U. Tylén

Interstitial lung disease in polymyositis and dermatomyositis: Longitudinal evaluation by pulmonary function and radiology

OBJECTIVE To estimate predictors and long-term outcome of interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM). METHODS We conducted a prospective study in which newly diagnosed PM/DM patients, regardless of clinical symptoms of pulmonary disease, were investigated with repeated chest radiography, high-resolution computed tomography (HRCT) of the lungs, and pulmonary function test (PFT). Clinical, radiologic, and lung function outcome was based on the last followup results. RESULTS Twenty-three patients with a mean followup period of 35 months were included. Findings on radiographic examination and/or PFT compatible with ILD were recorded in 18 patients (78%). Patients with ILD had lower lung function, higher radiologic scores, and higher creatine kinase values than those without ILD. All patients were treated with high-dose glucocorticoids and other immunosuppressive agents. Two patients died due to ILD, both with active myositis. During the followup, total lung capacity (TLC) improved in 33%, remained stable in 39%, and deteriorated in 28%. Changes in TLC correlated only partially with HRCT findings, which persisted even after normalizing for lung function. CONCLUSION ILD associated with PM/DM is in most cases mild, chronic, and has a nonprogressive course during immunosuppressive treatment. PFT can be normalized during treatment with immunosuppressive therapy, even if radiologic signs of ILD persist. The course of ILD could not be predicted on the first examination. Therefore, myositis patients with ILD need careful evaluation of clinical features as well as PFT and radiologic features during followup.

Chronic lower limb ischaemia. A prospective randomised controlled study comparing the 1-year results of vascular surgery and percutaneous transluminal angioplasty (PTA)

In a prospective randomised study, performed over a 6-year period, 102 patients with severe lower limb ischaemia or claudication resistant to exercise training were randomised either to percutaneous transluminal angioplasty (PTA) or vascular surgery. Only patients who could be treated by both methods were included, constituting only 5% of the total number of patients treated during this period. The two groups were similar regarding age, severity of symptoms and diabetes. The immediate and 1-year results showed similar success and complication rates. There was, however, a significantly shorter hospital stay for patients treated with PTA. Due to early complications and initial failures PTA should, however, only be used in institutions where vascular surgical facilities are available since PTA demands access to such treatment.

Survival of patients with disseminated midgut carcinoid tumors after aggressive tumor reduction

Abstract. Sixty-four consecutive patients with disseminated midgut carcinoids were treated during an 8-year period according to a single clinical protocol aimed at aggressive tumor reduction by surgery alone or with subsequent hepatic artery embolization. All patients had markedly elevated urinary 5-hydroxyindoleacetic acid (5-HIAA) levels (581 ± 79 μmol/24 h) and hormonal symptoms. Fourteen patients (22%) reached anatomic and biochemical cure by surgery alone. At follow-up, the mean 5-HIAA levels were still normal after 69.0 ± 6.2 months; two patients had died from unrelated causes. With the introduction of somatostatin receptor scintigraphy, subclinical disease was diagnosed in 7 of these 14 patients. Forty patients with bilobar hepatic disease underwent embolization in combination with octreotide. In this group, 5-HIAA levels were still reduced by 55% after 71 ± 11 months of follow-up, and the 5-year survival was 56%, estimated from the total death hazard function. After embolization, two subgroups could be identified with marked differences in their long-term response to treatment. Ten patients were not embolized owing to complicating diseases. The 5-year survival for the entire series was 58%. A significantly increased risk of cardiovascular deaths was seen, which underlines the importance of total survival analysis in a disease with multiple hormonal effects. It is concluded that an active surgical approach must be recommended to patients with the midgut carcinoid syndrome. In patients with bilobar hepatic disease, embolization combined with octreotide treatment markedly reduced the 5-HIAA excretion and suggested a prolonged 5-year survival.

