The colon in the pseudoobstructive syndrome

Colonic pseudoobstruction can occur as part of a generalized chronic intestinal pseudoobstruction syndrome or as an isolated entity. Isolated colonic pseudoobstruction can occur in two unrelated forms: the acute and chronic forms. Acute colonic pseudoobstruction is frequently a hospital-acquired disease that arises as a complication of other illnesses. The syndrome must be recognized and treated with early colonoscopic decompression to prevent cecal or colonic perforation. Chronic colonic pseudoobstruction is a syndrome of many causes. The prognosis of patients with chronic colonic pseudoobstruction is much better than that of generalized chronic intestinal pseudoobstruction, because the patients become asymptomatic with appropriate operations. The pathogenesis of acute colonic pseudoobstruction and several types of chronic colonic pseudoobstruction is not known. Further investigations should include bacteriologic study, histopathologic studies (examinations of smooth muscle and myenteric plexus), and examination of extrinsic nerves of the colon. With these approaches, a better understanding of the pathogenesis of these syndromes will be achieved.     

Pain syndromes, disability, and chronic disease in childhood.

Childhood disability and chronic disease are common, and their prevalence is increasing as children survive with conditions that were previously fatal. It is important that physicians in training learn about disability and handicap, and the functioning of multidisciplinary teams to manage these problems. Chronic ill-health is often very expensive to manage, and some serious and creative thinking about the best way to fund such health care is urgently needed. Pediatric rheumatologists are involved with the care of many children with chronic and recurrent musculoskeletal pain; however, they have not perhaps focused enough research effort on the investigation of pain and its management. Whether reflex neurovascular dystrophy, fibromyalgia, and chronic fatigue syndrome are part of a disease continuum is unclear, but it seems probable that psychosocial problems are often important contributing factors in all three conditions. Immunoglobulin subclass deficiencies are being increasingly delineated, occurring in chronic fatigue syndrome as well as many other disease states. Their clinical relevance still remains, for the most part, uncertain. Short stature occurs in many chronic illnesses, and the role of growth hormone treatment in these conditions is beginning to be investigated.     

Geriatric assessment for the rheumatologist

The population of industrialized countries is aging rapidly. Rheumatologic disorders are the most common illnesses causing functional disability in the elderly and medications for rheumatologic disorders are among the most frequently prescribed. As the population of patients seen by rheumatologists ages, it is important for the rheumatologist to understand the techniques of geriatric assessment that are useful in understanding and treating this special population. This article reviews practical ways to assess function, cognition, affect, directives, and end-of-life care in elderly populations. The article concludes with a "Top Ten" list of geriatric principles for rheumatology fellows to learn.     

Nonatherosclerotic Obstructive Vascular Diseases of the Mesenteric and Renal Arteries

Nonatherosclerotic vascular diseases of the mesenteric and renal arteries are considered to occur less frequently than those caused by occlusive atherosclerotic disease. However, when present, they pose a significant diagnostic and therapeutic challenge. Such disorders include fibromuscular dysplasia, median arcuate ligament syndrome, the renal nutcracker syndrome, and some forms of acute and chronic mesenteric ischemia (embolic and thrombotic). This is a heterogeneous group of disorders with substantial differences in the pathogenesis and diagnostic approaches to these diseases. We provide an overview of the pathogenesis, clinical presentation, diagnosis, and current management of fibromuscular dysplasia, median arcuate ligament syndrome, and the renal nutcracker syndrome.     

Nontuberculous mycobacteria in women,young and old

Lady Windermere syndrome is a unique entity within the spectrum of pulmonary NTM diseases. There are differences in several clinical aspects between Lady Windermere syndrome and the classic pulmonary NTM disease, including manifestations, pathogenesis, and natural history. Recently, emerging pieces of information provide a more scientific explanation of why women are more susceptible to this form of infection and how they develop clinical disease. As the result, these patients probably require quite different diagnostic and therapeutic approaches compared with those with the classic presentation. Studies exclusive to LWS are lacking and are absolutely necessary as they will enhance our understanding of, and hence successful management strategies for, this increasingly recognized disease.     

The biopsychosocial model—a tool for rheumatologists

Rheumatologists grapple in daily practice with many controversial syndromes including fibromyalgia, late whiplash syndrome, chronic fatigue syndrome, Gulf War syndrome, the adverse outcomes of silicon breast implants and so on. For decades, much of the debate surrounding, and the approach to these controversial syndromes has centred on a model creating two camps–organic versus non-organic. While each camp has its support, this model seems to have failed in achieving the desired understanding of these syndromes, most particularly in offering the rheumatologist a practical and coherent approach to effective treatment. This chapter will thus introduce the biopsychosocial model, its elements, its advantages over the traditional model and the practical application of this model. Examples will be given of how rheumatologists can approach the treatment of these syndromes through patient education and the implementation of a change in illness behaviour.     

