Crohn’s disease: a patient’s perspective

Aim As healthcare providers for Crohn’s disease, we assume that we have a good understanding of the disease progression and its symptoms. The aim of this study was to gather information about what patients with Crohn’s disease think are relevant to their symptoms and what helps them cope with this lifelong benign disease. Materials and methods A questionnaire was sent to all patients with a diagnosis of Crohn’s disease seen in the Digestive Disease Center in the last 5 years. The returned forms were downloaded into a database and sent for analysis. Results Sixty-two percent of respondents were female. One third were between the ages of 35 and 50 years. Seventy percent were married. Thirty-eight percent had a graduate degree, 19% were unemployed. Fifty percent still smoked, half of them less than one pack a day. Sixty-eight percent said that their symptoms affected work, and one fourth changed jobs due to this. Foods worsened symptoms in 60%, with a decrease in symptoms while on low fiber foods and white meats. Lifestyle change worsened symptoms in 66%. A change in the caregiver was not a significant stressor. More than half used Remicade, with one third stating that it was helpful. Eight percent had never used steroids. Alcohol increased symptoms in 40%. Factors that did not cause a significant change were children at any age, pregnancy, menopause, and hormone replacement therapy. Surgery caused half the patients to improve for many years, although one third felt a lowered self-esteem postoperatively. Conclusion Patients with Crohn’s disease should be managed in a more comprehensive manner to provide optimal care. Thus, a team approach that includes a dietician and counselor should be considered as an integral part of this team. This will allow patients to have enhanced skills to cope with changes in their symptoms, whether they are due to the disease itself or the changes in their routine.     

Crohn’s disease complicated by adult-onset Still’s disease

A 31-year-old man with Crohns disease developed arthritis, spiking fever, and skin rash indistinguishable from that of adult-onset Stills disease. He was admitted to our hospital because of a periumbilical intestinal skin fistula. Crohns disease had been diagnosed in 1991, and had required intestinal resection twice, and schizophrenia had been diagnosed in 1993. He developed polyarthritis and spiking fever, accompanied by a macular skin rash on both forearms. Marked hepatosplenomegaly and bilateral pleural effusion were detected on computed tomography examination. These findings are indistinguishable from those of adult-onset Stills disease. Because his mental status had deteriorated following high-dose prednisolone on a previous admission, he was treated with an immunosuppressive agent on this occasion, with the treatment being successful. This is the first report of adult-onset Stills disease complicating Crohns disease. In patients with Crohns disease, polyarthritis and skin rash can easily be misdiagnosed as enteropathic arthritis with erythema nodosum associated with the Crohns disease. Although adult-onset Stills disease may not be fatal, early diagnosis is important because it can, in rare cases, result in life-threatening complications.     

Lemierre’s syndrome

Lemierre’s syndrome is characterized by an oropharyngeal infection followed by internal jugular vein septic thrombophlebitis and metastatic emboli, most often to the lungs and joints. The syndrome is most commonly associated with the anaerobic gram-negative rod Fusobacterium necrophorum. Diagnosis is established with evidence of metastatic infection and internal jugular vein thrombophlebitis. CT is considered the diagnostic procedure of choice. Treatment should include an extended course of a b-lactamase-resistant antibiotic and surgical drainage of any purulent fluid collection. Anticoagulation remains controversial, and ligation of the internal jugular vein is reserved for patients with persistent sepsis and recurrent emboli. With appropriate therapy, mortality is 4% to 12%; but mortality is increased when therapy is delayed.     

Takayasu’s arteritis

Takayasu's arteritis is a worldwide entity that requires astuteness for early diagnosis. The challenge for the practicing physician is to document active disease, not only to justify immunosuppressive therapy but also to determine the appropriate timing for surgical intervention. No single laboratory assay or imaging modality establishes disease activity, though T2-weighted magnetic resonance imaging, magnetic resonance angiography, and spiral computed tomographic angiography have shown potential in identifying acute inflammatory changes within the vessel wall. Treatment consists primarily of glucocorticoids, but cytotoxic agents such as methotrexate and cyclophosphamide may be added to treat recalcitrant disease. New immunomodulating agents such as leflunomide and tumor necrosis factor inhibitors require further study before they can be added to the treatment armamentarium.     

