Frequent Systemic Disease Research Articles

Mesenteric Panniculitis in Sjögren's Syndrome: A New Systemic Manifestation to Consider?

The association between mesenteric panniculitis and Sjögren’s syndrome, although rare, is starting to be recognized. Usually, mesenteric panniculitis is symptomatic, presenting with either general or gastrointestinal symptoms. Sjögren’s syndrome is an autoimmune disease that typically affects secretory glands, but may have serious systemic involvement. We report the case of a 77-year-old patient in whom accidental discovery of asymptomatic mesenteric panniculitis on computed tomography led to the diagnosis of Sjögren’s syndrome with several systemic manifestations.LEARNING POINTSMesenteric panniculitis is a rare condition, sometimes associated with autoimmune diseases, which can make the differential diagnosis with more frequent systemic diseases difficult. There is no specific treatment for mesenteric panniculitis, but it is agreed that only symptomatic patients should be treated.Although Sjögren’s syndrome typically presents with xerostomia and xerophthalmia (dry eyes and mouth syndrome, DEMS), it should always be kept in mind that systemic manifestations can occur, especially in more severe cases.

Safety and efficacy of microwave ablation for medically inoperable colorectal pulmonary metastases: Single-centre experience.

To evaluate the safety and therapeutic efficacy of percutaneous microwave ablation (MWA) for colorectal pulmonary metastases. Retrospective review of CT-guided lung MWA at a single tertiary institution. Adverse events within 30days of MWA were considered procedure-related complications. Imaging follow-up was with 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT and contrast-enhanced CT. Response of index tumours was assessed using modified response evaluation criteria in solid tumours (mRECIST). The CT-guided lung MWA was performed in 14 patients with treatment of 20 colorectal pulmonary metastases during 19 procedures. Mean follow-up from completion of the treatment protocol was 24.4±11.8months. Primary and secondary technique effectiveness rates were 75% and 50% respectively. Mean pre- and post-procedural carcino-embryonic antigen (CEA) levels were 7.9±10.8µg/L and 2.5±2.1µg/L respectively. Procedure-related complications were major in three procedures (15.8%) and minor in six procedures (31.6%). No procedure-related deaths occurred. Microwave ablation is safe and efficacious in the local control of colorectal pulmonary metastases. The frequent systemic disease progression despite local control would favour a minimally invasive treatment option over invasive surgery in the setting of oligometastatic disease.

Demographic and Clinical Features of Pediatric Uveitis at a Tertiary Referral Center in Iran.

Purpose:To determine the clinical features and distribution of uveitis in the pediatric age group at a referral eye care center in Shiraz, South Iran.Materials and Methods:All new cases of uveitis in patients 18-year-old or younger referred from January 2007 to December 2013 were enrolled in this study. The patient underwent a complete history of systemic and ocular diseases, comprehensive ophthalmic examination. Patients were classified according to the International Uveitis Study Group definitions. The definitive diagnosis was based on clinical manifestations and laboratory investigations.Results:Seventy-seven eyes (54 patients) comprised the study sample. The mean age at the onset of uveitis was 12.5 years. The female-to-male ratio was 1.25. Anterior uveitis was the most frequent anatomical location (40.7%), followed by intermediate uveitis (33.3%), posterior uveitis (18.5%), and panuveitis (7.5%). Seventy-four percent of patients presented with chronic uveitis. Noninfectious uveitis (81.5%) was the most frequent etiology. Thirty-seven percent of patients had a specific diagnosis for uveitis, and 63% were classified as idiopathic cases. Toxocariasis was the most common infectious cause. Associated systemic diseases were present in 14.8% of patients. The most frequent systemic disease was juvenile idiopathic arthritis in 9.2% of patients. Complications occurred in 66 (85.5%) of affected eyes. The most common complications were posterior synechia (20.7%), cataract (18.8%), and cystoid macular edema (12.9%).Conclusions:Uveitis in the majority of children had an insidious onset and was chronic. Over half the patients had a specific diagnosis. Idiopathic cases were more common in the intermediate uveitis group.

Knowledge of medical doctors in Turkey about the relationship between periodontal disease and systemic health.

