Bilateral phrenic paralysis in a patient with systemic lupus erythematosus.

Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. They include respiratory muscle abnormalities, which have been implicated in the pathogenesis of the "shrinking lung syndrome" (SLS). We report the case of a patient with this syndrome, in whom diaphragmatic paralysis due to demyelinating phrenic lesions was diagnosed at the same time as SLE. Follow-up studies showed a favorable clinical and diaphragmatic outcome with corticosteroid therapy, but little change in spirometry. It is concluded that severe diaphragm palsy is possibly due to phrenic nerve lesions in SLE, and that the link between diaphragm dysfunction and the SLS is probably not a straightforward one.

[Recurrent bilateral pleurisy in an 80-year-old man]. / Pleurésie bilatérale récidivante chez un homme de 80 ans.

An 80-year-old man was admitted with recurrent asphyxiating pleurisy, first attributed to heart failure. During the recurrent episodes, the patient presented fever, signs of inflammation, no signs of heart failure, and subnormal cardiac function, prompting further investigations which disclosed that the patient was a homozygous carrier of the severe type of periodic disease mutation. The patient's age at symptom onset and the clinical features of this case of periodic disease are exceptional. These points emphasize the usefulness of available genetic tests in difficult diagnostic cases. It also reflects current difficulties in trying to establish correlations between genotype and phenotype in periodic disease.

Cardiac cause of hypoxaemia in a kyphoscoliotic patient.

This report presents the case of a 73 yr-old female in whom kyphoscoliosis, osteoporotic vertebra compression fractures and sternal injury resulted in severe respiratory failure and hypoxaemia. Pulmonary function testing showed moderate restrictive pattern and rare mismatches were found on lung ventilation/perfusion scanning. Transoesophageal echocardiography with contrast studies showed abnormal anatomic mediastinal interactions which led to right-to-left interatrial shunt, through patent foremen ovale. First-intention treatment, because of orthopaedic and respiratory surgical restraints, was to close the shunt using transcatheter devices. Follow-up after 6 months demonstrated that these interauricular umbrella devices corrected arterial hypoxaemia. True right-to-left interatrial shunts can be found in kyphoscoliotic patients, as a result of thoracic deformation, and can be safely treated with percutaneous trancatheter closure.

Acute life-threatening toxocaral tamponade.

An unusual case of life-threatening visceral larva migrans (toxocariasis) is reported herein. The patient was admitted with acute dyspnea and bilateral pleural effusion; rapidly pericardial tamponade developed. Blood and body fluid eosinophilia were elevated. Extensive investigations revealed no malignant process or vasculitis, but Toxocara infection was confirmed by rising specific antibody titers. The high seroprevalence of Toxocara antibodies, particularly in children, suggests that a diagnosis of visceral larva migrans should be considered before a diagnosis of systemic hypereosinophilic syndrome even when clinical presentation is unusual. Prophylaxis against this widespread polymorphic zoonotic infection is desirable in view of the potentially dramatic consequences of infestation.

Tuberculosis generates a microenvironment enhancing the productive infection of local lymphocytes by HIV.

Tuberculosis (TB) contributes to the progression of HIV disease but, so far, the mechanism involved is not clear. Several cytokines accumulating in vivo at the site of mycobacterial infection up-regulate HIV expression in vitro. In this study, we assessed the role of pleural fluids recovered from seronegative patients with TB on HIV replication in acutely infected blast cells. Pleural fluids from subjects with congestive heart failure served as controls. In all cases, TB pleural fluids stimulated HIV replication in vitro. TNF-alpha, IL-6, IFN-gamma, and granulocyte/macrophage (GM)-CSF, as well as very low levels of IL-2, were detected in TB pleural fluids. An anti-IL-2 Ab preincubated with TB pleural fluids exhibited no blocking effect on HIV replication similarly to anti-IFN-gamma and anti-GM-CSF Abs. In contrast, anti-TNF-alpha and anti-IL-6 Abs decreased HIV replication by 60 and 90%, respectively. Recombinant TNF-alpha and IL-6 stimulated HIV replication, while IFN-gamma and GM-CSF had a more ambiguous role. The capacity of pleural fluids to stimulate HIV replication was specific for TB, since the capacity of control fluids was significantly lower. Finally, in contrast to PBL, which require in vitro activation for their productive infection by HIV, unstimulated tuberculous pleural lymphocytes were productively infectable by HIV. Taken together, our data suggest that the microenvironment generated by TB might increase the HIV burden in infected subjects, partly through cytokines other than IL-2, namely TNF-alpha and IL-6.