Takayasu’s arteritis

Opinion statement Takayasu’s arteritis is a rare disease that causes arterial inflammation with resulting stenosis or aneurysm of affected vessels. It primarily affects young females and has a predilection for the aorta, its primary branches, and the pulmonary arteries. Patients may present with symptoms of organ ischemia, such as stroke or claudication, or with signs of arterial stenosis, such as absent pulses or asymmetric blood pressures. Most patients require chronic immunosuppressive therapy to control inflammation. The foundation of therapy is glucocorticoids, which are often used in combination with other immunosuppressive agents, such as methotrexate, azathioprine, or mycophenolate mofetil. The goal of systemic therapy is to induce and maintain disease remission, often defined as the absence of new arterial lesions, lack of systemic symptoms, and ability to taper prednisone to less than 10 mg/d. Promising results have been seen with the use of agents directed against tumor necrosis factor in patients who have failed to maintain remission with standard therapies. Revascularization procedures such as percutaneous transluminal angioplasty (PTA) or bypass grafting are reserved for patients who have critical organ ischemia or severe extremity claudication. Bypass grafting has a lower restenosis rate than PTA and is the preferred method of restoring blood flow to affected organs. Frequent laboratory testing is recommended to monitor for drug toxicity and signs of disease relapse. At each clinic visit, physicians should auscultate for bruits over primary arterial branches and evaluate for asymmetry between arm and leg blood pressures. Biannual radiographic imaging with magnetic resonance or CT angiography is helpful to examine for evidence of new aortic aneurysms or arterial stenoses.     

Cushing’s disease

Cushing’s disease, i.e., pituitary ACTH-secreting adenoma causing excess glucocorticoid secretion, is a rare disease with significant mortality and morbidity. Timely diagnosis and appropriate treatment can alter the course of the disease and are therefore mandatory. First step of the diagnostic work-up is the endogenous glucocorticoid excess by measurement of urinary free cortisol, cortisol circadian rhythmicity or suppression by low doses of dexamethasone. In patients with equivocal results, second line tests, such as the dexamethasone-suppressed CRH test and desmopressin stimulation, usually enable the diagnosis to be confirmed. Measurement of plasma ACTH then allows the distinction between ACTH-dependent (e.g., pituitary or extrapituitary neuroendocrine tumors) and ACTH-independent causes (e.g., adrenal tumors). The last step in the diagnostic algorhythm is often the most fraught with problems as the distinction between Cushing’s disease and ectopic ACTH secretion relies on judicious interpretation of several diagnostic procedures. Positive responses to stimulation with CRH and inhibition by high doses of dexamethasone, if concurrent, enable a pituitary origin to be established whereas conflicting results call for inferior petrosal sinus sampling, the latter to be performed in experienced centres only. Visualisation of the tumor at pituitary imaging is helpful but not required for the diagnosis, as microadenomas often remain undectected by MRI and/or CT scan and, on the other hand, visualisation of a non-secreting incidentaloma may be misleading. Surgical removal of the pituitary tumor is the optimal treatment choice and should be attempted in every patient. Surgical failures as well as relapses can be treated by radiotherapy, medical therapy or, if necessary, bilateral adrenalectomy. Finally, patients cured of Cushing’s disease require long-term monitoring given the risk of relapse and clinical burden of associated ailments.     

Whipple’s disease

Whipple’s disease is a chronic multisystemic infection, due to Tropheryma whipplei, a bacterium ubiquitously present in the environment. Although it is very rare, its clinical features are non-specific and can affect several different districts. Whipple’s disease is therefore a condition that should always be kept in mind by doctors working in several branches of medicine, such as internal medicine, gastroenterology, rheumatology, neurology, and cardiology. The condition is fatal if not promptly recognized and treated, but the best treatment is still not completely defined, especially in relapsing disease, neurological manifestations, and in cases of immunoreconstitution after initiation of antibiotic treatment.     

Wegener’s Granulomatosis

Wegener's granulomatosis is an organ- and/or life-threatening autoimmune disease of as yet unknown etiology. The classic clinical triad consists of necrotizing granulomatous inflammation of the upper and/or lower respiratory tract, necrotizing glomerulonephritis, and an autoimmune necrotizing systemic vasculitis affecting predominantly small vessels. The detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 (PR3-ANCA) is highly specific for Wegener's granulomatosis. ANCA positivity is found only in about 50% of the patients with localized Wegener's granulomatosis (which is restricted to the respiratory tract and affects < or = 5% of the patients), whereas PR3-ANCA positivity is seen in 95% of the patients with generalized Wegener's granulomatosis. Studies showing an expansion of circulating tumor necrosis factor-(TNF-)alpha-producing Th1-type CD4(+)CD28(-) T-cell effector memory T-cells and their presence as Th1-type cytokine profile- driving cell population within granulomatous lesions provide the rationale for using TNF-alpha-blocking agents in Wegener's granulomatosis refractory to standard induction therapy with cyclophosphamide and corticosteroids ("Fauci's scheme"). Vasculitis is an independent risk factor for diffuse endothelial dysfunction and may be a consequence of TNF-alpha action on endothelial cells. Recently, another study has shown intima-media thickening of the wall of the common carotid artery and bulb, as well as a significantly increased incidence of stroke, myocardial infarction and occlusive artery disease in Wegener's granulomatosis. This study suggests that systemic inflammation and vasculitis contribute to accelerated arteriosclerosis in Wegener's granulomatosis.