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Sarcoidosis
Kinds of Sarcoidosis Selected AbstractsSarcoidosis and giant midesophageal diverticulumDISEASES OF THE ESOPHAGUS, Issue 4 2000A. Raziel Traction diverticula of the midesophagus result from granulomatous inflammation of mediastinal lymph nodes. Tuberculosis and histoplasmosis are known etiologies of this condition. To the best of our knowledge, this is the first report of a traction diverticulum caused by sarcoidosis. [source] Sarcoidosis presenting with granulomatous uveitis induced by pegylated interferon and ribavirin therapy for hepatitis CINTERNAL MEDICINE JOURNAL, Issue 3 2008K. K. L. Yan Abstract Sarcoidosis is a systemic granulomatous disease that is triggered by an autoimmune process, and is now a well recognized but uncommon complication of antiviral therapy for Hepatitis C virus (HCV) infection, likely related to its immunomodulatory effects. The clinical presentation of HCV related sarcoidosis is as varied as systemic sarcoidosis, but ocular presentation alone has not been reported previously. We present a 23 year-old female who developed visual disturbances due to ocular sarcoidosis during the course of antiviral therapy for chronic HCV infection. Our case presentation is then followed by a review of the literature on the topic. We aim to stress the importance of screening for eye problems in following HCV patients undergoing antiviral therapy, and raise clinicians' awareness of sarcoidosis as a possible cause for eye problems even in the absence of respiratory complaints. [source] Sarcoidosis in the sunburnt countryINTERNAL MEDICINE JOURNAL, Issue 6 2007R. K. A. Allen No abstract is available for this article. [source] Seasonal variation of the onset of presentations in stage 1 sarcoidosisINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 11 2006S. S. DEMIRKOK Summary Sarcoidosis is a chronic disease with an unknown aetiology. Our aim was to evaluate the pattern of seasonality of stage 1 sarcoidosis subjects who had symptoms by all cases, by age and by both genders. In this study, we used Roger's test for cyclic variation to prove that this seasonal variation was more than chance. Four hundred ninety-two consecutive patients with sarcoidosis who presented different clinical symptoms were included in this retrospective cohort study. According to the chest X-ray examinations, 185 patients had stage 1, while 307 patients in control group had stage 0, 2, 3 and 4 sarcoidosis. The demographic features, presenting clinical features, course of the disease, initial diagnostic methods and both the month and the age at the initial diagnosis for each patient were analysed on chart reviews. Roger's test for cyclic variation was used to determine the significance of any seasonal variation of incidence. Otherwise, t -test was used. The distribution of cumulative monthly presentations for patients with stage 1 peaked in April (108% above the average) and was lowest in October, November and December (48% below the average) (p < 0.001). Seasonality of the control group peaked in May (84% above the average) and was lowest in August (69% below the average) (p < 0.001). The seasonal pattern of subjects within both groups was influenced by some age groups and by both genders (p < 0.05). Some differences in the amplitude of the seasonal variation by age and by both genders increase the possibility of interactions among age, gender and the disease. Further well-designed and prospective studies are required to better understand the importance of our findings and the pathogenesis of the disease. [source] Cutaneous sarcoid-like granulomas with alveolar hemorrhage and c-ANCA PR-3INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 9 2004Natividade Rocha MD A 28-year-old woman, employed as a leather factory worker, noted asymptomatic, well-delimited plaques on both knees, 6 years ago. The plaques were violaceous with a smooth surface. One appeared over a post-traumatic scar from childhood (Fig. 1). Two years later, she began to complain of symptoms suggestive of polyarthritis, first of the small joints of the hands (proximal interphalanges) and then of the larger joints (wrists, elbows, and knees). She was diagnosed with rheumatoid arthritis and began treatment with nonsteroidal anti-inflammatory drugs for 1 month without any change. Deflazacort, 12 mg/day, and hydroxychloroquine, 400 mg/day, were administered for 3 months, with improvement of her articular complaints, but not her skin lesions. Figure 