Manifestações intratorácicas das doenças do colágeno na tomografia computadorizada de alta resolução do tórax

Abstract

Intrathoracic manifestations of collagen vascular diseases are very common. The frequency of intrathoracic manifestations and the patterns of abnormality are variable depending on the type of collagen vascular disease and may simultaneously involve one or more of the following: lung parenchyma, airways, pulmonary vessels, pericardium, and pleura. The most common pulmonary manifestations are diffuse interstitial pneumonia and pulmonary hypertension which together represent the main causes of morbidity and mortality of these patients. Pulmonary, airway and pleural involvement may also be secondary to the disease therapy, or result from bacterial pneumonia or opportunistic infection. In the present review, the authors summarize the main intrathoracic manifestations of collagen vascular diseases and the differential diagnosis on high-resolution chest computed tomography. Keywords: Lung; Collagen vascular disease; Interstitial pneumonia; Pulmonary hypertension; Computed tomography.As manifestações intratorácicas das doenças do colágeno são bastante comuns. O padrão e a freqüência de comprometimento dependem do tipo específico de doença do colágeno, que pode envolver um ou vários compartimentos simultaneamente, tais como parênquima, vias aéreas, artérias pulmonares, pleura, e pericárdio. As manifestações mais importantes incluem as pneumonias intersticiais difusas e a hipertensão pulmonar, que em conjunto representam as principais causas de mortalidade e morbidade nesses pacientes. O acometimento pulmonar, pleural e de via aérea pode ser também secundário a terapêutica instituída ou ser decorrente de processos infecciosos bacterianos ou por germes oportunistas, por causa da imunossupressão. Nesta revisão os autores sumarizam as manifestações intratorácicas e o diagnóstico diferencial das principais doenças do colágeno na tomografia computadorizada de alta resolução do tórax. Unitermos: Pulmão; Doença do colágeno; Pneumonia intersticial; Hipertensão pulmonar; Tomografia computadorizada. AbstractResumo

Pulmonary Manifestations of Rheumatologic Diseases in Pediatric

“…Therefore, the systematic investigation of patients with diseases known to cause lung damage should be precocious. The most sensitive and specific method for detection of pulmonary involvement is the chest HRCT, which is able to show abnormalities even when CXR and PFTs are normal [19,20,[26][27][28].…”

“…Periodic monitoring is mandatory in these patients, with history and physical examination, in order to assist the health professional to decide on the need for additional pulmonary investigation. The gold standard is the chest HRCT, because of its high sensitivity and specificity to detect pulmonary abnormalities [19,20,[26][27][28]. This test, however, does not assess lung function.…”

“…For this purpose, PFTs should be ordered. The CXR has significant limitations in detection of subtle alterations, presenting a considerable amount of false negatives [26][27][28]. There is a variety of pulmonary manifestations in the rheumatic diseases and there are no pathognomonic signs of pulmonary disease in each [1,5,7].…”

Case 3 - Congestive Heart Failure in Male with Systemic Sclerosis

“…In this case, the pulmonary impairment pattern demonstrated by the chest computed tomography is not the most common one in DSS. The most frequent is the non-specific interstitial pneumonia 8 - 10 . However, we cannot discard that the pulmonary impairment identified is a result of chronic inflammatory changes in pulmonary interstice caused by the disease, which are more active ("ground glass" pattern) and already show older changes, such as pulmonary fibrosis which, by traction, induce bronchiectasis and subpleural thickenings, classifying the tomographic findings as acute exacerbation of the chronic evolution interstitial pneumonia, common on collagenosis 8 , 11 .…”

Which is your diagnosis?

“…Pulmonary involvement and the consequential respiratory compromise are observed in only 10% of patients and may primarily occur either in the form of interstitial lung disease or by weakness of respiratory muscles (6,7) .…”