Treatment of liver metastases of carcinoid tumors

Abstract. Liver metastases imply a major problem in patients with carcinoid tumors. Patients with localized disease should always undergo resection for cure. Patients with distant metastatic disease can also undergo resection for potential cure or symptom palliation because of the slow growth rate of many carcinoid tumors. In patients with the midgut carcinoid syndrome and bilobar hepatic disease we have performed primary surgery to relieve such symptoms as intestinal obstruction and ischemia, followed by successive embolizations of the hepatic arteries to reduce functional tumor burden in the liver. For optimal palliation, all patients with residual tumor were treated by octreotide. In a consecutive series of 64 patients with the midgut carcinoid syndrome we thus attained a 5-year survival rate of 70%. Fourteen of the patients underwent intentionally curative surgery (e.g., primary surgery followed by liver surgery). Of these patients, none died from their tumor disease during the period of study. The value of adjunctive interferon therapy is currently under evaluation.

Interventional Treatment of Gastrointestinal Neuroendocrine Tumours

Neuroendocrine (NE) tumours of the gastrointestinal tract (carcinoids and endocrine pancreatic tumours) are rare diseases. In the presence of liver metastases these patients may suffer from disabling symptoms due to hormone overproduction. Patients with localized disease can be resected for cure and also patients with liver metastases can undergo potentially curative tumour resection. However, long-term follow-up of the latter cases indicates frequent recurrence of tumour. Using close biochemical monitoring of tumour markers combined with newer techniques for tumour visualization, these recurrences can often be diagnosed at an early stage so that repeat surgical procedures can be performed. During the last years very active surgery has been recommended for NE tumours, many of which have a relatively slow growth. Even in patients not amenable to curative liver surgery, debulking can be considered if the main tumour burden can be safely excised. The primary aim of this type of treatment is palliation of hormonal symptoms. An important question is whether the aggressive treatment actually prolongs survival. No prospective studies have been performed. Such studies are hampered by the lack of strict surgical programs running over long periods and the relative rarity of NE tumours. Liver transplantation may be another treatment modality in selected cases.

Uneven distribution of ventilation in acute respiratory distress syndrome

IntroductionThe aim of this study was to assess the volume of gas being poorly ventilated or non-ventilated within the lungs of patients treated with mechanical ventilation and suffering from acute respiratory distress syndrome (ARDS).MethodsA prospective, descriptive study was performed of 25 sedated and paralysed ARDS patients, mechanically ventilated with a positive end-expiratory pressure (PEEP) of 5 cmH2O in a multidisciplinary intensive care unit of a tertiary university hospital. The volume of poorly ventilated or non-ventilated gas was assumed to correspond to a difference between the ventilated gas volume, determined as the end-expiratory lung volume by rebreathing of sulphur hexafluoride (EELVSF6), and the total gas volume, calculated from computed tomography images in the end-expiratory position (EELVCT). The methods used were validated by similar measurements in 20 healthy subjects in whom no poorly ventilated or non-ventilated gas is expected to be found.ResultsEELVSF6 was 66% of EELVCT, corresponding to a mean difference of 0.71 litre. EELVSF6 and EELVCT were significantly correlated (r2 = 0.72; P < 0.001). In the healthy subjects, the two methods yielded almost identical results.ConclusionAbout one-third of the total pulmonary gas volume seems poorly ventilated or non-ventilated in sedated and paralysed ARDS patients when mechanically ventilated with a PEEP of 5 cmH2O. Uneven distribution of ventilation due to airway closure and/or obstruction is likely to be involved.

High-resolution computed tomography in healthy smokers and never-smokers: a 6-year follow-up study of men born in 1933.