1. Well-delimited, violaceous plaques with a smooth surface on the knees, one over an old post-traumatic scar One year later, she complained of dysphonia, which remitted spontaneously after some weeks. After one additional year, she noted papules, with similar characteristics to the plaques, on the elbows, and two well-delimited orange-to-brown plaques on the forehead (Fig. 2). Figure 2. Orange,brown plaques symmetrically placed on the forehead During the fifth year of the disease, she was referred for the first time to a dermatologist, who biopsied one of the knee lesions. The histologic result was compatible with "sarcoid granuloma." At that time, she presented with skin lesions as her only complaint. Sarcoidosis was suspected based on a chest X-ray, which revealed hilar lymphadenopathy and diffuse accentuation of the interstitium. In November 2000, she suddenly developed fever (40 °C), cough with hemoptysis, dysphonia, and subcutaneous nodules on the palmar surface of the fingers of both hands that were painless, well-delimited, 5 mm in diameter, and firm (Fig. 3). She reported a weight loss of 12 kg in the previous 3 months. Pulmonary condensation was found on auscultation, and she had palpable hepatomegaly. Peripheral lymphadenopathy was not present. Figure 3. Painless, well-delimited, firm subcutaneous nodules on the palmar surface of the fingers Laboratory investigations revealed normochromic, normocytic anemia (hemoglobin, 7.7 g/dL), iron deficit, a white blood cell count of 16,000/µL with neutrophilia, an erythrocyte sedimentation rate of 130 mm/h, elevation of liver enzymes, a slight increase in angiotensin-converting enzyme (ACE) level (72 U/L), hypergammaglobulinemia (IgG, 3350 mg/dL), antinuclear antibody (ANA) of 1 : 320, and a slight increase in CD4 and decrease in CD8 lymphocytes with normal cellular morphology in blood. Renal function, urine sediment, urine and serum calcium, complement (C4), dsDNA, antimitochondrial antibody, direct and indirect Coombs test, antineutrophil cytoplasmic antibody (ANCA), tuberculin skin tests, viral markers of hepatitis B, C, and human immunodeficiency virus (HIV), electrocardiogram (ECG), ophthalmic examinations, and culture for infectious agents in blood and sputum were all normal or negative. Computed tomography (CT) scan showed an infiltrate in the upper right pulmonary lobule with a central cavity and bilateral hilar lymphadenopathy (Fig. 4). Homogeneous hepatosplenomegaly was present. The bronchoalveolar lavage (BAL) showed a slight lymphocytic increase predominantly of CD8 cells and hemosiderosis. Stains for infectious agents, including acid-fast bacillus, fungi, Mycoplasma, and Legionella, were negative. Three biopsies from the forehead, elbows, and knees showed well-formed noncaseating epithelioid cell granulomas with giant cells of the Langhans type in the dermis, suggestive of sarcoidosis (Figs 5 and 6). A fourth biopsy from a finger nodule demonstrated inflammatory infiltration of the dermis and necrosis with cellular debris. Vasculitis was not seen (Fig. 7). Figure 4. Computed tomography scan showing an infiltrate in the upper right pulmonary lobule with a central cavity Figure 5. Beneath a flattened epidermis, several sarcoid granulomas composed of epithelioid histiocytes and several multinucleated giant cells of Langhans type can be seen (hematoxylin and eosin, ×10) Figure 6. Less well-formed sarcoid granulomas in a hyperkeratotic area, surrounded by a sparse rim of lymphocytes (hematoxylin and eosin, ×20) Figure 7. Foci of necrosis and fibrinoid degeneration with some neutrophil infiltration and nuclear dusting (hematoxylin and eosin, ×40) The patient was treated with a broad-spectrum empirical antimicrobial (levofloxacin, 500 mg daily intravenously) over 12 days, with prompt improvement in her symptoms and remission of the forehead and finger lesions. Nevertheless, on the first evaluation after hospitalization, the CT scan showed persistence of the pulmonary cavity (Fig. 8). A repeat ANCA determination was positive (cytoplasmic pattern, c-ANCA) at 1 : 640 by indirect immunofluorescence (IIF). Antiproteinase-3 antibody was demonstrated at 78 by enzyme-linked immunosorbent assay (ELISA). Figure 8. Computed tomography scan showing persistence of the pulmonary cavity She underwent an open lung biopsy which revealed intra-alveolar hemorrhage and scanty noncaseating epithelioid cell granulomas of the sarcoidosis type in the peripheral blood vessels without vasculitis. A diagnosis of