Jean-François Cordier

The Syndrome of Combined Pulmonary Fibrosis and Emphysema

Combined pulmonary fibrosis and emphysema (CPFE) is a recently identified syndrome occurring almost exclusively in smokers or ex-smokers, characterized by severe dyspnea and limitation to exercise capacity. Tobacco smoking is the main etiologic factor. However, it may occur in the context of connective tissue disease especially rheumatoid arthritis and systemic sclerosis. Velcro-crackles are present at lung auscultation. CPFE may be overlooked due to preserved lung volumes and subnormal spirometry, however gas exchange is severely impaired, with hypoxemia at exercise. The diagnosis is based on the presence at HRCT of both emphysema predominating in the upper lobes and frequently paraseptal, and interstitial abnormalities suggesting pulmonary fibrosis in the lower zones of the lungs, with especially reticulation, traction bronchiectasis, honeycombing, and possible ground glass opacities. Thick-walled large cysts are another distinctive feature at imaging. Pathologic radiological correlations are difficult owing to various pathology and difficulties in identifying honeycombing at chest HRCT in the setting of coexistent emphysema. Lung pathology when available may show a pattern of usual interstitial pneumonia, however other patterns can also be found. Survival is generally comparable to that of idiopathic pulmonary fibrosis (without emphysema), however with an increased risk of precapillary pulmonary hypertension associated with a dismal prognosis. Lung cancer and acute exacerbation of pulmonary fibrosis may further hamper the prognosis. The CPFE syndrome impacts modalities of follow-up with a relative lack of restrictive physiology despite progression of disease, and the need to monitor for pulmonary hypertension. Management is mostly supportive, with smoking cessation, inhaled bronchodilators, and long-term oxygen therapy or lung transplantation when appropriate. Drug therapy with pirfenidone or nintedanib, which reduce the decline in lung function in idiopathic pulmonary fibrosis, might be used pending further evaluation. There are no data to support treatment specific for pulmonary hypertension in CPFE outside clinical studies or prospective registries.

Idiopathic chronic eosinophilic pneumonia and asthma: how do they influence each other?

Since idiopathic chronic eosinophilic pneumonia (ICEP) and asthma are frequently associated, their possible reciprocal influence on clinical presentation and evolution were investigated. The clinical and follow-up features of 53 cases of ICEP, of which 41 (77%) had asthma, were reviewed retrospectively. Asthma preceded the diagnosis of ICEP in 26 patients, was contemporaneous in eight patients, and developed 17±12 months after ICEP in seven patients. Presentation of ICEP was similar in asthmatics and nonasthmatics with the exception of a higher level of total immunoglobulin E in the former group. Patients with asthma at the time of diagnosis of ICEP were more likely to remain free of relapse of ICEP (56 versus 23%) and had a lower number of relapses per year of follow-up (median 0 versus 0.24). Moreover, they were treated more frequently with long-term inhaled corticosteroids (88 versus 31%) at last follow-up. Asthma got worse after the diagnosis of ICEP and frequently required long-term oral corticosteroids. To conclude, among patients with idiopathic chronic eosinophilic pneumonia, asthmatics have a lower frequency of relapse than nonasthmatics, possibly because of a higher use of inhaled corticosteroids. The occurrence of idiopathic chronic eosinophilic pneumonia in asthmatics is often associated with the development of severe asthma.

