[Churg-Strauss syndrome. Retrospective study of 20 cases]. / Le syndrome de Churg et Strauss. Etude rétrospective de vingt observations.

Churg-Strauss syndrome (CSS) is a disorder characterised by hypereosinophilia and systemic vasculitis complicating a preexisting asthma. Twenty cases have been studied. Mean duration of asthma before CSS was 8 years, the peripheral-blood eosinophilia, always > 1,700/microL, went above 5,000/microL in 17 cases. The clinical manifestations were the following: 20 impairements of general state with fever, 13 peripheral neuropathies, 15 cutaneous injuries, 10 pericardial or myocardial attacks, 10 digestive impairements, 9 muscular and articular diseases, 7 renal diseases--all of them linked with the vasculitis--and 9 upper respiratory tract involvements. Pleuropulmonary or cardiac anomalies have been discovered at the chest X-ray in 14 cases. The diagnosis has been histologicaly confirmed in 15 cases. No clinical or biological or evolutive distinction was noticed between the 15 positive biopsy patients and the 5 negative ones. During 8.4 (+/- 7.9 years) the evolution was caracterised by relapses which have always been announced by increasing eosinophilia. Eighteen patients have been successfully treated with corticoids alone or associated with cyclophosphamid in 9. Survival at 5 years was 85%. Five deaths occured because of CSS (2 because of an unadapted treatment). We have to focus the need for an emergency treatment of CSS. The diagnosis can be done by clinical investigation only, before any anatomopathologic results.

[Occupational asthma: current and future perspectives. The point of view of an expert]. / L'asthme professionnel: actualités et perspectives futures. Le point de vue de l'expert.

Occupational asthma remains a difficult problem for physicians and for experts. The prevalence of occupational asthma is underestimated and depends on industrial agent and conditions of work. Diagnosis is made based on detailed description of the patient's work environment, on skin and serological tests, and on lung function tests including non-specific bronchial provocation tests. Rhinomanometry, and peak flow rate measurements may also be useful. Bronchial provocation tests should be performed by experienced staff in a hospital setting with facilities for resuscitation. The prognosis of occupational asthma is variable: --some patients with short and limited exposure may fully recover if their disease is early recognized and appropriate measures are taken; --however, many patients do not recover completely after removal of the responsible agents. Clinical symptoms and bronchial hyperreactivity persist and respiratory insufficiency may develop. The patterns of occupational asthma are changing rapidly, in parallel with industrial evolution: nowadays, bakers's asthma is less frequent than it used to be. Asthma triggered by wood dust and in particular western red cedar (which is exported all over the world) is increasingly recognized. Isocyanates and, in particular toluene isocyanate, are widely used in plastics and paints. Preventive measures have succeeded in decreasing the incidence of asthma caused by exposure to isocyanates. Computer industry has recently developed in many countries. It requires the use of numerous hazardous and highly toxic materials that are potentially responsible for occupational asthma; attention should be given to respiratory diseases especially in industrializing countries (such as Eastern Asian countries). Preventive measures include safety rules, replacement of harmful materials by less toxic agents and detection of susceptible workers. Regulation must be defined under experts' control to continuously match the rapid changes in modern industry.

[Allergic angiitis with granulomatosis: Churg-Strauss syndrome. Retrospective study of 16 cases]. / L'angéite allergique avec granulomatose: syndrome de Churg et Strauss. Etude rétrospective de 16 observations.

Sixteen patients with Churg-Strauss syndrome (CSS), a disorder characterized by hypereosinophilia and systemic vasculities which complicate preexisting asthma, were analyzed. The mean duration of asthma before CSS was 8 years; peripheral blood eosinophilia was always greater than 1,900/microliters and exceeded 5,000/microliters in 14 cases. The clinical manifestations were the following: 16 in generally poor condition with fever; 12 peripheral neuropathies; 11 cutaneous lesions; 9 pericardial or myocardial involvement; 9 digestive disorders; 9 muscular or articular diseases; 5 renal involvement, all associated with the vasculitis; 7 upper respiratory tract disorders. Chest radiographs showed pleuropulmonary or cardiac anomalies in 11 patients. The diagnosis was confirmed histologically in 11 cases, however, no clinical, biological or evolutive differences were observed between these patients and those with negative biopsies (5). Follow-up for 6.35 +/- 5.55 years was characterized by relapses always preceded by increased eosinophilia. Fourteen patients were successfully treated with corticosteroids, associated with cyclophosphamide in 7 of them. Five-year survival was 87%. Four deaths occurred, all CSS-associated, two because of a poorly adapted therapeutic regimen. The need for rapid and effective treatment must be stressed. The diagnosis can be made based on clinical manifestations alone before histological confirmation can be obtained.

[Cervical tracheogenic air cyst. Apropos of 2 cases]. / Kyste trachéogénique aérien cervical. A propos de 2 observations.

The authors report 2 cases of gas-containing cervical cysts. Surgical excision by cervicotomy showed that both of these well-defined cysts were attached to the right border of the cervical trachea by a blind "pseudo-pedicle" and were lined with respiratory-type epithelium. These were 2 tracheogenic cysts, analogous to bronchogenic cysts theoretically linked to an abnormality in the development of the bronchial buds during embryogenesis. These two cases were exceptional not only by virtue of their cervical site, but also the fact that they contained air and occurred in already old women.

