IFNl[1] release test was conducted on their lymphocytes from heparinized venous blood cultured with medium, phytohemagglutinin and one of 32 drugs, or medium and phytohemagglutinin alone.

Results Ten of the patients and 13 of the 32 drugs exhibited a positive response to the test. Eight of the 10 patients with positive IFNl test results had a less severe course of the disease, with fast reduction in steroid dosage.

Conclusions The findings demonstrate both the ability of the IFNl release test to identify drugs that can induce pemphigus, and its usefulness in the diagnostic workup of pemphigus patients.

Background: Dupuytren’s disease is a fibroproliferative disorder of the palmar fascia that can cause disabling digital contractures. The pathogensis of the disease is still unclear, and it afflicts predominantly white males of northern European origin. Gender-related differences of Dupuytren's disease and the distinctive characteristics of the disease in females are not yet well defined.

Objectives: To evaluate and illustrate the distinctive characteristics of Dupuytren's disease in females.

Methods: A retrospective study was performed of all female patients with Dupuytren’s disease seen and followed at our Hand Surgery Unit over a 20 year period. The study group consisted of 48 women (56 hands). The collected data included clinical and epidemiological features on admission, and outcome of surgical intervention.

Results: Of the 48 women (56 hands) with Dupuytren's disease, 23 (26 hands) underwent limited fasciectomy. The average age at presentation was 60.1 years. A few of the patients originated from Asia and Africa. Manifestations and pattern of the disease were nearly comparable to those observed in the male group, except for a slightly higher incidence of proximal interphalangeal joint contracture in female patients. Generally, females expressed less severe contractures on presentation, and a slow progression thereafter. A favorable functional postoperative outcome was observed. Seven patients had minor complications including local hematoma and painful scars. Two patients developed moderate signs of complex regional pain syndrome.

Conclusions: Further investigations are needed to assess the potential role of androgens in the pathogenesis of Dupuytren's disease, and a possible protective role of estrogenic hormones, rendering Dupuytren’s contracture a postmenopausal affliction.
 

Neonatal lupus erythematosus is an uncommon transplacentally Acquired Autoimmune Disorder. The most common clinical manifestations are skin rash, congenital atrioventricular block, thrombocytopenia, leucopenia, anemia, and hepatosplenomegaly. Usually, the skin rash resembles subacute cutaneous lupus, but different forms of rash have been reported in Neonatal lupus erythematosus and some are rare forms. NLE should be suspected in babies with atypical skin lesions, even if present at birth.  

Chronic disease management has been a rapidly growing entity in the 21st century as a strategy for managing chronic illnesses in large populations. However, experience has shown that disease management programs have not been able to demonstrate their financial value. The objectives of disease management programs are to create quality benchmarks, such as principles and guidelines, and to establish a uniform set of metrics and a standardized methodology for evaluating them. In order to illuminate the essence of disease management and its components, as well as the complexity and the problematic nature of performing economic calculations of their profitability and value, we collected data from several reports that dealt with the economic intervention of disease management programs. The disease management economic evaluation is composed of a series of steps, including the following major categories: Data/information technology, information generation, assessment/recommendations, actionable customer plans, and program assessment/reassessment. We demonstrate the elements necessary for economic analysis. Disease management is one of the most innovative tools in the managed care environment and is still in the process of being defined. Therefore, objectives should include the creation of quality benchmarks, such as principles and guidelines, and the establishment of a uniform set of metrics and a standardized methodology for evaluating them.

Background: Idiopathic frozen shoulder is a self-limiting regional skeletal problem of unknown etiology. Clinically, patients first experience a phase of pain, progressing to a freezing stage when glenohumeral motion is lost, followed by a thawing phase when pain gradually subsides and most of the lost motion returns.

Objectives: To identify possible specific and non-specific risk factors for idiopathic frozen shoulder.