Pneumopathies interstitielles diffuses idiopathiques

INTRODUCTION The classification of the idiopathic interstitial pneumonias includes seven clinico-pathologic entities. The diagnosis is based on a multidisciplinary approach, integrating the clinical evaluation, the high-resolution computerised tomography, and the pathological pattern. STATE OF THE ART A definitive diagnosis of idiopathic pulmonary fibrosis relies on the association of a suggestive clinico-radiological profile and a pathological pattern of usual interstitial pneumonia. Nonspecific interstitial pneumonia is a recently described clinico-pathologic entity, with a better prognosis than that of idiopathic pulmonary fibrosis. Cryptogenic organising pneumonia has been included in the group of idiopathic interstitial pneumonias because of its idiopathic and multifocal characteristics, although it does not predominate in the lung interstitium. Desquamative interstitial pneumonia and respiratory bronchiolitis with interstitial lung disease are rare entities with predominance in young smoking adults. Lymphoid interstitial pneumonia, usually encountered in the context of Sjögrens syndrome, is very rare in its idiopathic form. Acute interstitial pneumonia is responsible for idiopathic acute respiratory distress syndrome. PERSPECTIVES The current classification of idiopathic interstitial pneumonias better defines the diagnostic criterias of each clinico-pathologic entity, and is expected to facilitate clinical research. CONCLUSIONS This classification has clinical implications, with prognostic and therapeutic significance.

Infections opportunistes et sarcoïdose

Resume Introduction Chez les patients atteints de sarcoidose, malgre la frequence de la lymphocytopenie T-CD4, et l’immunodepression induite par la corticotherapie, les infections opportunistes semblent rares. Methodes Nous decrivons cinq cas de sarcoidose compliquee d’infection opportuniste. Une recherche bibliographique des cas rapportes d’infection opportuniste chez des patients atteints de sarcoidose, a ete effectuee. Resultats Parmi les 5 cas d’infection opportuniste que nous decrivons, quatre (une aspergillose necrosante chronique, une infection pulmonaire a mycobacterie atypique, une cryptococcose neuro-meningee, et une pneumocystose pulmonaire) sont survenus sous corticotherapie, et un seul (aspergillose necrosante chronique) chez une patiente non traitee. Dans 4 cas, il existait une lymphocytopenie T-CD4 au moment du diagnostic de l’infection. Dans la litterature, parmi les 65 cas documentes de sarcoidose compliquee d’infection opportuniste, 36 sont survenus chez des patients traites par corticoides. Une lymphocytopenie T-CD4 a ete notee dans 5 cas (sur 11 documentes). La cryptococcose a ete l’infection la plus frequemment rapportee. Conclusion Les infections opportunistes sont rares au cours de la sarcoidose, et surviennent principalement au cours d’un traitement par corticoides, dans un contexte de lymphocytopenie T-CD4. Sauf peut-etre pour la cryptococcose, la sarcoidose en elle-meme ne represente pas un facteur de risque infectieux.

Orphan Lung Diseases from Definition to Organisation of Care

The rare diseases have been called “orphan” because this term means that patients affected by such conditions feel that most physicians are reluctant to care for their disease because of its rarity, and that insufficient research is developed to improve diagnosis, clinical management, and especially the more distressing manifestations. The patients with rare diseases thus feel abandoned and orphan when comparing with patients with common diseases who benefit from extensive research worldwide and continuous drug development.

From Cancer Mimicking Orphan Lung Disease to Orphan Thoracic Oncology

A variety of rare neoplastic and non-neoplastic disorders may develop in the lung, the pleura, and the mediastinum. Some may have a propensity to mimic lung carcinoma as well as benign orphan lung diseases at some level of examination, as they may share with these clinical, imaging, pathological, and even molecular features. Challenges in the differential diagnoses among reciprocal mimics are well illustrated through examples as bronchioloalveolar carcinoma, primary pulmonary lymphomas, and vascular sarcomas. Pseudotumors have further been described, actually corresponding to a heterogeneous group of diseases characterised by circumscribed fibrous tissue and inflammatory cells. Among the inflammatory pseudotumors, neoplastic/non-neoplastic borderline disorders have been identified, such as inflammatory myofibroblastic tumor, which presents with clonal proliferation and has eventually emerged as a true neoplasm. Finally, some rare pulmonary diseases are emerging as borderline neoplastic non-neoplastic disorders, that require multidisciplinary expertise both in the field of orphan pulmonary diseases and in thoracic oncology, including amyloidosis or even Langerhans cell histiocytosis. Ultimately, implementing multi-disciplinary expert consensus is mandatory to determine the optimal management of these disorders.