[Multicentric Castleman's disease with mediastinal involvement in a patient with HIV infection]. / Maladie de Castleman multicentrique à expression médiastinale chez un patient infecté par le VIH.

Castleman's disease is most often seen by a thoracic physician as a mediastinal tumour which is discovered fortuitously and whose surgical excision leads to a cure. We report a case of a patient of 30 who was seropositive for HIV and was suffering from Castleman's disease initially localised to the mediastinum. The disease was associated with a cutaneous and bronchial Kaposi sarcoma. The mediastinal disease, associated with cutaneous and bronchial Kaposi sarcoma, was marked by evolving in a multicentric manner. We review the histological definition and recent data concerning the pathophysiology and the diagnostic and therapeutic management of this disorder and the very varied clinical expression.

Extensive apoptosis of lung T-lymphocytes maintained in vitro.

The phenotypic and functional properties of T cells recovered from the lung indicate that many of these cells have been recently activated. Because such recently activated cells are often more susceptible to death through apoptotic mechanisms, the viability of lung T cells recovered from bronchoalveolar lavage and those isolated from peripheral blood was compared. The progressive loss of viable cells following in vitro culture was considerably greater for lavage T cells than blood T cells, and was observed for cells from both patients with sarcoidosis and control subjects. Following 4 days of culture, 76 +/- 14% of blood cells, but only 31 +/- 13% of lavage cells from sarcoid patients were viable. The evaluation of morphologic features and flow cytometric profiles, as well as the demonstration of typical oligonucleosomal fragmentation of DNA extracted from these cells indicated that lavage T cells were dying by apoptotic mechanisms. CD4+ T cells appeared to be particularly sensitive to apoptosis. Most lavage T cells from controls and sarcoid patients expressed Fas (CD95) antigen. Although some lavage T Cells were sensitive to Fas-induced apoptosis, the viability of lavage T cells was not improved by incubation in the presence of a monoclonal antibody that inhibits Fas-induced apoptosis. Culture in the presence of interleukin 2 did prevent, at least in part, the progressive death of lavage T cells, suggesting that the viability of T cells in the lung may depend on the presence of locally delivered trophic signals. These studies emphasize that T cells on the alveolar surface are in a different state of activation and differentiation compared with that of circulating T cells, and offer a possible explanation for the impaired functional capacities observed for lavage T cells in some in vitro studies.

Cytomegalovirus-induced alveolar hemorrhage in patients with AIDS: a new clinical entity?

We report five cases of alveolar hemorrhage associated with intravascular hemolysis in patients with AIDS. Cytomegalovirus was the only pathogen recovered from the lungs of these patients. There was evidence of multivisceral spread of the virus in all patients, and all had viremia. All had clinical, biological, and pathological features of pulmonary vasculitis, and the conditions of four improved with specific anti-cytomegalovirus therapy.

[Disseminated histoplasmosis in an HIV seropositive patient]. / Histoplasmose disséminée chez un patient séropositif pour le VIH.

The authors report a case of disseminated pulmonary histoplasmosis in an HIV positive patient who had travelled in countries where the disease was endemic. The principle clinical characteristics of the disease are reviewed including the radiology and the methods of treatment particularly in the forms occurring in this context. Finally the authors underline the difficulty in the differential diagnosis with tuberculosis.

[Primary pulmonary hypertension in a HIV positive patient]. / Hypertension artérielle pulmonaire d'allure primitive chez un patient séropositif pour le VIH.

Apparently primary pulmonary hypertension is rarely described in patients infected with the human immunodeficiency virus (HIV). We observed a case in a HIV seropositive patient with no known risk factor for pulmonary vascular disease. The characteristics of this new association are described.