RESUMO
Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since publication of the international IPF guideline in the year 2011 and Update 2018 several studies and technical advances occurred, which made a new assessment of the diagnostic process mandatory. In view of the antifibrotic drugs which have been approved for the treatment of IPF patients, the goal of this guideline is to foster early, confident and effective diagnosis of IPF. The guideline focusses on the typical clinical setting of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardised questionnaires, serologic testing and cellular analysis of bronchoalveolar lavage. High resolution computed tomography remains crucial in the diagnostic work-up.âIf it is necessary to obtain specimen for histology transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom bronchoscopic diagnosis did not provide the information needed. Despite considerable progress, IPF remains a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.
Assuntos
Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/terapia , Pulmão/diagnóstico por imagem , Guias de Prática Clínica como Assunto , Biópsia , Humanos , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Doenças Pulmonares Intersticiais , Tomografia Computadorizada por Raios XRESUMO
Sarcoidosis is a multisystemic granulomatous disorder which affects the respiratory system in the majority of the cases. Symptomatic cardiac manifestations are found in less than 10â% of the affected cohorts and show a large heterogeneity based on the ethnic background. Cardiac sarcoidosis is not only found in patients with rhythmogenic heart disease, such as atrial and ventricular fibrillation but also in all phenotypes of cardiomyopathy. The overall morbidity and mortality caused by cardiac sarcoidosis in Germany remains unclear and large prospective international observational studies.underline the importance of this disease entity. This consensus paper on diagnostic and therapeutic algorithms for cardiac sarcoidosis is based on a current literature search and forms an expert opinion statement under the auspices of the German Respiratory Society and the German Cardiac Society. The rationale of this statement is to provide algorithms to facilitate clinical decision-making based on the individual case situation.
Assuntos
Cardiologia/normas , Guias de Prática Clínica como Assunto , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/terapia , Cardiomiopatias , Consenso , Alemanha/epidemiologia , Humanos , Comunicação Interdisciplinar , Pneumologia/normas , Sociedades MédicasRESUMO
BACKGROUND: Bronchoalveolar lavage (BAL) is standard diagnostic procedure. Procedural recommendations have been made by pneumological societies including normal values for interpretation of BAL cytology. These normal values derive from small studies in healthy volunteers and have never been analysed for their sensitivity and specificity. OBJECTIVES: This study aims to analyse sensitivity and specificity of these normal values by assessing lavage cell composition in healthy and diseased individuals. METHODS: More than 6000 BAL were retrospectively analysed for their cellular distribution including BALs of 250 healthy individuals. All BALs were obtained under similar conditions. RESULTS: Bronchoalveolar lavage cytology of healthy individuals mirrors data from previous studies with smoking being the most important manipulator of BAL cytology. Analyses of proposed normal values demonstrate specificity between 80% and 95%, whereas sensitivity ranges between 35% and 65%. Using different mathematical models, a value summing up the differences to ATS-proposed normal values of the cytological pattern was found to best discriminate between healthy and diseased individuals with a sensitivity of nearly 60% with a predefined specificity of 95%. CONCLUSION: In summary, our analysis confirmed prior results for healthy volunteers and enlarged these findings by analysing sensitivity and specificity of lavage results in an independent validation cohort of diseased individuals. Thereby, the study may influence the acceptance of BAL in the diagnostic workup of individuals with pulmonary diseases. Additionally, the study proposes a novel value that facilitates lavage interpretation and may therefore be useful in further studies.