[Allergic granulomatosis and angiitis (Churg-Strauss syndrome) or bronchial asthma with periarteritis nodosa (author's transl)]. / Syndrome de Churg et Strauss ou asthme grave au cours de la périartérite noueuse. A propos de trois observations.

The coexistence of hypereosinophilic asthma and periarteritis nodosa, though rare, has been recognised for a long time (Wilson and Alexander, 1945). Based on clinical (asthma, hypereosinophilia) and histological (extravascular granulomas) criteria, Churg and Strauss, in 1951, described a single entity which they considered to be distinct from PAN, and which they called allergic and granulomatous angiitis. The authors describe three such cases, compare their findings with those in the published literature, and discuss the classification of Churg and Strauss' syndrome in relation to PAN. Apart from the asthma and hypereosinophilia, symptomatology is comparable with that of PAN, and histological examination demonstrates very similar vascular lesions in both affections. Extravascular granulomas do not appear to be truly specific. It would seem that there is a lack of essential features that could separate PAN from Churg and Strauss' syndrome, the preexistence of an asthmatic state perhaps simply modifying the PAN disorder in certain particular ways.

[Periarteritis nodosa and carcinoma of the colon. A case report (author's transl)]. / Périartérite noueuse et cancer du côlon. Une nouvelle observation.

It is extremely rare to observe the simultaneous progression of periarteritis nodosa (PAN) and cancer. A patient was found to have histologically confirmed PAN shortly after excision of an adenocarcinoma of the descending colon. High levels of carcino-embryonic and then HbS antigens were discovered in the serum. A favorable medium-term result was obtained after corticoids and multiple cytotoxic chemotherapy. If one accepts that the typical vascular lesions of PAN are due to an antigen-antibody conflict producing circulating immune complexes, it is reasonable to suggest the hypothesis that many antigens could be involved in the production of immune complexes of this type. Many authors accept that HbS antigen is implicated, certain tumoral antigens may also play an analagous role.

[Pulmonary actinomycosis grafted on former tuberculous lesions. Report of one case (author's transl)]. / Actinomycose pulmonaire greffée sur des lésions tuberculeuses anciennes. A propos d'une observation.

The occurrence of actinomycosis localized in former tuberculosis cavities in a 39-year-old woman is reported. Exeresis of the lesions allowed the discovery of Actinomyces israelii in one of the cavities which no longer communicated with the bronchial tree. In contrast to diffuse actinomycosis found in immuno-depressed patients, this form of the disease was localized in a woman in good health. A decrease of pulmonary defense mechanisms could be involved locally. The infrequency of a tuberculosis-actinomycosis sequence, contrary to aspergilloma, is perhaps due to the anaerobic metabolism of the Actinomyces.

[Primary gastric plasmacytoma with IgG dysgammaglobulinemia and macrocytic anemia (author's transl)]. / Plasmocytome gastrique primitif avec dysglobulinémie à IgG et anémie macrocytaire.

The authors report a case of a woman aged 76 years with a single plasmocytoma of the stomach secreting large quantities of a paraprotein of the IgG type with Kappa light chains. A macrocytic anemia due to deficiency of gastric intrinsic factor was also present. This pathological association is extremely rare. About sixty cases of gastric plasmocytoma are been reported in the published literature and their principal characteristics are briefly outlined.

[Intrapulmonary solitary round opacities. Diagnosis and therapy]. / Les opacités rondes solitaires intra-pulmonaires. Conduite diagnostique et thérapeutique.

The authors envisage the practical problems brought by unexpected intraparenchymal single round images seen on X ray. Thirty to 40% of these opacities proved to be malignant; the proportion rising over 50% in patients more than 50 years old. The great majority of these malignant tumours are primitive and the earlier the exeresis, the better the prognosis. The criteria of benignity (age under 35, presence of calcification amidst the opacity) are not always absolute. For fear of overlooking a malignant tumour, exploring thoracotomy is recommended.

[Multiple angioleiomyomas of both lungs. Probable hamartomatous tumors. Very late death due to respiratory insufficiency]. / Angio-léiomyomes multiples des 2 poumons. Tumeurs hamartomateuses probables. Décès très tardif par insuffisance respiratoire

Case presentation : Multiple tumor nodules in both lungs, identified by biopsy and necropsy, in a 48 year old woman, 8 years after radiological discovery ; death due to respiratory failure after long tolerance ; peculiar angio-leiomyomatous type pulmonary tumors. This purely pulmonary tumor disease is difficult to understand ; the most probable hypothesis envisaged is that of hamartomatosis ; comparison with certain general dysplastic disease is possible, and in particular Bourneville's disease and lymphangio-myomatosis.

[Gram-negative bacterial lung diseases in an internal medicine department]. / Les pneumopathies à bacilles a gram négatif observées dans un service de médecine interne

In a department of internal medicine in Paris, the pneumonopathies with Gram negative "wild" bacilli represent about the third of recognized bacterial pneumonopathies. If started before the results of bacterial tests are known, the treatment should always include a drug active on Gram negative bacteria. Together with KLASTERSKY (1972) we think that synergic associations including the use of Gentamicin are the most effective. Once the responsible germ isolated and its sensitivity to antibiotics determined, therapy can be modified accordingly. With an appropriate antibiotic and by treating an unfavourable background and the initial localization, prognosis is favourable in most cases. This contrasts with the heavy mortality of pulmonary infections with Gram negative bacilli pertaining to hospital.