Wegener's granulomatosis was made and she began prednisolone (1 mg/kg/day) and oral cyclophosphamide (2 mg/kg/day). One year later, she is asymptomatic, the skin lesions have completely remitted, c-ANCA is negative, and the CT scan shows partial regression of the pulmonary cavity. [source] Sarcoidal granulomatous tenosynovitis of the hands occurring in an organ transplant patientJOURNAL OF CUTANEOUS PATHOLOGY, Issue 8 2007J. Andrew Carlson Six years after kidney-pancreas transplant, a 47-year-old white man developed multiple subcutaneous and tenosynovial nodules of hands and wrists, limiting mobility. Biopsy of multiple nodules showed fibrosing, sarcoidal granulomas, some of which contained pigmented material. Microbiology, immunohistochemistry, scanning electron microscopy with backscattered electron imaging and energy dispersive X-ray analysis and polymerase chain reaction assays failed to show any infectious agents or foreign material. There was no historical, clinical or laboratory evidence of systemic sarcoidosis. It is not known whether the donor had sarcoidosis. Despite empiric antimycobacterial therapy and ongoing immunosuppressive therapy (corticosteroids, mycophenolate, cyclosporine), the man has progressively developed more nodules, limiting hand function. Sarcoidosis occurring in non-donor tisssue post-transplantation is an exceedingly rare complication of transplantation. We discuss this case and review the literature on sarcoidal tenosynovitis and sarcoidosis occurring post-transplantation. [source] Clinical spectrum and histological analysis of 32 cases of specific cutaneous sarcoidosisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2006Cristina Mangas Background:, Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology in which skin involvement is frequent. Objective:, To review histological characteristics of biopsies of specific cutaneous lesions of sarcoidosis and their relationship with clinical course. Patients and methods:, Biopsies from 32 patients with specific cutaneous sarcoidosis were reviewed. Histological findings and clinical characteristics of these patients were analysed. Results:, The initial clinical lesions of the patients were ten infiltrated nodule-plaques, eight papules, four maculopapular eruptions, five scar sarcoidosis, four subcutaneous nodules and one lupus pernio. Sarcoidal granulomas were located at dermis in 31 cases (74%) and at subcutaneous fat in 12 (28%) but only four were subcutaneous exclusively. Perivascular or periannexial distribution of granulomas was observed in eight cases (19%) and they had coalescence in 29 samples. The presence of foreign material was demonstrated in 11 cases (26%). Conclusions:, Clinical spectrum of specific lesions of cutaneous sarcoidosis showed a good correlation with granulomas localization in the biopsies. However, traditional classification of specific cutaneous sarcoidosis is often overlapping. On the other hand, foreign bodies and other atypical histological findings were more common than initially expected. [source] A common haplotype of the C-C chemokine receptor 2 gene and HLA-DRB1*0301 are independent genetic risk factors for Löfgren's syndromeJOURNAL OF INTERNAL MEDICINE, Issue 5 2008P. Spagnolo Abstract. Aim., Sarcoidosis is a heterogeneous disorder with a strong genetic influence. Genetic factors are also thought to influence disease severity and outcome. We sought to determine whether polymorphisms within CCR2 gene predispose to Löfgren's syndrome , a clinically and genetically distinct sarcoidosis phenotype , and, importantly, whether this association is independent of the known association with the HLA-DRB1*0301 allele. Methods., We investigated 5 CCR2 variants and HLA-DRB1*0301 by sequence-specific primer (SSP) polymerase chain reaction (PCR) in 176 Spanish (76 Löfgren's syndrome, 100 controls) and 387 Swedish subjects (126 Löfgren's syndrome, 77 non-Löfgren sarcoidosis, 184 controls). Results., One of the deduced haplotypes (CCR2 haplotype 2) was associated with Löfgren's syndrome in both Spanish (OR: 2.03, uncorrected P = 0.02; permuted P = 0.041 vs. controls) and Swedish patients (OR: 3.02, uncorrected P = 0.0007; permuted P = 0.0027 vs. non-Löfgren sarcoidosis; OR: 2.46, uncorrected P = 0.0005; permuted P = 0.0031 vs. controls). HLA-DRB1*0301 allele frequency was also increased in Spanish (OR: 3.52, P = 0.0004 vs. controls) and Swedish patients with Löfgren's syndrome (OR: 10.98, P < 0.0001 vs. non-Löfgren sarcoidosis, OR: 7.71, P < 0.0001 