Understanding the relationship between periodontal disease (PD) and systemic health (SH) is necessary for the accurate diagnosis and treatment of both. The aim of this study was to evaluate the knowledge of medical doctors in Turkey with regard to the association between PD and SH. This study was carried out using self-reported questionnaires that were sent to medical doctors who work at various universities and public and private hospitals in different cities in Turkey. The questionnaires consisted of questions about the demographic information of the medical doctors, as well as the knowledge of those doctors about the relationship between PD and SH. In total, 1,766 responses were received and 90.8% of the participants agreed that there was a relationship between PD and SH. Diabetes mellitus was the most frequent systemic disease (66.8%) known to be related to PD. Of the participants, 56.5% of the medical doctors referred their patients to periodontists for different reasons. Gingival bleeding was the most frequent reason for patient referrals, with 44% of doctors giving such referrals. Doctors who worked in basic medical sciences were significantly less aware of the relationship between PD and SH than the doctors in other specialties. Although the vast majority of the medical doctors reported that they knew the relationship between PD and SH, the findings of this study showed that this awareness was not supported by precise knowledge, and often failed to translate into appropriate clinical practice.

Bilateral femoral osteonecrosis in systemic lupus erythematosus — A clinical case

Systemic lupus erythematosus (SLE) is a systemic and complex autoimmune disease, characterized by clinical and laboratory heterogeneity. Osteonecrosis (ON) can complicate the course of SLE, with a variable frequency of 3% to 30%. Although the pathogenesis is not completely understood, SLE and chronic corticosteroid treatment seem to contribute to it. The use of high dose corticosteroid therapy is the major risk factor for the development of ON in SLE, being the most frequent systemic disease associated with ON caused by these drugs.

Therapeutical Approach of Osteoporosis — a Multidisciplinary Issue

AbstractOsteoporosis is the most frequent systemic disease of the bone, that affects elderly, mainly women in menopause. It can be defined by lowering of bone mass and microarchitectural deterioration of the bone tissue, resulting in an increased bone fragility. Main complications of osteoporosis are fractures of the vertebrae, hips and forearm. In view of its large variety of causes and manifestations, diagnostic and therapeutical approach in osteoporosis represents a multidisciplinary issue. The accurate diagnosis of osteoporosis is based on a method that measures the bone mineral density, expressed by the T-score, using dual energy X-ray absorptiometry, so called DXA. Lately, in practice in order for establishing the risk of osteoporosis and osteoporotic fracture the FRAX tool is increasingly used (The Fracture Risk Assessment). Treatment of osteoporosis is complex involving non-pharmacological and pharmacological measures. Non-pharmacological methods include preventive measures like exercise, external hip protectors, increase of dietary intake of calcium, vitamin D and proteins, especially in elderly, over 65 years. Pharmacological measures are represented by different types of drugs, including biphosphonates, bone formation stimulatory drugs, agents with new mechanisms of action, hormone replacement therapy and they will be indicated only after a detailed clinical and paraclinical examination of the patient. Regardless of the chosen pharmacological measure, periodical follow-up of efficacy, side-effects and complications of antiosteoporotic treatment, by clinical examination and laboratory investigations targeting bone remodelling, is strongly indicated.

Reporte de condiciones sistémicas de pacientes adultos. Facultad de Odontología, Universidad de Antioquia, 2011

Introducción: el objetivo de esta investigación fue determinar la prevalencia y la concordancia en el reporte de condiciones sistémicas entre la historia clínica odontológica (hc) y la entrevista telefónica (et), en los pacientes que asisten a dos clínicas del adulto de la Facultad de Odontología de la Universidad de Antioquia. Métodos: estudio transversal (n = 104). Se evaluaron las hc y se registraron las diez condiciones sistémicas más frecuentes según registros epidemiológicos de Medellín. Se realizó et para verificar información de las hc. Se calcularon prevalencias y el nivel de concordancia entre resultados de la hc y la et mediante el índice kappa y su intervalo de confianza al 95% (ic95%), según variables. Resultados: la condición sistémica más prevalente fue la enfermedad ocular (58% según et y 30% según hc y diferencias significativas p < 0,001), y la menos prevalente fue el hipertiroidismo (3% según hc y 5% según et, sin diferencias significativas). El análisis kappa mostró que las concordancias más débiles se presentaron para el hipertiroidismo (0,22; ic95%-0,38-0,82) y la enfermedad ocular (0,37; ic95% 0,20-0,72). Se evidenciaron variaciones en los índices de concordancia de acuerdo con sexo, clínica y edad. Conclusiones: existen variaciones en las prevalencias reportadas por hc y por et debidas a dificultades a la hora de diligenciar los antecedentes médicos personales, que produjeron un subregistro en la hc.

