RESUMEN
Health status and quality of life are impaired in patients with idiopathic pulmonary fibrosis (IPF) and idiopathic non-specific interstitial fibrosis (iNSIP). In Germany exists only the K-BILD questionnaire for patients with ILD 1 in a professional translation by Kreuter et al. 2 This questionnaire focuses on the main problems in patients with progressive lung fibrosis in a limited manner. Therefore a new quality of life questionnaire for patients with idiopathic pulmonary fibrosis was developed and linguistically validated. METHODS: The linguistic validation of our questionnaire was carried out in a multistage process in collaboration with the developer of the questionnaire and bilingual, professional translators. Review by the developers and back translations as well as clinical assessment by IPF- and iNSIP-patients ensured that the translated questionnaire reflected the intention of the original English version of our questionnaire.Cross-validation was carried out with the St. Georges Respiratory Questionnaire (SGRQ). RESULTS: The new questionnaire concerning the health status was composed in English and German language. The questions cover five scales (sensitivity, selectivity and symptoms like breathlessness and cough and a visual analog scale on general health status) with 23 items. CONCLUSIONS: The results show that the FFB maps the special needs of the patients with IPF and iNSIP well and can support clinical and scientific questions and can be helpful in monitoring the clinical course.
Asunto(s)
Fibrosis Pulmonar Idiopática , Calidad de Vida , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Lenguaje , Lingüística , Encuestas y CuestionariosRESUMEN
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.
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Líquido del Lavado Bronquioalveolar/citología , Enfermedades Pulmonares/diagnóstico , Lavado Broncoalveolar , Recuento de Células , Eosinófilos/metabolismo , Femenino , Humanos , Linfocitos/metabolismo , Macrófagos/metabolismo , Masculino , Persona de Mediana Edad , Neutrófilos/metabolismo , Valores de Referencia , Estudios Retrospectivos , Sensibilidad y Especificidad , Fumar/efectos adversosRESUMEN
BACKGROUND: Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies. MATERIAL AND METHODS: This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords "SSc" and "treatment". RESULTS: The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy. CONCLUSION: Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.
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Trasplante de Células Madre Hematopoyéticas , Inmunosupresores/uso terapéutico , Enfermedades Pulmonares Intersticiales , Esclerodermia Sistémica , Enfermedades del Tejido Conjuntivo , Ciclofosfamida , Humanos , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/terapia , Esclerodermia Sistémica/inmunología , Esclerodermia Sistémica/terapiaRESUMEN
Beginning in April of 2019, the US saw >â2,000 cases of hospitalized, often young, patients with severe acute lung injury, of which over 40 died, and the only existing connection between patients was their use of electronic cigarettes (e-cigarettes). The acronym EVALI ("e-cigarette, or vaping, product use associated lung injury") has since been established for the condition. This review article is intended to provide an overview of recent, mainly US literature on EVALI, including the case definition, epidemiology, clinical presentation, typical disease progression, as well as potential triggers. Ancillary to this, the review further provides a general overview of the basic function of e-cigarettes, the ingredients of the liquids used in these (e-liquids), as well as a brief description of the associated potential inhalation risks.
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Lesión Pulmonar Aguda/inducido químicamente , Dronabinol/efectos adversos , Sistemas Electrónicos de Liberación de Nicotina , Vapeo/efectos adversos , Lesión Pulmonar Aguda/epidemiología , Cannabidiol/administración & dosificación , Cannabidiol/efectos adversos , Brotes de Enfermedades , Dronabinol/administración & dosificación , Humanos , Nicotina/administración & dosificación , Nicotina/efectos adversos , Factores de RiesgoRESUMEN
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.
Asunto(s)
Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/terapia , Pulmón/diagnóstico por imagen , Guías de Práctica Clínica como Asunto , Biopsia , Humanos , Fibrosis Pulmonar Idiopática/patología , Pulmón/patología , Enfermedades Pulmonares Intersticiales , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Interstitial lung diseases (ILD) encompass different heterogeneous, mainly chronic diseases of the pulmonary interstitium and/or alveoli with known and unknown reasons. The diagnostic of ILD is challenging and should be performed interdisciplinary. The medical history is of major importance and therefore, in German-speaking countries the Frankfurter Bogen (published in 1985) was utilised to scrutinise the medical history of the patient. This by now more than 30-years-old questionnaire requires a revision with regard to content and language. METHOD: Under the auspices of the clinical section of the DGP the new Interstitial Lung Disease Patient Questionnaire was developed in collaboration amongst pulmonologist, occupational medicine physicians and psychologists and supported by patient support groups. The questionnaire was finally optimised linguistically with the help of patients. RESULTS: The newly developed patient questionnaire for interstitial and rare lung diseases encompasses different domains: initial and current symptoms, medical history questions including prior drug treatments, previous pulmonary and extrapulmonary diseases, potential exposition at home, work and leisure time as well as family history and travelling. CONCLUSION: The newly developed questionnaire can facilitate the diagnosis in patients with suspicion on interstitial lung disease in clinical routine.