Assuntos
Líquido da Lavagem Broncoalveolar/citologia , Pneumopatias/diagnóstico , Lavagem Broncoalveolar , Contagem de Células , Eosinófilos/metabolismo , Feminino , Humanos , Linfócitos/metabolismo , Macrófagos/metabolismo , Masculino , Pessoa de Meia-Idade , Neutrófilos/metabolismo , Valores de Referência , Estudos Retrospectivos , Sensibilidade e Especificidade , Fumar/efeitos adversosRESUMO
Sarcoidosis is a rare granulomatous disease mainly affecting lymph nodes and the lungs but joints, bones, muscles and other organs can also be affected. Sarcoidosis therefore represents an important differential diagnosis to various rheumatic diseases. For the diagnosis and differential diagnostic clarification, bronchoscopy including endobronchial ultrasound-guided fine needle aspiration of mediastinal and hilar lymph nodes represent the main procedures. Because of the high spontaneous remission rate initiating a therapy requires a therapeutic goal defined by sarcoidosis-associated functional organ impairment, especially for acute sarcoidosis. Cortisone represents the most commonly administered medication whereas methotrexate and azathioprine are well-established second-line medications. Antibodies which neutralize tumor necrosis factors (TNF) are a potential third-line therapy.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Broncoscopia/métodos , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Imunossupressores/uso terapêutico , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/tratamento farmacológico , Cortisona/uso terapêutico , Diagnóstico Diferencial , Quimioterapia Combinada , Medicina Baseada em Evidências , Alemanha , Humanos , Resultado do TratamentoRESUMO
The etiology of sarcoidosis is still elusive, yet there has been considerable progress in various areas of basic and clinical research. This review focuses on mechanisms of granuloma formation and on new findings in autoimmunity and genetics of sarcoidosis. A new promising concept arose, where serum amyloid A and/or mycobacterial antigens serve as nidus for granuloma formation. Furthermore, autoimmunity in sarcoidosis was neglected for a long time, yet new studies found autoantigens and abnormalities in antigen presentation in sarcoidosis. Last but not least, large genome-wide association studies discovered several new predisposing genes, leading to new hypotheses on pathomechanisms of sarcoidosis.In the second part, we focus on ongoing or recently completed clinical-pharmacological studies in patients with sarcoidosis: Positive studies were published in well characterized and homogenous subcohorts of sarcoid patients. Several drugs have shown a positive effect on sarcoidosis-associated fatigue, on sarcoidosis of the skin and on pulmonary hypertension in sarcoid patients. It seems that the generation of clinically closely defined subcohorts is necessary to achieve positive outcomes in studies on sarcoidosis.
Assuntos
Anti-Inflamatórios/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Medicamentos para o Sistema Respiratório/uso terapêutico , Sarcoidose/tratamento farmacológico , Sarcoidose/imunologia , Doenças Autoimunes/genética , Medicina Baseada em Evidências , Predisposição Genética para Doença/genética , Humanos , Fenômenos Imunogenéticos/genética , Modelos Imunológicos , Sarcoidose/genética , Resultado do TratamentoRESUMO
Sarcoidosis is a granulomatous disease that mainly affects the lungs and intrathoracic lymph nodes; however, virtually any organ can be affected. As an orphan disease, recommendations are mainly based on observational or small randomized studies as well as experts' opinion. Diagnosing sarcoidosis requires proof of non-necrotizing granulomas in patients with a compatible symptomatic pattern and the exclusion of other granulomatous diseases. Granulomas can be detected best in the lungs or intrathoracic lymph nodes. Therefore, bronchoscopy and endobronchial ultrasound with biopsies of lymph nodes are the major tools to diagnose sarcoidosis. Frequently, close follow-up and symptomatic therapy are sufficient to allow for spontaneous resolution. In case of functional organ impairment, cardial or CNS involvement, or other complications, steroid therapy is necessary with a starting dose of 0.5 mg/kg body weight that should be tapered-off over 6-12 months. Steroid-refractory disease can be treated by adding methotrexate or azathioprine, two drugs long known in sarcoidosis treatment. Monoclonal antibodies against TNF and lung transplantation are further therapeutic options.
Assuntos
Diagnóstico por Imagem/normas , Medicina Interna/normas , Guias de Prática Clínica como Assunto , Sarcoidose/diagnóstico , Sarcoidose/terapia , Esteroides/uso terapêutico , Anti-Inflamatórios/normas , Anti-Inflamatórios/uso terapêutico , Alemanha , Humanos , Esteroides/normasRESUMO
Spirometry is a highly standardized method which allows to measure the forced vital capacity (FVC) with high precision and reproducibility. In patients with IPF FVC is directly linked to the disease process which is characterized by scaring of alveoli and shrinkage of the lungs. Consequently, there is ample evidence form clinical studies that the decline of FVC over time is consistently associated with mortality in IPF. As for the first time effective drugs for the treatment of IPF are available it becomes obvious that in studies which could demonstrate that the drug reduces FVC decline, a numerical effect on mortality was also observed, while in one study where a significant effect on FVC decline was missed, there was also no change in mortality. Based on these studies FVC decline is a validated surrogate of mortality in IPF. It is concluded that FVC decline is not only accepted as an endpoint of clinical treatment trials in IPF but is also valid as a patient related outcome parameter which should be considered for the assessment of the efficacy of an IPF drug.