"Petrified ears" with idiopathic adult-onset pituitary insufficiency

“Petrified ears” or calcification of auricular cartilage is an uncommonly reported condition. The most common causes of this phenomenon are local trauma, frost bite, and inflammation. Adrenal insufficiency is the most frequent systemic disease associated with auricular calcification. We present a case of idiopathic adult-onset pituitary insufficiency with hypocortisolism and bilateral auricular calcification. Recognition of the association between auricular calcification and adrenal insufficiency can be an important step toward the identification of a life-threatening cortisol deficiency.

Micropapillary urothelial carcinoma of the urinary bladder: Early surgery or neoadjuvant chemotherapy?

e16007 Background: Micropapillary bladder carcinoma is a rare variant of urothelial carcinoma (UC) of the urinary bladder. As a particularly aggressive variant, patients are often urged to undergo up-front radical cystectomy. Though data is scarce on the treatment outcomes of patients with this entity, we present the case for neoadjuvant chemotherapy as opposed to early cystectomy in the setting of clinically localized micropapillary UC. Methods: A review of records of all patients evaluated at our institution for UC was conducted to identify micropapillary UC of the bladder over the period from 2000–2007. A total of 24 cases were found, and were evaluated for preoperative pathology and clinical stage, treatment course, pathological stage and cancer specific survival. Results: Mean patient age was 67.9 years with 19 males and 5 females. Twenty-one (87.5%) patients had clinically organ confined micropapillary UC at the time of diagnosis, three had minimally enlarged lymph nodes on pelvic CAT scans. Half of our patients had BCG refractory high grade non-muscle invasive UC. Twenty-two patients (91.67%) were offered radical cystectomy as first line management. Extended lymph node dissection was performed in eleven patients (45.83%). Final pathologic examination diagnosed metastatic lymph node involvement in 20 patients (83.33%), with 4 patients (20%) having positive LN outside the standard (pelvic) template of dissection. A stage upgrade was noticed in 95.23% of cases. Median cancer specific survival was 13 months. Survival at one year was 44% and 50% at 2years, with only one patient alive at 5 years. Conclusions: Our results suggest that clinically localized micropapillary UC is often metastatic to LN at the time of presentation. This setting of frequent systemic disease should encourage standard neoadjuvant chemotherapy rather than early surgical management for these patients. Extended LN dissection is warranted in these cases due to the high incidence of nodal involvement outside the standard template. No significant financial relationships to disclose.

Differenzialdiagnostik der diffusen alveolären Hämorrhagie

Diffuse alveolar haemorrhage (DAH) denotes a diffuse intra-alveolar bleeding from small vessels as a result of severe damage of the alveolocapillary membrane of the lungs. These are comparatively rare, but than often life threatening events. The differential diagnosis is broad and comprises immunologic as well as toxic, infectious, hemodynamic, neoplastic and physical causes. DAH may be a manifestation of systemic diseases, as well as a result of an injury restricted to the lungs. The most frequent systemic diseases, that cause DAH are the so-called small vessel vasculititis-microscopic polyangiitis and Wegener's granulomatosis. More rarely Goodpasture's syndrome and systemic lupus erythematosus are causal. Alongside the immunologic processes mainly diseases with increased left-ventricular preload, infections and side effects of drugs have to be kept in mind. This review summarizes the differential diagnostic spectrum and the diagnostic workup of patients with DAH. Because early aggressive treatment can be life-saving, early and aimed diagnostics is essential.

Clinical Significance of Cyclooxygenase-2 in NK/T-Cell Lymphoma, Nasal Type

Purpose/Objective: NK/T cell lymphoma, nasal type is a distinct disease entity characterized by local recurrence and frequent systemic disease recurrences. Cyclooxygenase-2 (COX-2) is well recognized as an enzyme, which enhances tumor invasiveness and metastasis potential by inducing apoptosis and angiogenesis. The aim of this study is to determine whether there are any differences in therapeutic response, patterns of systemic disease recurrence, and prognosis of nasal NK/T-cell lymphoma patients by the COX-2 expressions.