Airway obstruction in bronchial sarcoidosis: outcome with treatment.

STUDY OBJECTIVE: Airway obstruction (AO) in sarcoidosis is reported to be associated with respiratory symptoms, increased morbidity, and an increased mortality risk. Because AO in sarcoidosis may result from several causes, the therapeutic benefit of corticosteroids is difficult to determine. The aim of this study was to evaluate the therapeutic response of AO attributable to sarcoid granulomas in the bronchial wall. PATIENTS: We selected 11 patients who had sarcoidosis with AO (defined as FEV(1)/vital capacity [VC] < 70%) associated with sarcoid granulomas on an endobronchial biopsy. Exclusion criteria were history of asthma, smoker or ex-smoker, stage 4 disease, evidence of extrinsic compression by enlarged lymph nodes, and localized endobronchial stenosis seen during fiberoptic bronchoscopy. INTERVENTIONS: We compared the results of pulmonary function tests and clinical, radiologic, and biological findings at baseline with those obtained at the time of the last pulmonary function tests available, between the sixth and 12th months of treatment. Eight patients took oral corticosteroids (20 to 60 mg/d initially), one received IV methylprednisolone pulses, another took oral hydroxychloroquine, and the last one received IM methotrexate. MEASUREMENTS AND RESULTS: With treatment, FEV(1) and FEV(1)/VC significantly improved in eight patients (72%), normalized in four patients, and was unchanged in the remaining three patients. The mean FEV(1) increased from 60.8 +/- 10.8% to 76 +/- 13.7% of the predicted value (p < 0.02). VC did not change significantly. FEV(1)/VC increased from 76.1 +/- 6.4% to 87.6 +/- 10.7% of the predicted value (p < 0.01). Dyspnea on exertion and other clinical findings were attenuated in 10 patients; the chest radiograph improved in 9 patients, and normalized in 5 patients. The mean serum angiotensin-converting enzyme level decreased from 112 +/- 48 to 58 +/- 40 IU/mL (p < 0.05), and normalized in four patients. CONCLUSION: The present study indicates that AO caused by sarcoid granulomas in the bronchial wall can be either partially or completely reversed by treatment with a concomitant attenuation of pulmonary symptoms.

Bilateral hemothorax revealing mediastinal parathyroid adenoma.

We report the case of a 63-year-old woman admitted to hospital because of bilateral hemothorax associated with acute respiratory failure and laterotracheal neoformation. A right thoracoscopy biopsy revealed a paratracheal parathyroid adenoma which was responsible for bilateral hemothorax and primary hyperparathyroidism. A curative resection was successfully performed by cervicotomy.

[Chronic pseudo-hypertrophic myopathy in sarcoidosis. Apropos of 3 cases]. / Myopathie chronique pseudo-hypertrophique au cours de la sarcoïdose. A propos de 3 cas.

We report three cases of pseudohypertrophic chronic myopathy in patients with sarcoidosis. The patients were aged 41-50 years and were of African or mixed ethnic origin. All three patients had multiorgan sarcoidosis recently diagnosed by the presence of hiliary and/or mediastinal node enlargement with or without reticulomicronodulation in the lung fields. Hypertrophy of the proximal portion of the limbs dominated the clinical presentation. In 2 cases, the myopathy was preceded by myalgia and in 2 cases associated with muscle nodules. The muscle enzymes were elevated in 2 cases. The importance of tuberculoid granulomas and the discretion of the myopathy characterised the histological findings in all 3 cases. Cortisone therapy was given early and long-term treatment led to favorable course in 2 cases, probably due to the importance of the granulomatous muscle mass. In the third case, peripheral neuropathy developed.

Exogenous lipid pneumonia: a retrospective multicentre study of 44 cases in France.

A nationwide retrospective study of exogenous lipid pneumonia (ELP) was carried out to update the data on this disease, with emphasis on thoracic computed tomography (CT) scan and bronchoalveolar lavage (BAL) findings. The inclusion criteria were: 1) presence of abnormal imaging features compatible with the diagnosis of ELP; 2) presence of intrapulmonary lipids; and 3) exogenous origin of the lipid pneumonia. Forty four cases were included (20 males and 24 females; mean age 62 +/- 11 yrs), of which four were occupational (chronic inhalation of cutting mist or oily vapour in an industrial environment). Thirty of the 40 nonoccupational cases were related to aspiration of liquid paraffin used for the treatment of constipation. A condition possibly favouring oil aspiration or inhalation was present in 34 patients (77%), most commonly gastro-oesophageal reflux (n = 20) and neurological or psychiatric illness (n = 14). Fever (39%), weight loss (34%), cough (64%), dyspnoea (50%) and crepitations (45%) were the most frequent symptoms. BAL was performed in 39 cases: 23% had a lymphocytic alveolitis; 14% neutrophilic alveolitis; and 31% a mixed alveolitis (lymphocytic and neutrophilic). Alveolar consolidations (57%), ground glass opacities (39%), and alveolar nodules (23%) were the most common radiological abnormalities. The changes were bilateral (79%), predominant in the posterior and lower zones of the lobes concerned (74%), hypodense (71%), and spared the subpleural zones (52%). In 13 cases, hypodensity was retrospectively established on CT scan by the presence of a "positive angiogram". This sign may be of diagnostic value when the density measurement is either not possible or not reliable. In conclusion, this study provides an update of the clinical, biological and radiological profile of exogenous lipid pneumonia and, in particular, confirms the diagnostic benefit of computed tomography scan, which revealed bilateral and hypodense changes in a large majority of cases.

