RESUMEN
Pulmonary involvement is an important aspect in the management of rheumatic diseases. Connective tissue disease-associated interstitial lung diseases (CTD-ILD) are of particular importance, as their occurrence is often decisive for patient outcome. We use corticosteroids, immunosuppressants and eventually also biologicals in the therapy of CTD-ILD.Except for the "Scleroderma Lung Study" (SLS) I and II, which confirm the effectiveness of the immunosuppressive drugs cyclophosphamide (CYC) and mycophenolate mofetil (MMF) in pulmonary involvement of scleroderma (SSc-ILD), there is little data on the treatment of other CTD-ILD. Within the group of biologicals the use of Rituximab (RTX) increases in importance. The currently expected study results compare the efficacy of immunosuppressive drugs (in particular MMF and CYC) with RTX. Other investigated biologicals include Tocilizumab in SSc-ILD and Abatacept in pulmonary involvement in rheumatoid arthritis (RA-ILD). Autologous stem cell transplantation is a potent but potentially risky therapy for severe scleroderma.For the group of pulmonary interstitial diseases of different dignity, including CTD-ILD, with a increasingly fibrosing course despite adequate therapy, (e.âg. chronic hypersensitivity pneumonitis, sarcoidosis) the term and concept of "fibrosing interstitial lung diseases with progressive phenotype" (PF-ILD) is being established. In the last months studies were published, which show a positive effect of antifibrotic drugs (Nintedanib, Pirfenidon) in such constellations. Currently a number of other studies regarding the effectiveness of antifibrotics in CTD-ILD are expected to be published. The studies in this field bring new aspects in the understanding of interstitial diseases and have the potential of expanding treatment options in CTD-ILD.The topic of CTD-ILD is a difficult, but at the same time exciting field.
Asunto(s)
Enfermedades Pulmonares Intersticiales , Enfermedades Reumáticas , Corticoesteroides/uso terapéutico , Productos Biológicos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Humanos , Inmunosupresores/uso terapéutico , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/terapia , Enfermedades Reumáticas/complicaciones , Enfermedades Reumáticas/terapia , Trasplante AutólogoRESUMEN
Interstitial lung diseases compromise a group of diffuse predominantly chronic inflammatory and fibrosing disorders of the lung parenchyma. This article reviews practice-relevant publications on the diagnosis and treatment of idiopathic pulmonary fibrosis - the most common form of idiopathic interstitial lung diseases. The new patient questionnaire of the German Society of Pneumology facilitates collection of a complex case history of diverse interstitial lung diseases in individual patients. The Fleischner Society White Paper emphasize the importance of high-resolution computer tomography in the diagnosis of IPF. Histological diagnosis of the fibrotic changes is necessary in two situations: in case of CT findings indeterminate or suggestive for a diagnosis other than IPF and in case of inconsistence of medical history, laboratory findings and diagnostic imaging. The new international guidelines emphasize the importance of the interdisciplinary discussion prior to invasive diagnostic procedures, especially in the case of nonspecific interstitial lung changes. Bronchoscopy with lung cryobiopsy has gained importance in recent years. The recommendation to standardize this diagnostic method is speicified in a recently published expert opinion. The German Guideline for Idiopathic Pulmonary Fibrosis, published in 2017 suggests current therapeutic standards in this disease. The treatment with one of two registered antifibrotic drugs (pirfenidone, nintedanib) is to be started as soon as possible after the diagnosis and should be a long-term therapy. In case of intolerance or a significant progress of the disease despite the treatment, the therapy switching to an alternative drug may be considered. The combination of both therapies is not recommended. In current studies, new drugs are being tested (e.âg. the serum protein pentraxin 2, antibodies against the growth factor CTGF). On the other hand, the efficacy of the registered antifibrotic medicaments, nintedanib and pirfenidone, in interstitial lung diseases other than idiopathic pulmonary fibrosis is being evaluated.
Asunto(s)
Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/terapia , Humanos , Guías de Práctica Clínica como Asunto , Tomografía Computarizada por Rayos XRESUMEN
AIM: The analysis of the data from the psychiatric hospitals in Lubliniec (1894-1932, 1934-1936, 1970-1999) and Boleslawiec (1958-1999) proved a decrease in frequency of diagnosis of catatonic schizophrenia, what could testify to a decrease in morbidity with this form of schizophrenia. METHOD AND RESULTS: Basing on the facts from scientific literature there was ascertained that the decrease in frequency of diagnosis of catatonic schizophrenia is connected with following causes: firstly, with the changes in nozology, secondly, with the introduction of a new form of therapy and finally, with the fact that here psychological and sociological factors exist which eliminate catatonia as the most expressive form of schizophrenia.