Extra-intestinal manifestations of Crohn's disease

Extra-intestinal manifestations occur in at least 25% of Crohn's disease patients. Some extra-intestinal manifestations, such as erythema nodusum and peripheral arthropathy, will wax and wane in keeping with bowel inflammation. The more severe cutaneous ulcerations, uveitis, and axial arthropathy may precede bowel disease or persist after it subsides. Screening may be appropriate for eye disease and for osteoporosis to prevent complications. Medical management for extra-intestinal manifestations is similar to treatment for the bowel symptoms of Crohn's disease, with corticosteroids the mainstay. Pain and depression are associated with inflammatory bowel disease, and their control benefits patients. Recent small studies with anti-tumor necrosis factor (TNF) agents are promising for most extra-intestinal manifestations of Crohn's disease, and may permit more steroid-sparing disease control in the future.     

Hepatobiliary Manifestations of Inflammatory Bowel Disease

Hepatobiliary manifestations occur quite frequently in patients suffering from chronic ulcerative colitis and Crohn''s disease and carry with them considerable morbidity and mortality. Although the true incidence is difficult to determine, clinically, significant hepatobiliary disease occurs in 5%–10% of patients. At the present moment, the aetiology and pathogenesis of inflammatory bowel disease and its systemic manifestations remains speculative. For those hepatobiliary manifestations that respond to therapy of the underlying bowel disease, medical and/or surgical therapy must be aggressively pursued. More urgent research is required towards understanding the underlying cause(s) of the primary bowel disease and its systemic manifestations in order to improve the overall management of this condition.     

Pedal manifestations of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease with numerous pedal manifestations. Since pedal manifestations are often its first presentation, it is important that the podiatrist knows how to recognize the disease. This article discusses the manifestations, diagnosis, and treatment of SLE as they relate to podiatry.     

Respiratory involvement in the gastroesophageal reflux disesease

The pathological gastroesophageal reflux disease may lead to the gastroesophageal reflux disease (GERD), manifested as a spectrum of conditions including erosive esophagitis, Barrett esophagus and has been linked to the development of adenocarcinoma of the esophagus. The gastroesophageal reflux has been incriminated in the occurence of a number of pulmonary symptoms and diseases, otolaryngologic symptoms, and other extraesophageal manifestations. Clinicians must be aware of the possibility of some extraesophageal reflux-related manifestations, even in the absence of heartburn and acid reflux, classic esophageal symptoms of GERD. Although the correlation between gastroesophageal reflux disease and the extraesophageal manifestations has been established, a cause-and-effect relationship has not been proved yet. In this article, we present the respiratory manifestations of gastroesophageal reflux disease, referring to epidemiology, pathogenesis, diagnosis and treatment.     

Intestinal tuberculosis, Crohn's disease and ulcerative colitis in an urban black population

The clinical, epidemiological and pathological manifestations of intestinal tuberculosis, Crohn's disease and ulcerative colitis in the Johannesburg Black population are reviewed. Crohn's disease and particularly ulcerative colitis have emerged as definite disease entities in urban Blacks. There is an evident similarity between the clinical manifestations of intestinal tuberculosis and of Crohn's disease, and also between those of Crohn's disease and ulcerative colitis. Furthermore the data suggest that genetic and environmental factors play a vital role in susceptibility to these diseases. The similarity between the clinicopathological manifestations of intestinal tuberculosis and those of Crohn's disease lends support to the view that microbial, viral or cellular toxic agents are involved in the aetiology of Crohn's disease. Moreover, the clinical and epidemiological features of ulcerative colitis support the concept that environmental factors present in an urbanized milieu are important in its aetiology.     

Cerebral aneurysms in Beh?et's disease: a case report.

A 42-year-old male patient was surgically treated for two cerebral aneurysms, 18 months later he developed manifestations of Beh?et's disease. Lack of clinical manifestations as well as angiographic evidence of vasculitis at the time of the cerebral aneurysmal disease argue against the possibility that the aneurysms were complications of the neural involvement of Beh?et's disease.     