vs. controls). Finally, multivariate analysis revealed that the CCR2 association was independent of HLA-DRB1*0301 in both Spanish (P = 0.02 vs. controls) and Swedish cohorts (P = 0.002 vs. non-Löfgren sarcoidosis, P = 0.001 vs. controls). Conclusions., This study confirms that CCR2 haplotype 2 and HLA-DRB1*0301 are independent genetic risk factors for Löfgren's syndrome. [source] Cutaneous sarcoidosis: updates in the pathogenesisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 7 2010MM Ali Abstract Sarcoidosis is a multiorgan granulomatous disease in which the skin is one of the frequently involved target organs. Cutaneous involvement occurs in a third of patients with sarcoidosis and has protean manifestations. More than a century has passed since the initial description of sarcoidosis, but its cause continues to be an enigma. Recent studies have introduced several new insights into the pathogenesis of this disease. The aim of this literature review was to provide a comprehensive overview on the current updates in the pathogenesis of sarcoidosis. This review has revealed that several genetic polymorphisms are associated with an increased risk of developing sarcoidosis, suggesting that genetic susceptibility to sarcoidosis is probably polygenic. Environmental factors may also modify the susceptibility to sarcoidosis. Evidence favouring an infectious aetiology has been accumulating, but the results of studies are conflicting. The current concept is that the pathogenesis of sarcoidosis involves a T-helper-1-mediated immune response to environmental antigens in a genetically susceptible host. The studies carried out on sarcoidosis have largely focused on the pulmonary aspects and have been mainly conducted by respiratory physicians. In contrast, research conducted on the cutaneous aspects of sarcoidosis is comparatively limited. Although tremendous advances have been made, there is a significant gap between the vast knowledge accumulated on sarcoidosis in recent years and the understanding of this disease. [source] Sarcoidosis of the skin , A dermatological puzzle: important differential diagnostic aspects and guidelines for clinical and histopathological recognitionJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2010G Tchernev Abstract Sarcoidosis of the skin may have an extremely heterogeneous clinical presentation, so that the definitions of ,great imitator' and ,clinical chameleon' have long been used. There is, in fact, a large group of skin diseases that can enter the differential diagnosis with cutaneous sarcoid manifestations, either clinically or/and pathologically. As the clinical consequences and the prognosis of these groups of diseases are often very different, it is important to correctly plan the diagnostic workup. The diagnostic process in this case often presents a challenge as no single test is sufficiently specific, so that a certain diagnosis can be only made in the presence of a compatible clinical and radiographic picture, along with histopathological evidence of non-necrotizing, epithelioid cell granulomas, and exclusion of other potential aetiologies. For practical reasons, four main groups of skin conditions capable of mimicking sarcoidosis can be identified: (i) transmissible, infectious diseases; (ii) allergic and immunological manifestations of various aetiologies; (iii) granulomatous diseases of various aetiologies; and (iv) lymphomas and pseudolymphomas. The aim of this article is to describe the main clinical and histopathological findings of such disease entities, and to discuss the role of those features (morphological, pathological and laboratory) that can help distinguish them from sarcoidosis of the skin. [source] Spectrum of Fibrosing Diffuse Parenchymal Lung DiseaseMOUNT SINAI JOURNAL OF MEDICINE: A JOURNAL OF PERSONALIZED AND TRANSLATIONAL MEDICINE, Issue 1 2009Adam S. Morgenthau MD Abstract The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky-Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end-stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed. Mt Sinai J Med 76:2,23, © 2009 Mount Sinai School of Medicine [source] "End-stage" Pulmonary Fibrosis in SarcoidosisMOUNT SINAI JOURNAL OF MEDICINE: A JOURNAL OF PERSONALIZED AND TRANSLATIONAL MEDICINE, Issue 1 2009Alvin T. Teirstein MD Abstract Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation. Mt Sinai J Med 76:30,36, © 2009 Mount Sinai School of Medicine [source] Mycobacterial heat shock protein-induced blood T lymphocytes subsets and