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Biomedical subjects

Chris T Derk

Publications and source records attributed to Chris T Derk.

18 recordsLinked to original sources

A cohort study of cancer incidence in systemic sclerosis.

OBJECTIVE: To describe the incidence of cancer in a large cohort of patients with systemic sclerosis (SSc) and compare it to the Surveillance Epidemiology and End Results (SEER) cancer registries. METHODS: Cancer risk in a large cohort of patients with SSc followed at our institution was assessed. A total of 769 patients with SSc who were followed between 1987 and 2002 were screened for the development of cancer. Standardized incidence ratios (SIR) for malignancies identified after diagnosis of SSc were calculated using the SEER cancer registries and stratified by sex. RESULTS: Ninety malignancies were diagnosed in 769 patients followed at our institution between 1987 and 2002. Sixty-two malignancies were diagnosed after diagnosis of SSc in a total of 3,775 patient years of followup. Twenty-eight malignancies were diagnosed prior to diagnosis of SSc. The SIR for all cancers diagnosed after diagnosis of SSc was 1.55 (1.16-1.93). The SIR for esophageal cancer was 15.9 (4.2-27.6) while that of oropharyngeal cancer was 9.63 (2.97-16.29). CONCLUSION: We identified an overall increase in the incidence of cancer in a cohort of patients with SSc compared to the general population, with statistically significant differences in the incidence of esophageal and oropharyngeal cancers.

Cohort Studies↗

Acute myocardial infarction in systemic sclerosis patients: a case series.

To characterize the clinical manifestations of patients with systemic sclerosis who develop a myocardial infarction (MI), a retrospective review of the medical records of all patients who were admitted to our institution between 1982 and 2002 and had the dual diagnosis of systemic sclerosis and an acute MI was done. From 1,009 systemic sclerosis hospital admissions, 11 (1.09%) were for an acute MI. Three of these patients had normal coronaries, and instead of wall motion abnormalities, left ventricular hypertrophy was the predominant finding of an echocardiography. The odds ratio of finding normal coronaries in systemic sclerosis vs the general population who develops an acute MI is 33.89 (14.08-81.39). Seven of our patients had an elevated creatinine level on presentation. Acute MI is an uncommon manifestation in systemic sclerosis patients. Normal coronaries are seen more commonly in these patients as compared to the general population, while vascular, gastrointestinal, and renal involvement is prevalent in these patients.

Coronary Artery Disease↗

Pantoprazole and perinuclear antineutrophil cytoplasmic antibody-associated vasculitis.

Systemic vasculitis may, at times, be drug-induced and associated with antineutrophil cytoplasmic antibodies (ANCA). However, pantoprazole, a commonly used and well-tolerated proton pump inhibitor, has not previously been reported to cause ANCA-associated syndromes. We describe a patient who developed interstitial nephritis, cutaneous vasculitis, a perinuclear ANCA staining pattern (pANCA) on immunofluorescence, and anti-myeloperoxidase antibodies (MPO-ANCA) in association with pantoprazole. We review various immune-mediated syndromes reported in association with proton pump inhibitors, including one report of omeprazole associated with interstitial nephritis and the development of ANCA.

2-Pyridinylmethylsulfinylbenzimidazoles↗

Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control study.

This study aims to characterize the clinical features of a cohort of patients diagnosed with systemic sclerosis (SSc) after the age of 75 and compare them to a group diagnosed at a younger age. We record the review of 769 patients diagnosed with SSc over the past 16 years. Utilizing a nested case-control model, we compare demographics, disease severity, morbidity, and mortality data of all patients diagnosed after the age of 75 to sex- and disease-type-matched, randomly selected group of patients diagnosed with SSc before the age of 60. Twelve patients were diagnosed with SSc after the age of 75, seven with the diffuse, and five with the limited form. It took longer to diagnose SSc in the older patients, and comparison of disease severity revealed a worse pulmonary picture and a more frequent development of malignancy in the older patients as compared with the younger ones. During a mean follow-up of 36.2 months, our cohort of patients did not have worsening in their disease severity, though 6 months after the last follow-up, six patients died. We conclude that a diagnosis of SSc at an older age appears to be a poor prognostic indicator related to both disease severity and comorbidities. A higher clinical suspicion will lead to an earlier diagnosis and a potential decrease in morbidity and mortality.

Aged↗

Statins and the vasculopathy of systemic sclerosis: potential therapeutic agents?