Neuropsychiatric systemic lupus erythematosus

Opinion statement The treatment of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) can be difficult and complex owing to the variety of nervous system manifestations that can occur, which include peripheral nerve disease, headaches, seizures, cerebrovascular disease, chorea, transverse myelitis, and psychiatric and cognitive disorders. Many of these manifestations can result from metabolic abnormalities or infection or as side effects of medications. Thus, in any patient with suspected NPSLE, it is crucial to exclude secondary causes of the presenting symptoms before assuming that they are due to NPSLE. It is especially important to exclude infection because this is a common cause of both morbidity and mortality in patients with systemic lupus erythematosus (SLE). Symptoms such as anxiety and depression may or may not be related to disease activity. Treatment decisions are based on accurate diagnosis of the specific NPSLE manifestation, which is usually made using tools such as brain imaging, electroencephalography, cerebrospinal fluid analysis, nerve conduction studies, or special serologic tests (eg, determination of antiphospholipid or antiribosomal P antibody levels). It is also important to assess the degree of other SLEmediated systemic disease activity in a patient with neurologic manifestations to determine if activation of systemic disease activity is also occurring. This is done by measuring complement levels, anti-double-stranded DNA levels, complete blood count, and urinalysis. For some NPSLE manifestations (eg, infrequent seizures, headaches, depression, anxiety, or peripheral neuropathy) that appear without activation of systemic disease, symptomatic treatment is appropriate. For others (eg, psychosis, delirium, or transverse myelopathy without other obvious cause), treatment with high-dose glucocorticoids with or without cyclophosphamide is appropriate whether there is evidence of other systemic disease activity or not. In general, the activity and severity of the leading organ manifestations dictate pharmacologic treatment.     

Isolated brachial artery aneurysm: a rare presentation of paediatric Behçet’s disease

Behçet’s disease is a rare disease characterised by recurrent oral ulcers, with systemic manifestations including genital ulcers, ocular disease, skin lesions, gastrointestinal disease, neurologic disease, vascular disease and arthritis. Most clinical manifestations of Behçet’s disease are believed to be due to vasculitis. The heterogeneous clinical spectrum is influenced by sex, ethnicity and country of residence. Vascular manifestation in the form of isolated large brachial artery aneurysm is rare in children. Treatment involves aneurysmorrhaphy to avoid rupture or ischaemic sequelae in addition to lifelong medical management to control vasculitis.     

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multisystemic inflammatory rheumatic disease of unknown etiology. It is understood that immune complexes have dominant role in the pathogenesis of the disease with vasculitis being the basis of many clinical manifestations. SLE is considered to be the autoimmune disease because of many autoantibodies, some of them with the known pathogenetic role. Characteristic features of the disease are skin manifestations, arthritis, serositis as well as various tissue and organ lesions, particularly that of the kidney and nervous system. On the basis of disease activity and organ involvement the decision on the institution of immunosuppressive therapy is made.     

Unexplained polyarthralgia and celiac disease

Celiac disease is an immunological disorder whose best-known manifestations are gastrointestinal symptoms. However, early joint manifestations are common and frequently overlooked features of celiac disease. We report a case in which unexplained inflammatory polyarthralgia and iron-deficiency anemia led to the diagnosis of celiac disease. Autoimmune thyroiditis was also a feature. Early diagnosis and treatment of celiac disease protect patients against complications such as digestive neoplasis. A simple and rapid tool for achieving the early diagnosis is the measurement of the serum of anti-gliadin, anti-endomysial and anti-tissue transglutaminase antibodies. However, a duodenal biopsy remains the only means of making the definitive diagnosis of celiac disease.     