[Hypersensitivity pneumopathies: x-ray computed tomographic aspects]. / Les pneumopathies d'hypersensibilité: aspects tomodensitométriques.

Chest radiographs and computed tomographic scans obtained from nineteen patients with hypersensitivity pneumonitis were reviewed retrospectively. Thirteen of them had a subacute form. Six of them had a chronic form. In subacute forms, CT scans were normal in two patients. In the 11 other cases, CT findings were ground glass attenuation (n = 10), nodules (n = 7) and linear opacities (n = 5). Ground glass attenuation was diffuse or patchy. Two patterns are quite suggestive of hypersensitivity pneumonitis: the association of micronodules and ground glass attenuation and the presence of hyperlucent secondary lobules within the ground glass. After cessation of exposure, CT showed a return to normal or improvement. Ground glass opacities, nodules and also linear opacities disappeared. CT scans were always abnormal in chronic forms. CT findings were linear opacities (n = 6), ground glass attenuation (n = 4), micronodules (n = 2) and cysts (n = 3).

[Peritoneal pseudomyxoma with pleural metastases. A case report]. / Pseudomyxome péritonéal avec métastases pleurales. A propos d'un cas.

The authors report a case of a 69 year old man with a peritoneal pseudo-myxoma probably secondary to a mucocele of the appendix and complicated by pleural metastases which were nodular, unilateral and asymptomatic. These were discovered on computerised tomography during examination for the recurrence of the peritoneal disease. A study of the histology of the pleural fragments obtained by thoracotomy showed the presence of lesions which were identical to those found in the peritoneal masses.

[Necrotizing sarcoid granulomatosis. Apropos of 4 cases]. / Granulomatose nécrosante sarcoid-like. A propos de 4 observations.

Necrotizing sarcoid granulomatosis (NSG) was first described by Liebow in 1973 among the heading "pulmonary angeitis and granulomatosis". We reported four cases of NSG. The diagnostic was made by open lung biopsy in all, and the distinctive features were those described by Liebow: presence of numerous sarcoid-like granulomas, foci of fibrinoid necrosis and granulomatous vasculitis. Clinical, radiological, and clinical course characteristics allow the reasonable conclusion that NSG is the histopathological substratum of a variety of sarcoidosis affections termed "nodular sarcoidosis".

[Chronic diffuse interstitial pneumonia associated with chronic active hepatitis. 2 cases]. / Pneumopathie interstitielle diffuse chronique associée à une hépatite chronique active. 2 observations.

Two cases of chronic active hepatitis associated with diffuse interstitial pneumonia (lymphoid in one patient, lymphoid and fibrosing in the other) are reported. Histopathological data from the lungs, together with the parallel course of pulmonary and hepatic manifestations observed under corticosteroid therapy, suggest that these two diseases shared a common dysimmune pathogenesis. A few cases identical with these have been found in the literature. The clinical, laboratory and histopathological elements obtained from these cases also suggest an immune cause in most patients.

Bronchogenic carcinoma in patients with pulmonary histiocytosis X.

Five cases of bronchogenic carcinoma were observed among 93 patients with pulmonary histiocytosis X (Hx). Mean age at the time of diagnosis of Hx was 42 years; on the average, cancer occurred 10.5 years later. All patients were smokers and continued to smoke heavily at the time of diagnosis of cancer. Comparison of the five cases associating Hx and lung carcinoma with a group of 88 control patients suffering from Hx alone suggested that smoking played the predominant role in the pathogenesis of cancer. In fact, among the four patients with Hx and carcinoma older than 45 years, tobacco consumption was significantly greater (64.7 +/- 37 pack-year, mean +/- SD) than that of the 15 control patients of the same age with only Hx (40.8 +/- 11.6, p less than 0.01). In light of this good correlation, the diagnosis of Hx strongly advocates stopping tobacco smoking and long-term medical follow-up.