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Enfermedades Pulmonares Intersticiales/diagnóstico , Encuestas y Cuestionarios , Adulto , Humanos , PulmónRESUMEN
In October 2016, a group of German IPF experts were invited by Boehringer Ingelheim to meet in Frankfurt with the aim, (a) to discuss relevant aspects of the management and treatment of idiopathic pulmonary fibrosis (IPF) using nintedanib; and, (b) to provide supportive advice for daily clinical practice with nintedanib. The resulting information compiled in this document is confined to practical issues regarding the use of nintedanib in patients with IPF. Where different therapeutic options were available, the choice of IPF medication was not discussed and the experts alluded to current guidelines for the diagnosis and treatment of IPF.The participants discussed a comprehensive spectrum of clinical questions related to 10 different topics, including patient-related aspects at initiation of IPF therapy, the treatment of anticoagulated IPF patients, and the handling of nintedanib-related adverse events such as gastrointestinal side effects and elevated liver enzymes. In addition, the experts evaluated therapeutic options for IPF patients with continuous disease progression, clinical scenarios that justify discontinuation of nintedanib treatment, and therapeutic options for IPF patients with an acute exacerbation or severe infection. Finally, the participants discussed the handling of nintendanib before/after elective surgical intervention (e.âg. lung transplantation) and the current evidence for antifibrotic combination therapy in patients with IPF.For each topic discussed, the resulting information incorporates published evidence from clinical trials. In case of insufficient or lacking evidence, the experts have formulated recommendations based on their personal clinical experience and evaluation.
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Competencia Clínica , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Indoles/uso terapéutico , Anticoagulantes/efectos adversos , Anticoagulantes/uso terapéutico , Terapia Combinada , Comorbilidad , Progresión de la Enfermedad , Interacciones Farmacológicas , Hemorragia/inducido químicamente , Humanos , Indoles/efectos adversos , Trasplante de PulmónRESUMEN
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal pulmonary disease with an estimated 5-year survival of approximately 20%. Pirfenidone is a novel orally available antifibrotic agent that reduces disease progression and improves survival of patients with IPF. The most common adverse effects of pirfenidone include gastrointestinal symptoms, hepatic dysfunction or skin photosensitivity and rash. A 64-year-old male patient presented in our clinic with a strong generalized exfoliative erythema and intense itching accompanied by fatigue and mild fever after a mild sun exposure for 5 days during holidays in Turkey. The patient had been diagnosed with IPF 2 months ago and 1 month later he started a therapy with pirfenidone with good tolerability. OBJECTIVE: In this report, we noted a severe phototoxic reaction under treatment with pirfenidone which underlies the potential phototoxic effect of this drug besides the already reported photosensitivity. METHODS: Routine laboratory tests and a skin biopsy were performed. RESULTS: Laboratory tests indicated increased markers of inflammation. The skin biopsy showed a perivascular lymphocytic inflammatory infiltrate, ballooning of keratinocytes with increased apoptosis. These findings were most consistent with a severe phototoxic reaction to pirfenidone which had been directly discontinued. The patient was started on oral methylprednisolone 100 mg/day which was gradually tapered off along with topical corticosteroids (mometasone furoate 0.1% cream) and oral antihistamines. This treatment led to a slow but complete resolution of the skin lesions within 20 days. CONCLUSION: To our knowledge, this is the first reported case of a severe phototoxic reaction during treatment with pirfenidone. Our aim by presenting this case is to increase the awareness of clinicians for severe phototoxic effects of oral pirfenidone.