Manifestations digestives et ischémie mésentérique au cours des vascularites systémiques

Primary systemic vasculitides are rare and primarily classified according to the size of the vessels predominantly affected. Secondary vasculitides can be caused by infection or drug or develop as a complication of cancer or other systemic disease like rheumatoid arthritis. Gastrointestinal manifestations occur at variable frequencies in vasculitis patients and may reveal the disease (15% of the patients). Abdominal pain, nausea, vomiting, and diarrheas are common, but non specific, manifestations. Mucosal inflammation, purpuric infiltrate or necrosis and ulceration (possibly with a granulomatous appearance) can also be observed. Gastrointestinal ischemia, bowel infarction, intestinal perforation, especially of the small bowel, pancreatitis, and gastrointestinal bleeding are more severe manifestations. Prompt and combined medical and, when necessary, surgical management is mandatory. Ten-year survival rate of patients with primary systemic necrotizing vasculitides is now about 80% in the absence of severe visceral involvement or in case of only minor gastrointestinal manifestations. Conversely, at 5 years, survival rate is only 56% for patients with severe and surgical gastrointestinal manifestations.     

Treatment concepts in osseous manifestations of Langerhans cell histiocytosis]

INTRODUCTION: Patients with Langerhans Cell Histiocytosis (LCH or Eosinophilic granuloma) were assessed from the orthopaedic point of view to give recommendations for the management of the disease. MATERIAL AND METHODS: The results of 36 cases of histologically proven bony manifestations out of 48 treated cases were reviewed. A retrospective analysis of our treated cases with bony manifestations of LCH between 1970 and 1995 was performed. RESULTS: Twenty-two cases exhibited isolated bony manifestations, 18 were monoostotic and 4 were polyostotic. We treated 14 cases with multi-organ disease including bony manifestations of LCH. In the cases of exclusive bony manifestations reactivations were rare and usually occurred in other bones. CONCLUSIONS: In order to assure stability local control is the general goal of orthopaedic treatment. In isolated lesions control can be achieved by excochleation and filling with cancellous bone or prednisolon instillation. Multiple lesions should be treated primarily by systemic drugs and operative procedures are only necessary if severe local problems occur. Additionally, we recommend interdisciplinary cooperation between ortopedic surgeon, pediatrist and pathologist.     

Paraneoplastische Syndrome des Nierenzellkarzinoms

Renal cell carcinoma associated paraneoplastic symptoms include constitutional symptoms as well as specific metabolic and biochemical abnormalities. These are present in up to 40% of patients with renal cell carcinoma during the course of the disease. This report provides information on the most common manifestations and their therapy; some rare variants are also mentioned. The importance of paraneoplasia lies partly in the fact that paraneoplastic symptoms may be the precursor of either primary or recurrent disease. The presence of paraneoplastic manifestations does not necessarily imply a poor prognosis or metastatic disease.     

Manifestations articulaires des affections intestinales

Inflammatory enterocolonopathies or intestine chronic inflammatory diseases refer to both Crohn's disease and haemorrhagic rectocolitis. They are part of the spondylarthropathies group. Osteoarticular lesions occur in 1/3 of affected patients; they are the most frequent extra-digestive manifestations of these diseases and may reveal the intestinal affection. The frequency of osteopenia and osteoporosis imposes early detection and adapted management. Arthralgias or non destructive asymmetric pauciarticular arthritis often occur together with enterocolonopathy-induced digestive crises. Frequently, they are associated with other extra-intestinal manifestations. Sacro-iliitis may occur as an isolated event. About 6% of patients with an intestine chronic inflammatory disease have also a real spondylarthropathy as defined by Amor's criteria or the European criteria. The clinical manifestations occur independently from the digestive crises. When managing a spondylarthropathy, systematic screening for inflammatory enterocolonopathy should be undertaken. Whipple's disease is a curable chronic bacterial infection with a multisystemic pattern, due to Tropheryma whipplei. It generally begins with a recurrent joint lesion in a middle-aged man, followed some years later by emaciation and diarrhoea diversely associated with other clinical manifestations. Arthralgia, and even real polyarthritis and tenosynovitis may be encountered when managing intestinal bypass, celiac disease, and microscopic colitis.     

Musculoskeletal manifestations of Lyme disease in children

Lyme disease, caused by a tick-transmitted spirochete, has significant musculoskeletal manifestations in children as well as in adults. A series of 23 children with Lyme disease is examined. Acute arthritis was present in five cases, with the knee being involved in three of these cases. Twelve cases (52%) presented with arthralgias, and 10 cases (43%) presented with myalgias. Neurological manifestations were present in 39% of the cases. Five cases have demonstrated recurrent polyarthralgias. Lyme disease should be considered in the differential diagnosis of arthralgias and arthritis in children.     