[CD8+T-cell lymphocytic alveolitis in sarcoidosis. 4 cases]. / Alvéolite lymphocytaire à lymphocytes T CD8+ au cours de la sarcoïdose. 4 observations.

We report 4 cases of histologically proven sarcoidosis with lymphocytic alveolitis. The T CD4+/T CD8+ ratio was below 1 in the bronchoalveolar lavage fluid. Since there was no clinical and paraclinical evidence of extrinsic allergic alveolitis or HIV infection, and since 3 of the patients were nonsmokers and the 4th one smoked moderately, we considered that the T CD8+ lymphocytic alveolitis was related to the sarcoidosis. In 3 patients the presence of mediastinal adenopathy suggested that sarcoidosis was of recent onset. The fact that T CD4+/T CD8+ ratio was normal in blood indicated that T CD8+ lymphocytes were predominant only in the lung. Thus, the finding of T CD8+ lymphocytic alveolitis should not exclude a diagnosis of sarcoidosis.

[Chronic idiopathic eosinophilic pneumopathy. Carrington's disease]. / Pneumopathie chronique idiopathique à éosinophiles. Maladie de Carrington.

Eleven cases of idiopathic chronic eosinophilic pneumonia were studied. Ten of the patients were followed up for more than 3 years and the last one for 15 years. The female predominance observed, the mean age of 51 years, the late manifestations of asthma-like symptoms preceding the systemic signs by 8 1/2 months on average and the scarcity of extra-pulmonary manifestations characterized the clinical data. The most frequent radiological signs were non-systematized, bilateral alveolar opacities in the axillary and apical regions, sometimes migratory and giving in 9 cases a mirror image of pulmonary oedema. Beside this major inflammatory syndrome, blood eosinophilia was present in 10 patients with 4,500 eosinophils on average per cubic millimetre. Eosinophils were also present in the bronchoalveolar lavage fluid of those who underwent this examination. Both clinical and radiological signs responded to corticosteroids in all cases, with return to normal chest radiography within less than 10 days. Subsequently, the possibility of radiological relapse in the same or other territories, together with the development of a steroid-dependent intrinsic asthma accounted for the fact that after a follow-up of about 3 years corticosteroid therapy could not be discontinued in any of our patients.

[Predominantly bullous pulmonary sarcoidosis complicated by aspergillosis]. / Sarcoïdose pulmonaire, à forme bulleuse dominante, compliquée de greffe aspergillaire.

The authors report a case of multivisceral sarcoidosis which started in childhood and presented at radiography as multiple air-filled round cavities without signs of pulmonary fibrosis. The course of the disease was marked by progression of the bullae from apex to base and by the development of intracavitary aspergillosis responsible for haemoptysis.

[Bronchiolitis obliterans with cryptogenetic-like organizing pneumonia. Demonstration of gastro-esophageal reflux in 5 cases]. / Bronchiolite oblitérante avec pneumonie en voie d'organisation en apparence cryptogénétique. Mise en évidence d'un reflux gastro-oesophagien dans 5 cas.

Gastro-oesophageal reflux was demonstrated in 5 cases of bronchiolitis obliterans with apparently cryptogenetic organizing pneumonia. In addition, one patient had hiatus hernia and another, oesophageal diverticulum. In 4 patients, after failure of prolonged antibiotic therapy, medical or surgical treatment of the gastro-oesophageal reflux resulted in regression of the clinical and radiological signs of pulmonary lesions. The cure thus obtained persisted throughout a follow-up period of 2 months to 8 years. Gastro-oesophageal reflux therefore could be one of the causes of apparently cryptogenetic bronchiolitis obliterans with organizing pneumonia, and all patients with this respiratory disease should be investigated for gastro-oesophageal reflux.

[Early postoperative thrombosis of an aortic Saint-Jude prosthesis. Success of fibrinolytic treatment]. / Thrombose post-opératoire précoce d'une prothèse de Saint-Jude en position aortique. Succès du traitement fibrinolytique.

We are reporting the case of a 63 year-old woman presenting an early thrombosis of a mechanical aortic Saint-Jude prosthesis, on the 21st post-operative day, following an insufficient anti-coagulant treatment and discovered by the presence of a well tolerated murmur of aortic insufficiency. The treatment consisted in fibrinolysis using urokinase, administered intravenously at a dose of 4,400 IU/kg/hour, for 12 hours. The clinical, ultrasound and radiocinematographic control performed on the 75th day, were normal; the patient, at that time being treated with anti-vitamin K and platelets anti-aggregates. No complication was observed. This case demonstrates the advantages of fibrinolytic treatments in early thrombosis following insertion of a valvular prosthesis.