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RESUMO
O autor revê a terapêutica actual da doença sarcoide, essencialmente a do envolvimento pulmonar, mas também das manifestaçõoes multisistémicas mais frequentes. Na introdução tece comentários breves sobre epidemiologia, etiopatogenia, anatomia patológica e diagnóstico da doença sarcoide. Faz análise crítica dos indicadores de extensão e actividade da doença (radiografia e tomografia computorizada torácica, estudo funcional respiratório, enzima convertor de angiotensina, cintigrafia com Ga67 e lavado broncoalveolar).
Os corticosteroides são a terapêutica mais adequada, embora não haja unanimidade relativamente a quando iniciá-los, dose mínima eficaz, duração do tratamento e influência no curso do processo granulomatoso. Na sarcoidose pulmonar com sintomas respiratórios significativos, estudo funcional respiratório anormal e radiografias torácicas com alterações difusas, deve efectuar-se tratamento. Recomenda-se uma dose inicial de (prednisona/prednisolona) 0,5 mg/kg/dia em toma (mica durante 3 meses, progressivamente reduzida para 10-15 mg/dia e 12 a 18 meses de terapêutica. Têm sido experimentados outros fármacos: metotrexato, clorambucil, azatioprina, cidosporina, cloroquina.
Duas terapêuticas recentes são de considerar: corticosteróides inalados e transplante pulmonar. São considerados aspectos particulares na terapêutica da sarcoidose extrapulmonar (ocular, cutânea, cardíaca, neurológica, renal, gastrointestinal, hepática, óssea, pleural) e no controle da hipercalcemia e da hipercalciuria. A sarcoidose é uma doenç granulomatosa multisistémica com grande variabilidade de apresentação clfnica e evolução imprevisível. Globalmente, o prognóstico é bom. Pode acontecer: remissão espontânea ou terapêutica (60-70%); actividade persistente com lesoes residuals estáveis e repercussão clínico-funcional minima (20-30%); ou doença progressiva e incapadtante apesar de terapêutica corticosteroide (10-20%). A taxa de mortalidade é baixa (4%), sendo a fibrose pulmonar a mais frequente causa de morte.
SUMMARY
The author review the current concepts in the management of sarcoidosis, namely in the most common involved tissues (lungs and intrathoracic lymph nodes. It is done a brief commentary about incidence, etiology, pathogenesis and diagnosis of sarcoid disease. It is analized the techniques useful in assessing the extent and activity of sarcoidosis (chest radiographs, high resolution computorized tomography, pulmonary function tests, serum angiotensin – converting enzyme, Gallium67 scans, bronchoalveolar lavage).
Corticosteroides are generally considered to be adequated treatment of sarcoidosis, but there is no establish criterious about when to start, the minimal effective dose, the duration, and the effect of therapy on the course of the granulomatous process. All patients with respiratory symptoms, abnormal pulmonary function test and diffuse changes on chest radiographs need therapy. Generally an initial dose of 0,5mg/kg of prednisone daily during the first three months, progressively reduced to 10-15 mg/day is adequate. A course of 12 to 18 months is recommended. In patients who do not respond to tolerable doses of steroids, alternative drug therapy must be considered (methotrexate, chlorambucil, azathioprine, cyclosporine, chloroquine). Two recent therapies are under consideration: inhaled steroids and lung transplantation. It’s considered particular aspects of extrapulmonary sarcoidosis treatment (ocular, cutaneous, cardiac, neurologic, renal, gastrointestinal, hepatic, osseous) and hypercalcaemia, hypercalcinuria.
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology with diverse clinical presentation and a variable natural history. In general prognostic is good (spontaneous resolution is common). Mortality is low (4%) and pulmonary fibrosis is the most frequent cause of death.
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