The biopsychosocial model--a tool for rheumatologists.

Rheumatologists grapple in daily practice with many controversial syndromes including fibromyalgia, late whiplash syndrome, chronic fatigue syndrome, Gulf War syndrome, the adverse outcomes of silicon breast implants and so on. For decades, much of the debate surrounding, and the approach to these controversial syndromes has centred on a model creating two camps-organic versus non-organic. While each camp has its support, this model seems to have failed in achieving the desired understanding of these syndromes, most particularly in offering the rheumatologist a practical and coherent approach to effective treatment. This chapter will thus introduce the biopsychosocial model, its elements, its advantages over the traditional model and the practical application of this model. Examples will be given of how rheumatologists can approach the treatment of these syndromes through patient education and the implementation of a change in illness behaviour.     

Complementary and alternative therapies for fibromyalgia

Fibromyalgia (FM) is a syndrome of chronic widespread musculoskeletal pain that is accompanied by sleep disturbance and fatigue. Clinical treatment usually includes lifestyle modifications and pharmacologic interventions meant to relieve pain, improve sleep quality, and treat mood disorders. These therapies are often ineffective or have been shown in clinical studies to have only short-term effectiveness. Pharmacologic treatments have considerable side effects. Patients may have difficulty complying with exercise-based treatments. Thus, patients seek alternative therapeutic approaches and physicians are routinely asked for advice about these treatments. This article reviews nontraditional treatment alternatives, from use of nutritional and herbal supplements to acupuncture and mindbody therapy. Little is known about efficacy and tolerance of complementary and alternative therapies in FM and other chronic musculoskeletal pain syndromes. Most studies on these treatments have been performed for osteoarthritis, rheumatoid arthritis, or focal musculoskeletal conditions. Clinical trials are scarce; the quality of these trials is often criticized because of small study population size, lack of appropriate control interventions, poor compliance, or short duration of follow-up. However, because of widespread and growing use of alternative medicine, especially by persons with chronic illnesses, it is essential to review efficacy and adverse effects of complementary and alternative therapies.     

Dyspepsia and its overlap with irritable bowel syndrome

Patients with functional dyspepsia and the irritable bowel syndrome are commonly seen in both primary care and gastroenterology subspecialty settings. Although functional dyspepsia and the irritable bowel syndrome can occur separately, recent research suggests that they often appear together as an overlap syndrome and thus may represent different portions of a unifying spectrum of disease. Despite their widespread prevalence, the pathogenesis of these disorders is not well established but may include impaired gastric emptying, gastric dysrhythmias, hypersensitivity (to acid exposure and to stretch), and Helicobacter pylori infection. Once other disorders in the differential diagnosis have been excluded, treatment of patients with functional dyspepsia, irritable bowel syndrome, and the overlap syndrome without alarm signs underscores current prevailing pathophysiologies and is generally empiric and symptom based. It is hoped that management of these disorders will become more targeted and efficacious as our understanding of them improves.     

Eosinophilic disorders: molecular pathogenesis, new classification, and modern therapy

Before the 1990s, lack of evidence for a reactive cause of hypereosinophilia or chronic eosinophilic leukemia (e.g. presence of a clonal cytogenetic abnormality or increased blood or bone marrow blasts) resulted in diagnosticians characterizing such nebulous cases as 'idiopathic hypereosinophilic syndrome (HES)'. However, over the last decade, significant advances in our understanding of the molecular pathophysiology of eosinophilic disorders have shifted an increasing proportion of cases from this idiopathic HES 'pool' to genetically defined eosinophilic diseases with recurrent molecular abnormalities. The majority of these genetic lesions result in constitutively activated fusion tyrosine kinases, the phenotypic consequence of which is an eosinophilia-associated myeloid disorder. Most notable among these is the recent discovery of the cryptic FIP1L1-PDGFRA gene fusion in karyotypically normal patients with systemic mast cell disease with eosinophilia or idiopathic HES, redefining these diseases as clonal eosinophilias. Rearrangements involving PDGFRA and PDGFRB in eosinophilic chronic myeloproliferative disorders, and of fibroblast growth factor receptor 1 (FGFR1) in the 8p11 stem cell myeloproliferative syndrome constitute additional examples of specific genetic alterations linked to clonal eosinophilia. The identification of populations of aberrant T-lymphocytes secreting eosinophilopoietic cytokines such as interleukin-5 establish a pathophysiologic basis for cases of lymphocyte-mediated hypereosinophilia. This recent revival in understanding the biologic basis of eosinophilic disorders has permitted more genetic specificity in the classification of these diseases, and has translated into successful therapeutic approaches with targeted agents such as imatinib mesylate and recombinant anti-IL-5 antibody.     