Assuntos
Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/mortalidade , Guias de Prática Clínica como Assunto , Espirometria/estatística & dados numéricos , Espirometria/normas , Capacidade Vital , Medicina Baseada em Evidências , Alemanha , Humanos , Incidência , Prognóstico , Reprodutibilidade dos Testes , Medição de Risco/métodos , Sensibilidade e Especificidade , Espirometria/métodos , Taxa de SobrevidaRESUMO
Sarcoidosis is a multisystemic granulomatous disorder which affects the respiratory system in the majority of the cases. Cardiac manifestations are found in up to 10â% of the affected cohort and show a large heterogeneity based on the ethnic background. Cardiac sarcoidosis are not only found in patients with rhythmogenic heart disease such as atrial and ventricular fibrillation but also in all phenotypes of cardiomyopathies. The overall morbidity and mortality caused by cardiac sarcoidois in Germany is unclear and no large prospective international studies are published on this topic. This consensus paper on diagnostic and therapeutic algorithms in cardiac sarcoidosis is based on a current literature search and forms a expert opinion statement under the hospices of the "Deutsche Gesellschaft für Pneumologie" and "Deutsche Gesellschaft für Kardiologie". It is the rationale of this statement to offer algorithms to facilitate clinical decision-making based on the individual case.
Assuntos
Algoritmos , Cardiologia/normas , Cardiomiopatias/diagnóstico , Cardiomiopatias/terapia , Guias de Prática Clínica como Assunto , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/terapia , Alemanha , Humanos , Pneumologia/normasRESUMO
Granuloma formation occurs in the human body if there is a particle which persists in phagocytes and which the immune system cannot eliminate. The immune reaction of granuloma formation evolved in order to combat mycobacteria with the aim of localizing mycobacteria and to avoid spreading of mycobacteria throughout the body. Granulomatous lung diseases are often accompanied by severe, systemic inflammation. However, acute phase proteins may be only slightly elevated. The spectrum of granulomatous lung diseases is broad. Sarcoidosis is the most common granulomatous lung disease. To diagnose sarcoidosis, other infectious granulomatous lung diseases such as tuberculosis, atypical mycobacterial and fungal infection have to be ruled out. Pulmonary granuloma also evolve in the context of autoimmune diseases such as rheumatoid arthritis, granulomatosis with polyangiitis (GBA, Wegener's) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Furthermore, immunodeficiencies such as common variable immunodeficiency (CVID) and immune reconstitution syndrome in HIV can be associated with systemic granulomatous inflammation. Finally, occupational lung disease, particularly hypersensitivity pneumonitis, silicosis, hard metal lung, and chronic berylliosis are associated with pulmonary granuloma formation.
Assuntos
Diagnóstico por Imagem/métodos , Granuloma do Sistema Respiratório/diagnóstico , Pneumopatias/diagnóstico , Doenças Profissionais/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Diagnóstico Diferencial , HumanosRESUMO
Allogeneic hematopoietic cell transplantation (allo-HCT) of older or patients with comorbidities has become possible due to new regimens for reduced-intensity conditioning. The use of fludarabine, carmustine and melphalan as the preparative regimen (FBM) reduces toxicity while providing substantial anti-leukemic activity. Chronic GVHD (cGVHD) of the lung or bronchiolitis obliterans syndrome (BOS) remains a serious non-infectious complication contributing to treatment-related morbidity. We conducted a retrospective analysis of 259 patients (median age: 61.5, range: 24-76 years) transplanted after FBM conditioning to identify and characterize clinical risk factors for developing BOS. The cumulative incidence rate of BOS was 4.2% (95% confidence interval (CI): 2.4-7.6%) at 1 year and 8.5% (95% CI: 5.6-12.9%) at 5 years after allo-HCT with a median follow-up of 36.5 (range: 3-136) months. In multivariate analysis, age <55 years at allo-HCT (who received 25% higher carmustin-dose in preparative regimen), pulmonary complications after allo-HCT and GVHD prophylaxis without in-vivo T-cell depletion (cyclosporine-A/ATG or cyclosporine-A/alemtuzumab) were associated with BOS. We observed no significant differences in clinical variables such as smoking or lung diseases before allo-HCT. In contrast to cGVHD affecting other organs, BOS showed no impact on overall survival. In conclusion, we identified risk factors associated with developing BOS after conditioning with a reduced toxicity protocol.
