Organising pneumonia is defined histopathologically by intra-alveolar buds of granulation tissue, consisting of intermixed myofibroblasts and connective tissue. Although nonspecific, this histopathological pattern, together with characteristic clinical and imaging features, defines cryptogenic organising pneumonia when no cause or peculiar underlying context is found. Rapid clinical and imaging improvement is obtained with corticosteroid treatment, but relapses are common after stopping treatment.
This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema.A retrospective multicentre study was conducted in 40 patients (38 males; age 68¡9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart catheterisation.Dyspnoea was functional class II in 15%, III in 55% and IV in 30%. 6-min walk distance was 244¡126 m. Forced vital capacity was 86¡18%, forced expiratory volume in 1 s 78¡19%, and carbon monoxide diffusion transfer coefficient 28¡16% of predicted. Room air arterial oxygen tension was 7.5¡1.6 kPa (56¡12 mmHg). Mean pulmonary artery pressure was 40¡9 mmHg, cardiac index 2.5¡0.7 L?min -1 ?m -2 and pulmonary vascular resistance 521¡205 dyn?s?cm -5. 1-yr survival was 60%. Higher pulmonary vascular resistance, higher heart rate, lower cardiac index and lower carbon monoxide diffusion transfer were associated with shorter survival.Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catheterisation have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe haemodynamic parameters.
All types of sarcomas may be observed in the heart with a predominance of undifferentiated sarcomas. Histological grading, unlike histological type, seems to correlate with survival which remains extremely poor.
Eosinophilic pneumonias (EP) encompass a wide spectrum of lung diseases characterized by peripheral blood eosinophilia (>1 × 109 eosinophils/l) and/or alveolar eosinophilia (>25%). Blood eosinophilia may be lacking, as in the early phase of idiopathic acute EP, or in patients already taking oral corticosteroids. EP may present with varying severity, ranging from almost asymptomatic infiltrates to the acute respiratory distress syndrome necessitating mechanical ventilation. Possible causes of EP must be thoroughly investigated, especially drugs and the variety of parasitic infections (considering history of travel or residence in areas of endemic parasitic infection). However, chronic EP remains idiopathic in many cases. When present, extrathoracic manifestations lead to suspect Churg–Strauss syndrome (CSS) or the hypereosinophilic syndrome (HES), the prognosis of which is dominated by cardiac involvement. Apart from the treatment of specific causes when possible, corticosteroids remain the cornerstone of symptomatic treatment for eosinophilic disorders, usually with a dramatic response, but frequent relapses when tapering or after stopping the treatment. The adjunction of immunosuppressants to corticosteroids is necessary in patients with CSS and poor prognosis factors. Imatinib has recently proven effective in the treatment of the myeloproliferative variant of the HES.
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