cytokine pattern: Comparison of sarcoidosis with tuberculosis and healthy controlsRESPIROLOGY, Issue 3 2007Anna DUBANIEWICZ Background and objective: Sarcoidosis (SA) is a disorder of unknown aetiology. Mycobacterium tuberculosis heat shock proteins (Mtb-hsp) have been considered as causative agents of SA. The role of Mtb-hsp in the immune response in SA has not been investigated. Methods: Mtb-hsp-stimulated T-cell subsets and Th1/Th2 cytokine patterns in the supernatant from peripheral blood mononuclear cell cultures from 22 SA patients, 20 tuberculosis (TB) patients and 20 healthy volunteers were compared using flow cytometry. Results: In unstimulated cultures, a significantly higher percentage of CD8+,,+T-cells were present in SA versus controls. Similarly there was a significantly increased IL-6 and decreased IL-4 level in SA and significantly lower INF-,, IL-2, IL-4, IL-10 production in TB versus controls. After Mtb-hsp stimulation, there was a significantly increased TNF-,, IL-6, IL-10 and decreased INF-,, IL-2, IL-4 production in SA and significantly increased TNF-,, IL-6 concentrations in TB versus controls. CD8+,,+IL-4+T-cells were detected significantly less often in Mtb-hsp-induced cultures in SA versus controls. Comparing SA versus TB, CD4+,,+TCR-cells were significantly increased in Mtb-hsp-induced cultures in TB versus controls and SA. Before stimulation, significantly increased IL-6, IL-10 and decreased IL-4 level in SA versus TB was revealed, whereas Mtb-hsp stimulation caused significantly increased IL-10 and decreased IL-4 concentrations in SA. Conclusions: After Mtb-hsp stimulation, increased levels of pro-inflammatory cytokines, TNF-, and IL-6 were found in sera from SA and TB patients in comparison with healthy controls; SA patients demonstrated the lowest levels of IL-4 and the highest levels of IL-10. [source] Sarcoidosis in Singapore: Epidemiology, clinical presentation and ethnic differencesRESPIROLOGY, Issue 3 2007Devanand ANANTHAM Background and objectives: The aim of this study is to better understand the epidemiological and clinical features of patients with sarcoidosis in Singapore and to ascertain if ethnic differences exist. Methods: A review of hospital medical records from June 1998 to May 2004 to identify patients with sarcoidosis. Results: There were 59 patients with sarcoidosis identified (19 Chinese, 29 Asian Indian and 11 Malay). The estimated annual incidence of sarcoidosis in Singapore was 0.56 per 100 000. There was a significant difference between the observed and expected disease frequency in Chinese (32.2% vs 78.7%) and Indians (49.2% vs 6.0%, P < 0.005). A bimodal distribution of age at diagnosis was seen with peaks in the 30,39 years and 50,59 years age groups; 38.9% of cases were over the age of 50. Chinese patients were more likely to be asymptomatic (57.9%, P = 0.015) and less likely to have impaired spirometry (P = 0.013). Pulmonary sarcoid presented largely as stage 0 or stage 1 disease (74.4%). Overall mean spirometry was unimpaired and prognosis was good with 79.2% showing no radiological deterioration. There were no significant differences in organ involvement or treatment between ethnic groups. Conclusion: Sarcoidosis in Singapore is rare and the incidence differs between ethnic groups. Chinese appear to have a lower incidence and a less symptomatic presentation; Indians have a higher incidence and poorer clinical course. [source] Sarcoidosis of the Breast: An Unusual Presentation of a Systemic DiseaseTHE BREAST JOURNAL, Issue 1 2007Brandi T. Nicholson MD No abstract is available for this article. [source] Sarcoidosis: a view from afarTHE CLINICAL RESPIRATORY JOURNAL, Issue 2 2007Richard A. DeRemee No abstract is available for this article. [source] Clinical aspects and immune reactions in sarcoidosis,THE CLINICAL RESPIRATORY JOURNAL, Issue 2 2007Johan Grunewald Abstract Introduction:, Sarcoidosis is a granulomatous disorder of unknown aetiology, affecting young adults and frequently involving the lungs. Objective:, The aim of the present review was to give an overview of the clinical aspects in sarcoidosis. Results:, The majority of patients recover, but some develop a chronic disease that may result in fibrosis and respiratory failure. Besides the lungs, peripheral lymph nodes, the skin, the liver and the eyes are commonly affected as well. The genetic background, as well as environmental factors, is of importance for developing sarcoidosis. The incidence varies in different populations, in the