New criteria for diagnosing Sj?gren's syndrome: a step forward?--or...

Primary Sj?gren's syndrome seems to be the most common of the chronic systemic inflammatory connective tissue diseases, according to epidemiological investigations. Basic and clinical research, including treatment within this group, is increasing while simultaneously there is increasing confusion and uncertainty about the classification criteria for Sj?gren's syndrome. Within the last three decades nine different classification criteria sets have been introduced including the recent US-European classification criteria set. The diagnostic criteria used in daily practice seem to differ even more and many forget that the function of both the lachrymal glands and the salivary glands should be diminished. Consequently many rheumatologists prefer to diagnose patients according to classification criteria. The advantages and disadvantages of the various classification criteria for primary Sj?gren's syndrome are dealt with but it is stressed that the majority of these will exclude former/present smokers (from the diagnoses)--more than half of the population!     

Treatment of Comorbid Affective and Substance Use Disorders: Therapeutic Potential of Anticonvulsants

The authors examine the use of anticonvulsants/mood stabilizers to treat patients with substance use disorders. Although there is high comorbidity of bipolar and substance use disorders, there has been little research on the use of these medications to treat bipolar disorders in patients who also have a substance use disorders. However, symptoms of bipolar disorders, such as irritability and mood lability, may be difficult to distinguish from the effects of acute and chronic substance use; therefore, reliable diagnostic methods will need to be developed. Further, a new, hypothesized syndrome, Explosive Mood Disorder, is described that may be distinct from the bipolar spectrum. It is characterized by childhood onset of temper outbursts and irritable mood that persists into adolescence and adulthood, is connected to marijuana use, and responds to divalproex sodium. The authors review studies of prevalence, comorbidity, family history, longitudinal course, and placebo-controlled trials of anticonvulsant medications to evaluate the validity and treatment implications of this proposed entity.     

Evaluation and management of patients with chronic fatigue.

Chronic fatigue is a common and disabling problem in primary care practice. The differential diagnosis of chronic fatigue is extensive and includes medical disorders, altered physiologic states (eg, pregnancy, exertion), psychiatric disorders, lifestyle derangements, drugs, and controversial entities (eg, chronic candidiasis, food allergies, environmental illness, and chronic fatigue syndrome). The most common diagnoses are psychiatric disorders, including mood, anxiety, and somatoform disorders. A comprehensive approach to diagnosis and management is necessary, including structured psychiatric interviewing, functional assessment, and elicitation of the patient's diagnostic beliefs. Patients often believe they are suffering from an organic medical disorder (eg, viral or immunologic) and resist psychiatric labelling of their symptoms and referral to mental health practitioners. Establishing and maintaining rapport, having a flexible approach, and demonstrating a personal concern for the patient is essential. Drug therapy for specific psychiatric and medical illnesses and cognitive-behavioral approaches for enhancing coping mechanisms are effective.     

Rheumatological medicine literacy among Middle Eastern populations

Background: It has been observed for years that many Middle Eastern patients with autoimmune rheumatic disorders, are more likely to be delayed in seeing a rheumatologist for their symptoms and that the rheumatology services are in general under‐utilized by the population. Aim: To explore if patients with autoimmune rheumatic disorders were truly delayed in seeing rheumatologists and to explore the possible reasons for that delay should any delay be documented. Subjects and methods: Patients suffering from chronic autoimmune rheumatic disorders were interviewed and were asked to answer a questionnaire that assesses their initial set of actions when they had their first symptoms of disease, how much time they took to see a rheumatologist and their background knowledge about rheumatology as a specialty before and after they saw a rheumatologist. Results: Seventy‐eight patients, 57 (73%) females and 21 (27%) males were included in this study. Their ages ranged from 11 to 72 years with a mean of 38.9 ± 13 years. Patients’ explanations for their initial symptoms were ‘evil eye doing’, disease, exertion, cold weather and trauma in 44%, 37%, 20%, 16% and 8% respectively. Ninety‐six percent of patients had to make a total of 166 consultations first at other specialties before they were finally advised or directed to see a rheumatologist. Non‐rheumatologist referrals to rheumatologists happened in only 33% of the time. The duration from the onset of the disease until patients finally came to see a rheumatologist ranged from 0.5 weeks to 432 weeks with a mean of 51 ± 88 weeks. Conclusions: General health literacy and knowledge of the rheumatology scope of service is extremely limited among Middle Eastern patients. Most patients with autoimmune rheumatic diseases make their initial consultations at clinics other than rheumatology clinics and non‐rheumatologists have been shown to consistently not refer patients with rheumatic diseases to rheumatologists. Wrong diagnosis is attributed to rheumatology symptoms by non‐rheumatologists 82% of the time. Level of education of patients, has no impact on their choice of the right specialty to be consulted for their disease symptoms.     