It has been postulated that endothelial cell injury is the initiating event in the pathogenesis of systemic sclerosis, causing attraction, attachment, migration and infiltration of activated T-cells and subsequent production of cytokines and growth factors. As a result of the action of these cytokines and growth factors, chemoattraction of fibroblasts into the vessel wall and transdifferentiation of resident fibroblasts and smooth muscle cells into myofibroblasts occur leading to fibrosis and exaggerated collagen deposition in the vessel wall. To date, the therapeutic options for the vasculopathy of systemic sclerosis have been limited to drugs that cause vasodilation and inhibit platelet aggregation and only a few agents have shown vascular remodeling effects. Therapeutic agents that could potentially modify the course of this vasculopathy may have a disease-modifying effect, particularly, if instituted in the early stages of the disease. Extensive recent studies have shown that statins display numerous effects independent of their well-established lipid-lowering effect that may be of potential benefit in preventing vascular injury and ischemic vascular events. Here, we review the current literature, which suggests that statins may have a modifying effect on the vasculopathy of systemic sclerosis.

Animals↗

Increased incidence of carcinoma of the tongue in patients with systemic sclerosis.

OBJECTIVE: To describe the incidence of carcinoma of the tongue in a large cohort of patients with systemic sclerosis (SSc). METHODS: In total, 769 patients with SSc were prospectively followed over 16 years for the development of cancer. Patients with a diagnosis of carcinoma of the tongue were identified to determine the incidence of this cancer in SSc. The results were compared to the incidence of tongue cancer in the SEER cancer registries. RESULTS: A total of 3775 patient-years of followup of 769 patients with SSc (392 diffuse cutaneous, 377 limited cutaneous) prospectively evaluated for the occurrence of cancer disclosed 9 patients who were diagnosed with oral cavity and pharyngeal carcinomas. Six of these patients had squamous cell carcinoma of the tongue. One of these had both pharyngeal and tongue squamous cell carcinomas within a 4-year period, and another had 3 separate squamous cell carcinomas of the tongue. The standardized incidence ratio of squamous cell carcinoma of the tongue observed in this cohort of patients with SSc was 25-fold higher than that expected in an age adjusted population from the SEER cancer registries. All patients with SSc identified within this cohort with oral cavity carcinomas had the diffuse subset of the disease. CONCLUSION: There is a highly significant increase in the incidence of squamous cell carcinoma of the tongue in patients with SSc. A remarkable observation was that all patients within this cohort who developed oral cancer had the diffuse subset of SSc. This suggests a relationship between the etiology or pathogenesis of the diffuse form of SSc and development of squamous cell carcinoma of the tongue in this group of patients.

Aged↗

Rheumatoid arthritis: an update.

Rheumatoid arthritis is a chronic multi-system disorder of unknown etiology with most common symptoms being persistent inflammatory synovitis, usually involving peripheral joints in a symmetrical pattern. Improved understanding of the molecular mechanisms involved in the pathogenesis of rheumatoid arthritis has led to long awaited, highly effective treatments for this disorder.

Anti-Inflammatory Agents, Non-Steroidal↗

A case of anti-Jo1 myositis with pleural effusions and pericardial tamponade developing after exposure to a fermented Kombucha beverage.

The pathogenesis of the idiopathic inflammatory myopathies has been postulated to be an environmental trigger causing the expression of the disease in a genetically predisposed patient. We report a case of anti-Jo1 antibody-positive myositis which was associated with pleural effusions, pericardial effusion with tamponade, and 'mechanic's hands', probably related to the consumption of a fermented Kombucha beverage. Kombucha 'mushroom', a symbiosis of yeast and bacteria, is postulated to be the trigger for our patient's disease owing to the proximity of his symptoms to the consumption of the Kombucha beverage.

Antibodies, Antinuclear↗

Systemic lupus erythematosus and acute pancreatitis: a case series.

The aim of this study was to evaluate whether corticosteroid use is the etiological agent in acute pancreatitis in patients with systemic lupus erythematosus, or whether it is related to the underlying connective tissue disorder. Hospital admissions at Thomas Jefferson University Hospital between 1982 and 2002 that carried the dual diagnosis of systemic lupus erythematosus and pancreatitis were identified, and demographic data, clinical interventions and parameters of clinical progression of their disease were identified. From 2947 admissions with systemic lupus erythematosus 25 (0.85%) were diagnosed as having acute pancreatitis; 76% of cases had active systemic lupus erythematosus on presentation, with an average of 4.4 organ involvement, and a clustering of renal disease (56%), pleural effusion (48%) and arthritis (44%) in these patients. Fifteen patients with active disease and three whose disease was inactive received increased doses of corticosteroids, and four active cases and one inactive one stayed on the same doses. Two inactive patients received no corticosteroids before or after the diagnosis of pancreatitis. Eighty-two percent of patients had clinical and laboratory improvement on the higher or maintenance dose of corticosteroids. We therefore concluded that acute pancreatitis is a rare manifestation of systemic lupus erythematosus, and corticosteroids do not appear to be the etiological agent.