Nordic countries approximately with 20/100 000 new patients yearly. Sarcoidosis is diagnosed when clinical and radiological findings are supported by histological evidence in the form of non-caseating epithelioid cell granulomas, and when other causes of these features are excluded. Patients in need of treatment are usually treated with corticosteroids, topically or as oral steroids. A clinical effect of immunomodulatory drugs blocking tumour necrosis factor (TNF), has been suggested from several case reports, while two controlled studies showed only minor effects; however, with a tendency to a more pronounced effect on patients with a more severe disease. The immune response in sarcoidosis, with a typical accumulation of CD4+ T-cells to the lungs, indicate the existence of specific antigens in this disease. Recently, antigens derived from infectious agents such as Mycobacteria and Proprionibacterium acnes have come into focus. Lymphocyte populations with immunoregulatory functions have recently been investigated and seem to be dysfunctional in sarcoidosis, opening the possibility of developing new treatment strategies in this disease. Conclusion:, Recent technical developments have provided better tools, enabling detailed and more thorough analyses of the inflammatory process in sarcoidosis. Please cite this paper as: Grunewald J. Clinical aspects and immune reactions in sarcoidosis. The Clinical Respiratory Journal 2007; 1:64,73. [source] Acquired Nasopharyngeal Stenosis in a Patient with SarcoidosisTHE LARYNGOSCOPE, Issue S3 2010JR Brodsky [source] Fragmented QRS Complexes on 12-Lead ECG: A Marker of Cardiac Sarcoidosis as Detected by Gadolinium Cardiac Magnetic Resonance ImagingANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 4 2009Mohamed Homsi M.D. Background: Fragmented QRS complexes (fQRS) on a 12-lead ECG are a marker of myocardial scar in patients with coronary artery disease. Cardiac sarcoidosis is also associated with myocardial granuloma formation and scarring. We evaluated the significance of fQRS on a 12-lead ECG compared to Gadolinium-delayed enhancement images (GDE) in cardiac magnetic resonance imaging (CMR). Method and results: The ECGs of patients (n = 17, mean age: 52 ± 11 years, male: 53%) with established diagnosis of sarcoidosis who underwent a CMR for evaluation of cardiac involvement were studied. ECG abnormalities included bundle branch block, Q wave, and fQRS. fQRS, Q wave, and bundle branch block were present in 9 (53%), 1 (6%), and 4 (24%) patients, respectively. The sensitivity and specificity of fQRS for detecting abnormal GDE were 100% and 80%, respectively. Sensitivity and specificity of Q waves were 11% and 100%, respectively. Conclusions: fQRS on a 12-lead ECG in patients with suspected cardiac sarcoidosis are associated with cardiac involvement as detected by GDE on CMR. [source] Sarcoidosis with cutaneous granulomatous vasculitisAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 3 2010Chia-Hung Wei ABSTRACT Ulceration of non-caseating granulomas is a rare cutaneous presentation of sarcoidosis. Granulomatous vasculitis is classically associated with Wegener's granulomatosis, lymphomatoid granulomatosis or Churg,Strauss syndrome. It is also commonly noted in pulmonary sarcoidosis, but has seldom been reported in cutaneous sarcoidosis, particularly the ulcerative variant. We present a rare case of sarcoidosis with multiple purpuric leg ulcers showing a granulomatous vasculitis histologically. [source] Clinical features of intraocular inflammation in Hokkaido, JapanACTA OPHTHALMOLOGICA, Issue 4 2009Hirokuni Kitamei Abstract. Purpose:, We aimed to investigate the clinical features of intraocular inflammation/uveitis in Hokkaido, Japan. Methods:, We retrospectively reviewed the medical records of 1240 uveitis patients (511 men, 729 women) who visited Hokkaido University Hospital, Sapporo, Japan between 1994 and 2003. Results:, Mean age at disease onset was 41.7 ± 17.8 years in men and 45.7 ± 18.3 years in women. Anterior, posterior and combined anterior and posterior segment intraocular inflammation accounted for 45.1%, 4.7% and 50.2% of cases, respectively. Sarcoidosis was the most frequent aetiology (14.9%), followed by Vogt,Koyanagi,Harada (VKH) disease (9.7%) and Behçet's disease (6.7%). Aetiologies in 49.8% patients were unknown. In sarcoidosis, women represented 72.4% of patients, and disease onset occurred at 35.1 ± 19.0 years of age in men and 50.3 ± 16.5 years in women. In VKH disease, 54.2% of patients