Assuntos
Bronquiolite Obliterante/etiologia , Transplante de Células-Tronco Hematopoéticas/métodos , Condicionamento Pré-Transplante/efeitos adversos , Condicionamento Pré-Transplante/métodos , Adulto , Fatores Etários , Idoso , Bronquiolite Obliterante/tratamento farmacológico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Incidência , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Transplante Homólogo , Adulto JovemAssuntos
Beriliose/diagnóstico , Beriliose/epidemiologia , Beriliose/etiologia , Beriliose/prevenção & controle , Estudos Transversais , Diagnóstico Diferencial , Medicina Baseada em Evidências , Alemanha , Humanos , Concentração Máxima Permitida , Vigilância da População , Fatores de Risco , Sarcoidose/diagnósticoRESUMO
Idiopathic pulmonary fibrosis is a fatal lung disease with a variable and unpredictable natural history and limited treatment options. Since publication of the ATS-ERS statement on IPF in the year 2000 diagnostic standards have improved and a considerable number of randomized controlled treatment trials have been published necessitating a revision. In the years 2006 - 2010 an international panel of IPF experts produced an evidence-based guideline on diagnosis and treatment of IPF, which was published in 2011. In order to implement this evidence-based guideline into the German Health System a group of German IPF experts translated and commented the international guideline, also including new publications in the field. A consensus conference was held in Bochum on December 3rd 2011 under the protectorate of the "Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin (DGP)" and supervised by the "Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften" (AWMF). Most recommendations of the international guideline were found to be appropriate for the german situation. Based on recent clinical studies "weak negative" treatment recommendations for pirfenidone and anticoagulation were changed into "weak positive" for pirfenidone and "strong negative" for anticoagulation. Based on negative results from the PANTHER-trial the recommendation for the combination therapy of prednisone plus azathiorpine plus N-acetlycsteine was also changed into strong negative für patients with definite IPF. This document summarizes essential parts of the international IPF guideline and the comments and recommendations of the German IPF consensus conference.
Assuntos
Anti-Inflamatórios/uso terapêutico , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/terapia , Guias de Prática Clínica como Assunto , Pneumologia/normas , Tomografia Computadorizada por Raios X/métodos , Alemanha , Humanos , Fibrose Pulmonar Idiopática/sangue , InternacionalidadeRESUMO
BACKGROUND: The relevance of beryllium sensitization testing for occupational health practice and prevention is unclear. AIMS: To analyse the natural course of beryllium sensitization and clarify the prognosis following cessation of exposure among sensitized workers. METHODS: An electronic literature search was conducted in PubMed, Embase, Toxline and Cochrane databases supplemented by a manual search. Data abstraction and study quality assessment with adapted guideline checklists were performed independently by three reviewers. Seven studies met the eligibility criteria and were included in the systematic review; however, six of the seven studies were of low methodological quality. RESULTS: A substantial (although not specifically quantifiable) proportion of beryllium-sensitized employees will develop chronic beryllium disease (CBD). To date, it is unknown if cessation of exposure in sensitized workers reduces the progression rate to CBD. CONCLUSIONS: To determine the utility of regular assessments for beryllium sensitization among exposed workers, there is a need for prospective studies. This should include detailed and continuous exposure monitoring, regular tests for beryllium sensitization and a thorough diagnostic evaluation of sensitized workers to confirm or exclude CBD.