Methods: We compared the medical histories, drug treatment, previous hospital as well as health management organization blood tests of 126 new consecutive frozen shoulder patients from a shoulder clinic to those of an age-matched control group of 98 consecutive patients from an orthopedic foot and ankle clinic and to the regional population disease prevalence registry. Frozen shoulder was classified as idiopathic only if there was no history of trauma and no evidence of a rotator cuff tear.

Results: Among the frozen shoulder patients 29.4% had diabetes and 13.5% had thyroid disorders. The risk ratio for diabetes in the frozen shoulder group was 5.9 for males (95% confidence interval 4.1–8.4, P < 0.001) and 5.0 for females (95% CI[1] 3.3–7.5, P < 0.001). The risk ratio for thyroid disorders among females with frozen shoulder was 7.3 (95% CI 4.8–11.1, P = 0.001). No significant difference was found in the prevalence of thyroid disorders between frozen shoulder and the control group, but there was a significantly higher prevalence of diabetes in males and a trend for higher prevalence in females in the frozen shoulder group.

Conclusions: Physicians should be aware that diabetes is a specific risk factor for idiopathic frozen shoulder in both males and females and thyroid disorders are a non-specific risk factor in females only.  

Background: Infliximab and etanercept have been included in the Israeli national list of health services since 2002 for rheumatoid arthritis and juvenile idiopathic arthritis, and since 2005 for psoriatic arthritis and ankylosing spondylitis. The regulator (Ministry of Health and health funds) mandates using fixed doses of infliximab as the first drug of choice and increased dosage is not allowed. For other indications (e.g., vasculitis), anti-tumor necrosis factor therapy is given on a "compassionate" basis in severe refractory disease.

Objectives: To describe our experience with anti-TNF[1] therapy in a single tertiary referral center in northern Israel and to analyze the impact of the national health policy on the results.

Methods: We reviewed the medical records of patients who received anti-TNF therapy in our institution, and analyzed demographic data, diagnosis, clinical and laboratory features, previous and current therapies, and anti-TNF treatment duration and side effects.

Results: Between 2001 and 2006, 200 patients received anti-TNF therapy for rheumatoid arthritis (n=108), juvenile idiopathic arthritis (n=11), psoriatic arthritis (n=37), ankylosing spondylitis (n=29), adult Still's disease (n=4), overlap disease (RA[2] and scleroderma or polymyositis, n=6), temporal arteritis (n=1), polyarteritis nodosa (n=1), dermatomyositis (n=1), amyloidosis secondary to RA (n=1) and Wegener's granulomatosis (n=1). Forty percent of RA patients discontinued the first anti-TNF agent due to side effects or insufficient response. Higher sedimentation rate and lower or negative rheumatoid factor predicted better response to therapy among RA patients. AS[3] and PS[4] patients had a better safety and efficacy profile. Severe infections occurred in 2% of patients. All eight patients who presented lung involvement as part of their primary rheumatic disease remained stable or improved. A significant improvement was achieved in all six patients with overlap disease.

Conclusion: Our daily practice data are generally in agreement with worldwide experience. The ‘deviations’ might be explained by the local health policy at that time. The impact of health policy and economic and administrative constraints should be taken into account when analyzing cohort daily practice data.

Capsule endoscopy was launched at the beginning of this millennium and has since become a well‑established tool for evaluating the entire small bowel for manifold pathologies. CE[1] far exceeded our early expectations by providing us with a tool to establish the correct diagnosis for such elusive gastrointestinal conditions as obscure gastrointestinal bleeding, Crohn's disease, polyposis syndrome and others. Recent evidence has shown CE to be superior to other imaging modalities – such as small bowel follow‑through X-ray, colonoscopy with ileoscopy, computerized tomographic enterography, magnetic resonance enteroclysis and push enteroscopy – for diagnosing small bowel pathologies. Since the emergence of CE, more than 500,000 capsules have been swallowed worldwide, and more than 700 peer-reviewed publications have appeared in the literature. This review summarizes the essential data that emerged from these studies.