were women, and disease onset took place at 45.9 ± 15.8 years in men and 46.4 ± 14.1 years in women. In Behçet's disease, men accounted for 56.6% of patients, and disease onset occurred at 35.5 ± 8.5 years in men and 44.5 ± 11.5 years in women. Conclusions:, Women were more prone to developing sarcoidosis compared with men. By contrast, men were more prone to developing Behçet's disease. The mean age at disease onset in both sarcoidosis and Behçet's disease was significantly lower in men than in women. [source] Cutaneous sarcoid with varied morphology associated with hypercalcaemia and renal impairmentCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009H. Miida Summary Sarcoidosis is a multisystem disorder of unknown aetiology, which presents with hilar lymphadenopathy, pulmonary infiltration, and ocular and cutaneous involvement. Cutaneous lesions often present as erythema nodosum, maculopapular, plaque, scar, subcutaneous nodule or lupus pernio. Most patients with cutaneous involvement have a single type of skin lesion, but some cases may have , 2 types. We report a case of sarcoidosis presenting with various types of skin lesions. The case was also complicated by hypercalcaemia and renal dysfunction, and was successfully treated with oral corticosteroids. Presentation of various skin lesions may indicate systemic organ involvement requiring treatment with systemic corticosteroid. [source] Optic perineuritis as a rare initial presentation of sarcoidosisCLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 7 2007Patrick Yu-Wai-Man MRCOphth Abstract Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology and establishing the correct diagnosis can be challenging. Although dysfunction of the anterior visual pathways is uncommon, it is the most common neuro-ophthalmological manifestation of this condition and given the potential for irreversible, severe visual loss, prompt diagnosis and treatment are essential. We describe a patient with optic perineuritis as a rare initial presentation of sarcoidosis and discuss the underlying pathophysiology and management. [source] Pulmonary Sarcoidosis with Pulmonary Hypertension, Fossa Ovalis Aneurysm and Interatrial ShuntCLINICAL CARDIOLOGY, Issue 6 2008Rami N. Khouzam No abstract is available for this article. [source] Advances in the genetics of sarcoidosisCLINICAL GENETICS, Issue 5 2008G Smith Sarcoidosis is an uncommon disease of granulomatous inflammation. Genetic predisposition to sarcoidosis is indicated by observations of familial clustering, increased concordance in monozygotic twins over other siblings, and variations in susceptibility and disease presentation among different ethnic groups. Published studies on sarcoidosis have investigated a variety of genetic associations. These studies used techniques ranging from classic human lymphocyte antigen genotype correlations to genome-wide linkage scans. Results have both supported and refuted disease associations with a number of genes potentially involved in the pathogenesis of sarcoidosis. Here, we review representative studies concerning the genetics of sarcoidosis. While investigations to date have failed to identify a unifying genetic signature associated with sarcoidosis, numerous studies have identified genetic associations with disease subtypes or within specific populations. These studies suggest that genetic susceptibility to sarcoidosis is complex and polygenic in nature. Future studies will help clarify the genetics of sarcoidosis and allow for the development of diagnostic, prognostic and therapeutic technologies. [source] Tissue verification of stage I sarcoidosisCYTOPATHOLOGY, Issue 6 2007J. M. Reich No abstract is available for this article. [source] Transbronchial fine needle aspiration cytology in the diagnosis of mediastinal/hilar sarcoidosisCYTOPATHOLOGY, Issue 1 2007S. Smojver-Je Objective:, The diagnostic value of transbronchial/transtracheal fine needle aspiration (TBFNA) cytology in the patients with mediastinal and/or hilar lymphadenopathy has been investigated. Method:, Out of 116 patients with mediastinal/hilar lymphadenopathy, the diagnosis of sarcoidosis was established in 88 (75.9%). One hundred and seventy-one TBFNAs from different lymph node stations were performed using a cytological 26-gauge needle. Adequate lymph node samples were obtained in 157 of 171 (91.8%) TBFNA and 14 of 171 (8.2%) TBFNA samples were inadequate. Results:, Cytological findings consistent with sarcoidosis were found in 79 of 88 (89.77%) patients