Assuntos
Poluentes Ocupacionais do Ar/efeitos adversos , Beriliose/diagnóstico , Berílio/toxicidade , Doenças Profissionais/diagnóstico , Exposição Ocupacional/efeitos adversos , Radioisótopos/toxicidade , Poluentes Ocupacionais do Ar/imunologia , Beriliose/imunologia , Beriliose/prevenção & controle , Líquido da Lavagem Broncoalveolar/imunologia , Doença Crônica , Progressão da Doença , Feminino , Alemanha , Humanos , Ativação Linfocitária/imunologia , Masculino , Doenças Profissionais/imunologia , Doenças Profissionais/prevenção & controle , Exposição Ocupacional/prevenção & controle , PrognósticoRESUMO
The clinical outcome of sarcoidosis is quite variable. Several scoring systems have been used to assess the level of disease and clinical outcome. The definition of clinical phenotypes has become an important goal as genetic studies have identified distinct genotypes associated with different clinical phenotypes. In addition, treatment strategies have been developed for patients with resolving versus non resolving disease. A task force was established by the World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) to define clinical phenotypes of the disease based on the clinical outcome status (COS). The committee chose to examine patients five years after diagnosis to determine the COS. Several features of the disease were incorporated into the final nine categories of the disease. These included the current or past need for systemic therapy, the resolution of the disease, and current status of the condition. Sarcoidosis patients who were African American or older were likely to have a higher COS, indicating more chronic disease. The COS may be useful in future studies of sarcoidosis.
Assuntos
Comitês Consultivos , Predisposição Genética para Doença , Pneumologia , Sarcoidose Pulmonar , Adolescente , Adulto , Idoso , Criança , Congressos como Assunto , Diagnóstico Diferencial , Feminino , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Morbidade , Fenótipo , Estudos Retrospectivos , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/genética , Adulto JovemRESUMO
Sarcoidosis is a complex granulomatous inflammatory disorder that shares several clinical and pathogenic features with inflammatory bowel disease (IBD). Postulating a common genetic basis of inflammatory diseases, we tested 106 single-nucleotide polymorphisms (SNPs) that are known or have been suggested to be associated with IBD for a potential association with sarcoidosis and its acute and chronic subphenotypes. We genotyped 1,996 German sarcoidosis patients, comprising 648 acutely and 1,161 chronically affected individuals, 2,622 control subjects, and 342 German trios with affected offspring using SNPlex™ technology. The nonsynonymous SNP rs11209026 (Arg381Gln) in the interleukin (IL)-23 receptor (IL23R) gene was associated with chronic sarcoidosis (OR 0.63; p = 5.58×10(-5)), which was supported by the result of a transmission disequilibrium test analysis in the independent family sample (OR 0.50; p = 0.031). Marker rs12035082 located at chromosome 1q24.3 was found to be associated with the acute subphenotype (OR 1.36; p = 6.80×10(-7)) and rs916977 (HERC2 locus; OR 1.30; p = 4.49×10(-5)) was associated with sarcoidosis. Our results highlight the potential importance of the IL-23 signalling pathway for the development of chronic sarcoidosis. The finding links sarcoidosis pathogenesis to other inflammatory conditions and may contribute to new hypotheses on disease mechanisms.
Assuntos
Doenças Inflamatórias Intestinais/diagnóstico , Sarcoidose/diagnóstico , Estudos de Casos e Controles , Regulação da Expressão Gênica , Marcadores Genéticos , Genótipo , Alemanha , Fatores de Troca do Nucleotídeo Guanina/biossíntese , Humanos , Inflamação , Doenças Inflamatórias Intestinais/complicações , Razão de Chances , Fenótipo , Polimorfismo de Nucleotídeo Único , Controle de Qualidade , Receptores de Interleucina/biossíntese , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Sarcoidose/complicações , Ubiquitina-Proteína LigasesRESUMO
In previous years numerous advances in diagnostics, staging and therapy of lung cancer have been achieved. Nevertheless, it remains the most frequent cause of death from cancerous diseases. Early diagnosis and exact staging enable multimodal therapy regimens adjusted to age and comorbidities, which result in complete remission in a few and in prolonged survival and good quality of life in most patients. Curative surgery is possible in stage I non-small cell lung cancer (NSCLC) and results in a 5-year survival rates of up to approximately 75%. Using multimodal therapy approaches long-term survival can even be achieved in 40-50% of patients with advanced T4 tumors. However, in NSCLC with distant metastases median survival time is only 8-12 months. In elderly patients with no surgical options low cytotoxic monotherapy can be employed with a palliative intent. In the limited disease stage of small cell lung cancer (SCLC) long lasting remission after polychemotherapy has been observed in a minority of patients. However, in the extensive disease stage polychemotherapy prolongs the survival time of SCLC patients from 1-2 months to approximately 12 months.