Asunto(s)
Antiinflamatorios no Esteroideos/farmacología , Fibrosis Pulmonar/tratamiento farmacológico , Piridonas/farmacología , Baño de Sol , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: The metastasis suppressor 1 (MTSS1) is a newly discovered protein putatively involved in tumour progression and metastasis. MATERIAL AND METHODS: Immunohistochemical expression of MTSS1 was analysed in 264 non-small-cell lung carcinomas (NSCLCs). RESULTS: The metastasis suppressor 1 was significantly overexpressed in NSCLC compared with normal lung (P=0.01). Within NSCLC, MTSS1 expression was inversely correlated with pT-stage (P=0.019) and histological grading (P<0.001). NSCLC with MTSS1 downregulation (<20%) showed a significantly worse outcome (P=0.007). This proved to be an independent prognostic factor in squamous cell carcinomas (SCCs; P=0.041), especially in early cancer stages (P=0.006). CONCLUSION: The metastasis suppressor 1 downregulation could thus serve as a stratifying marker for adjuvant therapy in early-stage SCC of the lung.
Asunto(s)
Carcinoma de Pulmón de Células no Pequeñas/patología , Regulación Neoplásica de la Expresión Génica , Neoplasias Pulmonares/patología , Proteínas de Microfilamentos/metabolismo , Proteínas de Neoplasias/metabolismo , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Pulmón de Células no Pequeñas/metabolismo , Regulación hacia Abajo , Femenino , Humanos , Neoplasias Pulmonares/metabolismo , Masculino , Persona de Mediana Edad , Pronóstico , Análisis de Supervivencia , Análisis de Matrices TisularesRESUMEN
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF.
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Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/terapia , Indoles/administración & dosificación , Piridonas/administración & dosificación , Antiinflamatorios no Esteroideos/administración & dosificación , Medicina Basada en la Evidencia , Humanos , Fármacos del Sistema Respiratorio/administración & dosificación , Resultado del TratamientoRESUMEN
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.
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Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/mortalidad , Guías de Práctica Clínica como Asunto , Espirometría/estadística & datos numéricos , Espirometría/normas , Capacidad Vital , Medicina Basada en la Evidencia , Alemania , Humanos , Incidencia , Pronóstico , Reproducibilidad de los Resultados , Medición de Riesgo/métodos , Sensibilidad y Especificidad , Espirometría/métodos , Tasa de SupervivenciaRESUMEN
BACKGROUND: The consensus classification of idiopathic interstitial pneumonia (IIP, also known as pulmonary fibrosis) in 2002 has undergone a significant revision in 2013. OBJECTIVES: What are the key points of the new consensus statement of the American Thoracic Society (ATS)/European Respiratory Society (ERS) from 2013 for the classification of pulmonary fibrosis? MATERIAL AND METHODS: Evaluation of the available literature on the subject of pulmonary fibrosis in Pubmed. RESULTS: The interdisciplinary approach (i.e. pulmonology, radiology and pathology) for the diagnosis of lung fibrosis subtypes is highlighted. Nonspecific interstitial pneumonia (NSIP) is now a separate specific form of pulmonary fibrosis. Smoking-associated respiratory bronchiolitis with interstitial lung disease (RB-ILD) is now frequently diagnosed without a lung biopsy on the basis of clinical findings, computed tomography and bronchoalveolar lavage. The heterogeneous course of idiopathic pulmonary fibrosis (IPF) is now recognized. The term "acute exacerbation" will now be used for acute attacks (< 30 days) in patients with chronic lung fibrosis (IPF and NSIP). It has been recognized that it is necessary to provide a clinical classification algorithm for the management of IIP cases. Pleuroparenchymal fibroelastosis (PPFE) is recognized as a rare new entity of pulmonary fibrosis. The rapidly evolving field of molecular markers is promising for improving the diagnostic approach. CONCLUSION: The 2013 ATS/ERS update is a supplement to the previous classification of lung fibrosis from 2002. It outlines progress in recent years and shows potential areas for future innovations.
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Enfermedades Pulmonares Intersticiales/clasificación , Enfermedades Pulmonares Intersticiales/diagnóstico , Guías de Práctica Clínica como Asunto , Terminología como Asunto , Tomografía Computarizada por Rayos X/normas , Humanos , Neumología/normas , Radiología/normas , Estados UnidosRESUMEN
On the occasion of the 50th anniversary of the Scientific Working Group for the Therapy of Lung Diseases (WATL) the history is described from its foundation to the present situation. Research topics during this long period are specified and the studies are briefly outlined. In the beginning, WATL was engaged mainly in studies on tuberculosis, later on, the spectrum of WATL was broadened considerably to diseases like sarcoidosis, pulmonary Langerhans' cell histiocytosis, pulmonary emphysema due to α1-antitrypsin deficiency, chronic obstructive bronchitis and bronchial asthma as well as nontuberculous mycobacterioses. Finally, realising that the methodological capabilities of WATL were not sufficient to conduct large trials in classical lung diseases considering current requirements, WATL has begun to acquire competence in rare lung diseases such as lymphangioleiomyomatosis and alveolar proteinosis. In addition, WATL is dedicated to educative aims by organising conferences on topics which are not part of main stream respiratory medicine.