Acute Disease↗

Primary B-cell lymphoma of the tongue in a patient with systemic sclerosis.

We report a patient with systemic sclerosis who had the unusual development of a large-cell B-cell lymphoma of the base of his tongue. Patients with systemic sclerosis display an increased incidence of malignant neoplasms, particularly, breast and lung carcinomas [Arthritis. Rheum. 36 (1993) 460; Ann. Rheum. Dis. 52 (1993) 531]. The description of this case adds further evidence to the possible interrelationship of systemic sclerosis and the development of malignant neoplasms. A careful review of the English medical literature disclosed that B-cell lymphoma of the tongue in a patient with systemic sclerosis is an association which has not been described previously.

Aged↗

A primary care approach to Sjögren's syndrome. Helping patients cope with sicca symptoms, extraglandular manifestations.

Sjögren's syndrome is a chronic, systemic, autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, most notably the salivary and lacrimal glands. It results in loss of functional epithelium, diminished exocrine function, and gradual onset of dryness of the eyes, mouth, and other body parts (sicca syndrome). Because most patients with Sjögren's syndrome are female, this syndrome also represents a major women's health problem that is often misdiagnosed and undertreated. In this article, Drs Derk and Vivino review the criteria for diagnosis, common signs and symptoms, and strategies for increasing patient comfort.

Adaptation, Psychological↗

Systemic sclerosis: current views of its pathogenesis.

Systemic sclerosis (SSc) is an autoimmune disorder of unknown etiology characterized by severe and often progressive cutaneous and visceral fibrosis, pronounced alterations in the microvasculature, and numerous cellular and humoral immune abnormalities. Clinically, SSc is very heterogeneous, encompassing a spectrum ranging from mild limited forms of skin sclerosis with minimal internal organ involvement to severe skin and multiple internal organ fibrosis. Mortality and morbidity in SSc are very high and are directly related to the extent of the fibrotic and microvascular alterations. A better understanding of the pathogenesis of this incurable disorder will help to better target and design effective therapy in the future.

Autoimmune Diseases↗

Goodpasture-like syndrome induced by D-penicillamine in a patient with systemic sclerosis: report and review of the literature.

We report a case of Goodpasture-like syndrome developing in a patient who was treated with D-penicillamine for the diffuse form of systemic sclerosis. This unusual pulmonary-renal syndrome has been described on rare occasions in patients receiving D-penicillamine. This complication appeared to be uniformly fatal unless treated with aggressive immunotherapy. We review the cases reported to date in the literature and describe the clinical characteristics, therapy, and outcome of this group of patients.

Aged↗

Autoantibodies in patients with systemic sclerosis and cancer: a case-control study.

OBJECTIVE: To determine the prevalence of specific autoantibodies in a cohort of patients with systemic sclerosis (SSc) and a diagnosis of cancer, and to compare it to that in a group of patients with SSc who were never diagnosed with cancer. METHODS: From 769 patients with SSc followed at our center over the past 15 years, 77 had a diagnosis of cancer. The results of autoantibody studies in this group were compared to those from 159 SSc patients without cancer randomly selected from the rest of the patient population using chi-square test for independence and the null hypothesis for 2 population proportions. RESULTS: There was no statistically significant difference between the proportions of patients with positive autoantibodies between the 2 groups, except that a higher prevalence of autoantibodies with a nucleolar immunofluorescence pattern was observed in the group of patients who were never diagnosed with cancer (p < 0.01). CONCLUSION: In contrast to previous studies, in our case-control study we were not able to detect a significant difference in autoantibody frequency or patterns among SSc patients with and without a diagnosis of cancer. These results refute the conclusion made previously that certain autoantibodies may represent risk factors for the development of cancer in patients with SSc.

Age Distribution↗

Osteoporosis in premenopause. When are screening and treatment prudent?

Osteoporosis is considered a disorder of postmenopausal women because bone mineral loss and accompanying fragility fractures are common in this group. However, certain premenopausal women are also at risk for osteoporosis. Because of a lack of screening and treatment guidelines for premenopausal women, it can be difficult to determine whether a low bone mineral density correlates with a disease such as osteoporosis and to predict the potential risk of a fracture and formulate the correct course of action. In this article, Dr Derk identifies high-risk groups, presents an approach to judicious screening, and recommends prevention and treatment strategies.

Absorptiometry, Photon↗