and 133 of 157 (84.71%) samples. The sensitivity of TBFNA cytology in sarcoidosis presenting as mediastinal/hilar lymphadenopathy was 78.7%, specificity 92.3%. Conclusions:, Overall diagnostic accuracy of TBFNA cytology in the diagnosis of sarcoidosis was 86.2%, and cytological findings consistent with sarcoidosis were the only morphological diagnosis of sarcoidosis in 63.6% of patients. [source] A rare case of multiple myeloma initially presenting with pseudoachalasiaDISEASES OF THE ESOPHAGUS, Issue 6 2009Georgia Lazaraki SUMMARY Pseudoachalasia is a rare clinical entity with clinical, radiographic, and manometric features often indistinguishable from achalasia. Primary adenocarcinomas arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes of pseudoachalasia. Rarely, processes other than esophagogastric cancers including chronic idiopathic intestinal pseudo-obstruction, amyloidosis, sarcoidosis, Chagas' disease, vagotomy, antireflux surgery, pancreatic pseudocysts, von Recklinghausen's neuroinomatosis, gastrointestinal stromal tumor, and other malignancies and rare genetic syndromes, may lead to the development of pseudoachalasia. Secondary achalasia is extremely rare, with less than 100 cases reported in the literature so far. Gastrointestinal manifestations in primary or secondary amyloidosis include abdominal pain, diarrhea, constipation, malabsorption, obstruction, motility disturbance, intestinal infarction, perforation, and hemorrhage; however, gastrointestinal tract involvement is asymptomatic in most instances. We present here a rare case of multiple myeloma initially presenting with dysphagia because of esophageal amyloidosis and manometric findings typical of achalasia. [source] Sarcoidosis and giant midesophageal diverticulumDISEASES OF THE ESOPHAGUS, Issue 4 2000A. Raziel Traction diverticula of the midesophagus result from granulomatous inflammation of mediastinal lymph nodes. Tuberculosis and histoplasmosis are known etiologies of this condition. To the best of our knowledge, this is the first report of a traction diverticulum caused by sarcoidosis. [source] Impaired aortic elastic properties in patients with systemic sarcoidosisEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 2 2008I. Moyssakis Abstract Background, Systemic sarcoidosis (Sar) is a granulomatous disorder involving multiple organs. Widespread vascular involvement and microangiopathy are common in patients with Sar. In addition, subclinical cardiac involvement is increasingly recognized in patients with Sar. However, data on the effect of Sar on the elastic properties of the arteries and myocardial performance are limited. In this study we looked for differences in aortic distensibility (AoD) which is an index of aortic elasticity, and myocardial performance of the ventricles, between patients with Sar and healthy subjects. In addition, we examined potential associations between AoD and clinical, respiratory and echocardiographic findings in patients with Sar. Materials and methods, A total of 83 consecutive patients (26 male/57 female, mean age 51·1 ± 13·3 years) with Sar, without cardiac symptoms, were included. All patients underwent echocardiographic and respiratory evaluation including lung function tests. Additionally, 83 age- and sex-matched healthy subjects served as controls. AoD was determined non-invasively by ultrasonography. Results, AoD was lower in the Sar compared to the control group (2·29 ± 0·26 vs. 2·45 ± 0·20 ·10,6 cm2· dyn,1, P < 0·01), while left ventricular mass (LVM) was higher in the Sar group (221·3 ± 50·2 vs. 195·6 ± 31·3 g, P = 0·007). Furthermore, myocardial performance of both ventricles was impaired in the Sar group. Multivariate linear regression analysis in the total sample population demonstrated a significant and independent inverse relationship between AoD and the presence of Sar (P < 0·001). The same analysis in the Sar patients showed that AoD was associated significantly and independently with the stage of Sar, age, systolic blood pressure, LVM and myocardial performance of both ventricles. No significant relationship was found between AoD and disease duration, pulmonary artery pressure or lung function tests. Conclusions, Presence and severity of Sar are associated with reduced aortic distensibility, irrespective of the disease duration, pulmonary artery pressure and lung function. In addition, patients with Sar have increased LVM and impaired myocardial performance. [source] | ||||||||||||||||||||||||||||||||||||||||