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Comités Consultivos/organización & administración , Enfermedades Pulmonares/terapia , Neumología/tendencias , Alemania , HumanosRESUMEN
Sarcoidosis is a rare granulomatous disease of unknown aetiology belonging to the wide group of interstitial lung diseases.). Although the limitlessness of BAL fluid is debated, it remains one of the best matrices for studying the pathogenesis of sarcoidosis. Natural killer (NK) cells have been described in BAL fluid from sarcoidosis patients. Elevated NK cells in BAL fluid from sarcoidosis patients have been found to be associated with poor outcomes. In this study, NK cells were evaluated in BAL samples from sarcoidosis patients at the time of diagnosis and associated with clinical characteristics in order to evaluate their prognostic role. Of the 276 patients suspected to have sarcoidosis on the basis of clinical and radiological findings, 248 had a final diagnosis of sarcoidosis. Clinical parameters, Scadding stage, and extrapulmonary localization were collected in a database. It resulted in fibrotic sarcoidosis patients being associated with an increase in lymphocyte percentages in BAL samples, particularly NK cells when compared with other groups. From ROC analysis, NK cell percentages in BAL samples resulted as being the best predictive markers in discriminating stage 4 of sarcoidosis from other RX stages (AUC=0.85, p<0.0001). Furthermore, after the stratification of patients on the basis of the number of extrapulmonary localizations, patients with an higher number of extrapulmonary localizations also showed higher percentages of NK cells in BAL fluid. In conclusion, NK cell percentages in BAL fluid can be considered a good prognostic marker of fibrotic phenotypes of sarcoidosis and involvement of other organs, although their diagnostic utility was poor.
RESUMEN
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.
Asunto(s)
Antiinflamatorios/uso terapéutico , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/terapia , Guías de Práctica Clínica como Asunto , Neumología/normas , Tomografía Computarizada por Rayos X/métodos , Alemania , Humanos , Fibrosis Pulmonar Idiopática/sangre , InternacionalidadRESUMEN
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.
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Diagnóstico por Imagen/métodos , Granuloma del Sistema Respiratorio/diagnóstico , Enfermedades Pulmonares/diagnóstico , Enfermedades Profesionales/diagnóstico , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Diagnóstico Diferencial , HumanosRESUMEN
Over the past few years an increasing number of prospective controlled sarcoidosis treatment trials have been completed. Unfortunately, these studies utilize different endpoints making comparisons between studies difficult. At the recent World Association of Sarcoidosis and other Granulomatous disease (WASOG) meeting, a session was dedicated to the evaluation of clinical endpoints for various disease manifestations. These included pulmonary, pulmonary hypertension, fatigue, cutaneous, and a classification of clinical disease phenotypes. Based on the available literature and our current understanding of the disease, recommendations for clinical evaluation were proposed for each disease category. For example, it was recommended that pulmonary studies should include changes in the forced vital capacity. Additionally, it was recommended that all trials should incorporate measurement of quality of life.
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Ensayos Clínicos como Asunto/métodos , Manejo de la Enfermedad , Sarcoidosis Pulmonar/diagnóstico , Sarcoidosis Pulmonar/terapia , Humanos , Calidad de Vida , Pruebas de Función Respiratoria , Índice de Severidad de la EnfermedadRESUMEN
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.
Asunto(s)
Comités Consultivos , Predisposición Genética a la Enfermedad , Neumología , Sarcoidosis Pulmonar , Adolescente , Adulto , Anciano , Niño , Congresos como Asunto , Diagnóstico Diferencial , Femenino , Genotipo , Humanos , Masculino , Persona de Mediana Edad , Morbilidad , Fenotipo , Estudios Retrospectivos , Sarcoidosis Pulmonar/diagnóstico , Sarcoidosis Pulmonar/epidemiología , Sarcoidosis Pulmonar/genética , Adulto JovenRESUMEN
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.