Purpose: To elucidate whether emphysematous lesions and other high‐resolution computed tomography (HRCT) findings considered associated with smoking are part of a progressive process, and to measure the extent to which similar changes are found in never‐smokers. Material and Methods: Healthy smokers and never‐smokers were recruited from a randomized epidemiological study and investigated with a 6‐year interval. Emphysema, parenchymal and subpleural nodules, ground‐glass opacities, bronchial alterations, and septal lines were evaluated in 66 subjects (40 smokers, 11 of whom had stopped smoking in the interval, and 26 never‐smokers). Lung function was tested. Results: All except emphysematous lesions were present to some extent in never‐smokers. Emphysema, parenchymal nodules, and septal lines occurred significantly more in current smokers, and a progression in extent of emphysema, ground‐glass opacities, bronchial alterations and septal lines was seen. There was no significant change among those who stopped and never‐smokers except for bronchial alterations, which progressed in never‐smokers. Conclusion: In healthy, elderly never‐smokers a low extent of various HRCT findings has to be considered normal. Emphysema, parenchymal nodules, and ground‐glass opacities are indicative of smoking‐induced disease. Further progress may cease if smoking is stopped.

Management of disseminated midgut carcinoid tumours

Forty-one patients with disseminated midgut carcinoid tumours were treated over a 6-year period according to a strict programme including primary surgical treatment. In 10 patients, a total remission of the disease was obtained. Patients with bilobar hepatic disease had ischaemic treatment of their liver metastases by hepatic arterial embolisation after primary surgical and medical treatment (low dose octreotide). Thus, by combining surgical, radiological and medical treatment modalities, we wanted to offer these patients optimal palliation. This treatment programme resulted in good symptomatic relief in all patients accompanied by a marked reduction in 5-hydroxyindoleacetic acid (5-HIAA) levels. At recurrence of symptoms in combination with rising 5-HIAA levels, embolisation was repeated. Ten of the treated patients have deceased during the observation period, but only 5 from their carcinoid disease.

SMS 201-995 and provocation tests in preparation of patients with carcinoids for surgery or hepatic arterial embolization

Patients with midgut carcinoids undergoing surgical resection or ischemic treatment of hepatic metastases by embolization are at risk for development of carcinoid crises due to release of hormonally active tumor products. Eight such patients were treated on nine separate occasions with increasing subcutaneous doses of a synthetic somatostatin analogue (SMS 201–995) 4 days prior to surgery or hepatic arterial embolization. The patients were tested by pentagastrin provocation and simultaneous measurement of serotonin (5-HT) levels in peripheral blood before and after prophylactic treatment, to evaluate the efficacy of SMS 201–995. The provoked release of 5-HT was markedly diminished, and the basal levels of 5-HT were markedly reduced in patients with high initial levels. During surgery or embolization both SMS 201–995, as well as ketanserin, a 5-HT2 receptor blacker, were given. With this combined treatment all patients were hemodynamically stable during surgery or embolization. During embolization the arterial levels of 5-HT increased only moderately, while urinary excretion of 5-hydroxyindoleacetic acid remained un-changed despite a proven adequate embolization. Two patients were operated on without previous treatment with SMS 201–995; both developed severe crises at induction of anesthesia, hut IV SMS 201–995 rapidly reversed the bronchoconstriction and facial flush and gradually restored arterial blood pressure, even though cardiac output remained depressed for a prolonged period. The crisis reaction correlated well with high circulating levels of 5-HT, but after treatment with SMS 201–995 these levels were still high. These findings indicate that the acute IV administration of SMS 201–995 can antagonize the peripheral actions of 5-HT and tachykinins, an effect different from the reduced release seen after prophylactic treatment with SMS 201–995. SMS 201–995 may block an intracellular mechanism common for activation of monoamine and tachykinin receptors on both tumor cells and normal cells, which would explain the observed effects. The prophylactic use of SMS 201–995 is recommended prior to surgery or hepatic arterial embolization in patients with disseminated midgut carcinoid tumors, because peripheral blockade of 5-HT, receptors alone is not sufficient to prevent a crisis reaction.

Recognizing emphysema - a neural network approach

An accurate and fully automatic method for detecting and quantifying emphysema in CT-images is presented. The method is based on an image preprocessing step followed by a neural network classifier trained to separate true emphysema from artifacts. The proposed approach is shown to be superior to an established method when applied on real patient data.