Anesthetic management of the parturient with ankylosing spondylitis

Pregnancy may occur in patients with ankylosing spondylitis, a chronic inflammatory joint disease. This disease, which is included in the group of seronegative spondylarthropathies, may be characterized by both intra- and extra-articular manifestations. Although most pregnant patients with ankylosing spondylitis experience normal spontaneous vaginal deliveries, manifestations of the disease may interfere with labor and delivery as well as the administration of general and regional anesthesia. Therefore, physicians caring for a pregnant patient with ankylosing spondylitis should be aware of the obstetric and anesthetic implications and the ramifications of active disease. These patients should be referred to an anesthesiologist early in pregnancy so that the obstetrician and anesthesiologist can together formulate a plan.     

Surgical management of plantar von Recklinghausen neurofibroma

Clinical manifestations of von Recklinghausen's disease on the plantar aspect are very rare. Most of the neurofibromas involving the skin are asymptomatic, but when they occupy an unusual position or attain a large size, they lead to significant disability. A case report and the surgical treatment of a 39-year-old female with the pathognomonic clinical characteristics, along with a symptomatic plantar neurofibroma, are presented. The pathogenesis, clinical manifestations, diagnosis, complications, and treatment of this progressive disease are described in this report.     

The role of the ET nurse in managing Gardner's syndrome

Gardner's syndrome is a genetic disease that carries a 100% risk of malignant degeneration if left untreated. The syndrome is characterized by the presence of numerous colon polyps, along with a wide variety of extracolonic manifestations. The presence of these extracolonic manifestations increase morbidity and make treatment more difficult. The surgical procedure commonly used to manage the disease is total colectomy with creation of an ileoanal reservoir. The incidences of intestinal obstruction, genitourinary obstruction, septicemia, and fistula formation increase with the growth of desmoid tumors, which are one of the extracolonic manifestations of the disease. The ET nurse plays an essential role in the care of the patient with Gardner's syndrome. Whether acting as a consultant or primary care provider, the ET nurse provides emotional support, educates, and establishes a plan of care to manage the ostomies, skin problems, complex wounds, and fistulas associated with Gardner's syndrome.     

Von Hippel-Lindau disease simulating polycystic kidney disease

Polycystic kidney disease and the renal manifestations of von Hippel-Lindau disease have much in common. Making the distinction between these two diseases is important. There is a strong association of renal cell carcinoma with von Hippel-Lindau disease, whereas renal cell carcinoma is rare in polycystic kidney disease. Furthermore, the many extrarenal manifestations of von Hippel-Lindau disease are serious and can be fatal while those of polycystic kidney disease are generally benign. Early diagnosis of the lesions of von Hippel-Lindau disease could lead to effective surgical treatment and prevent death. A case of von Hippel-Lindau disease is presented which was incorrectly diagnosed as polycystic kidney disease for sixteen years. The case is instructive in that the possibility of making the correct diagnosis prior to the patient's terminal illness was only through careful assessment of the family. The case is also remarkable in that the patient suffered from progressive renal failure requiring hemodialysis, which has not been associated previously with von Hippel-Lindau disease.     

The epidemiology, pathophysiology, and natural history of chronic venous disease

Chronic venous disease includes a spectrum of clinical presentations ranging from varicose veins through chronic lower extremity pain and edema to venous skin changes and ulceration. Such manifestations may result from primary venous insufficiency or be secondary to other disorders, primarily acute deep venous thrombosis. Regardless of etiology, chronic venous disease has significant socioeconomic consequences and is among the most common problems encountered in surgical practice. Ambulatory venous hypertension underlies most of the sequelae of chronic venous disease, although such hemodynamic derangements may result from either valvular incompetence or venous obstruction. Unfortunately, the factors responsible for the progression of disease from mild to severe manifestations are only beginning to be understood. However, a thorough understanding of the pathophysiology and natural history of chronic venous disease is essential in its management.