Heritable disorders of connective tissue and disability and chronic disease in childhood.

The heritable disorders of connective tissue encompass a broad range of diseases, many of which occur in childhood and present to the pediatric rheumatologist's attention. The underlying theme of these disorders is fragility of connective tissue components such as bone, cartilage, and blood vessels. Over the past few years, investigations of patients with the major forms of heritable disorders of connective tissue, osteogenesis imperfecta, Marfan syndrome, and Ehlers-Danlos syndrome have revealed genetic mutations of collagen genes as the cause of the diseases. Review of the newest genetic information in these three disorders is presented. The rheumatic disorders of childhood present a paradigm for the effects of chronic disease on children and families. New work, discussed in this review, continues to suggest changes in psychosocial adaptation in children with chronic rheumatic diseases and their families, but these data are conflicting. The development of arthritis impact measures may help workers in pediatric rheumatology care to have a broader and more in-depth understanding of the effect of this chronic disease on functioning, psychosocial development, and family and peer interactions.     

Primary mucociliary transport failure

Among the disorders associated with male infertility and chronic sinopulmonary infections, primary ciliary dyskinesia or cystic fibrosis is characterized by ciliary dysfunction or abnormality of mucus secretion. In addition, Young's syndrome differs from the former because of the absence of ultrastructural cilia disorders and from the latter because of normal sweat and pancreatic functions. However, a number of manifestations seen in these disorders appear to overlap each other, e.g., male infertility and chronic sinopulmonary infections which often develop bronchiectasis. Therefore, I would like to propose that the term 'muco(secretion)ciliary transport failure' is used for illnesses in patients with primary impairment of mucosecretion and/or ciliary transport in organs containing the mucociliary transport system. Primary mucociliary transport failure encompasses three hereditary disorders, that is, primary ciliary dyskinesia, cystic fibrosis and Young's syndrome. Ciliary activity is closely associated with mucus production. For a better understanding of the relationship between ciliary activity and mucus production, further basic and clinical studies should be attempted.     

Functional nausea and vomiting

Although functional vomiting (FV), cyclic vomiting syndrome (CVS) and chronic idiopathic nausea (CIN) are fairly rare disorders, it has been increasingly recognized that these conditions can be highly disabling. Traditionally, FV, CVS and CIN have been under-investigated; however, interest in the cause and treatment of these disorders has increased, particularly with regard to their pathophysiology and the evaluation of new treatment approaches. This article presents a literature-based review of the nomenclature, pathophysiology, clinical presentation and management of CIN, CVS and FV. There is a dearth of randomized, controlled trials of treatments for these disorders, owing mainly to their low prevalence. Consequently, referral centers that see a large number of patients with these challenging disorders are working together to share their experience, so that the most productive treatment strategies can be used to help patients. Our knowledge of the treatment of FV, CVS and CIN, as best we know it, is presented here.     

Awareness and knowledge of fibromyalgia among French rheumatologists and general practitioners

OBJECTIVES: Fibromyalgia is a chronic disorder characterized by widespread musculoskeletal pain and fatigue. Its prevalence is estimated to be at 3.4% in women and 0.5% in men. It is a major cause of morbidity. Our objective was to evaluate, using a self-questionnaire sent by mail, the level of knowledge of French physicians, general practitioners, and rheumatologists on fibromyalgia and to analyse their therapeutic approach. METHODS: The demographic characteristics of a sample of general practitioners and rheumatologists were compared to those of the overall data available. This comparison demonstrated the good representativeness of our sample. RESULTS: Fibromyalgia was considered as a disease by 23% of rheumatologists and 33% of general practitioners. While on average, each rheumatologist followed 30 fibromyalgia patients, each general practitioner followed 6.1 patients (i.e., 2 to 5% of their practice's patient base). Among rheumatologists, 6.4% made no distinction between this disease and depression vs. 13.1% of general practitioners. The diagnosis of fibromyalgia was made based on tenderness that occurs in precise, localized areas of the body (trigger points) by 94% of rheumatologists and 79.1% of general practitioners. Of general practitioners and rheumatologists, 93.7% and 73.7% respectively, have not received any medical school training on fibromyalgia or chronic fatigue syndrome. CONCLUSION: Given the lack of medical school training and continuing professional education concerning fibromyalgia (rare use of pain rating scales, confusion in the classification of rheumatic diseases), there is an urgent need to initiate an explicit teaching effort on chronic pain, and on fibromyalgia in particular.