Assuntos
Carcinoma Pulmonar de Células não Pequenas/diagnóstico , Carcinoma de Células Pequenas/diagnóstico , Neoplasias Pulmonares/diagnóstico , Idoso , Antineoplásicos/uso terapêutico , Broncoscopia , Carcinoma Pulmonar de Células não Pequenas/mortalidade , Carcinoma Pulmonar de Células não Pequenas/patologia , Carcinoma Pulmonar de Células não Pequenas/cirurgia , Carcinoma de Células Pequenas/mortalidade , Carcinoma de Células Pequenas/patologia , Carcinoma de Células Pequenas/cirurgia , Causas de Morte , Quimioterapia Adjuvante , Terapia Combinada , Diagnóstico Diferencial , Intervalo Livre de Doença , Diagnóstico Precoce , Humanos , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/cirurgia , Estadiamento de Neoplasias , Cuidados Paliativos , PneumonectomiaRESUMO
Oxidative stress is an imbalance between oxidants (reactive oxygen and nitrogen species) and antioxidants that may affect lipids, DNA, carbohydrates and proteins. The lung is continuously exposed to endogenous and exogenous oxidants (cigarette smoke, mineral dust, ozone, radiation). Reactive oxygen and nitrogen species are mainly produced by phagocytes as well as by polymorphonuclear, alveolar, bronchial and endothelial cells. A potential role of oxidative stress in the pathogenesis of diffuse lung diseases (particularly idiopathic pulmonary fibrosis) has been demonstrated. Increased oxidant levels and decreased antioxidant defences can contribute to the progression of idiopathic pulmonary fibrosis and other diffuse lung diseases. The growing number of papers on the different aspects of oxidant/antioxidant imbalance in diffuse lung diseases in the last decade reflects increasing interest in this topic and suggests that specific DLDs may be characterized by specific patterns of oxidation and antioxidant responses. The study of oxidative stress can provide insights into etiopathogenesis and favour the discovery of new treatments. In this review of the literature on oxidants and antioxidants in diffuse lung diseases, the focus is on idiopathic pulmonary fibrosis, sarcoidosis, pneumoconiosis and pulmonary fibrosis associated with systemic sclerosis.
Assuntos
Antioxidantes/metabolismo , Pneumopatias/etiologia , Oxidantes/metabolismo , Estresse Oxidativo/fisiologia , Humanos , Pulmão/metabolismo , Pneumopatias/metabolismoRESUMO
Sarcoidosis is the most frequent interstitial lung disease of unknown origin affecting patients of younger age in many cases. In Germany its incidence is higher than that of tuberculosis. Diagnosis is made by demonstration of epitheloid, non-necrotizing granuloma in pulmonary parenchyma and by exclusion of other granulomatous disorders such as mycobacteriosis, berylliosis, or hypersensitivity pneumonitis. Its etiology is still elusive and since it is a systemic disease any organ can be involved. The lung is most frequently involved and, therefore, diagnosis can easily be made by bronchoscopy with transbronchial biopsy and bronchoalveolar lavage. Frequently a favorable spontaneous course takes place but in about one third of the cases a chronic course of disease is seen which requires systemic treatment with prednisolone. In progressive disease despite prednisolone treatment the combination with azathioprine or methotrexate might be helpful.
Assuntos
Anti-Inflamatórios/uso terapêutico , Broncoscopia/métodos , Imunossupressores/uso terapêutico , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/tratamento farmacológico , HumanosRESUMO
Sarcoidosis is a multifactorial and polygenic disorder. The current knowledge of its genetics will be presented and discussed in the context of other granulomatous disorders of known and unknown aetiology. The differing and common features of these disorders lead to the perspective that in near future it will be possible to establish genotype-phenotype correlations which will predict the course and therapy response in an individual case.