[A perimyocarditis revealing eosinophilic granulomatosis with polyangiitis]. / Une périmyocardite révélatrice d'une granulomatose éosinophilique avec polyangéite : une nouvelle observation.

Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by necrotizing arteritis, eosinophilic infiltration and extravascular granuloma; that may involve several organs. Cardiac involvement is the first cause of death in this vasculitis. These include myocarditis, pericarditis, coronary heart disease, dysrhythmias, and rarely valvular involvement. We report the observation of CSS revealed by acute perimyocarditis.

Pulmonary hypertension on systemic sclerosis-lupus erythematosus overlap syndrome.

PURPOSE: There are authentic observations of combination of systemic lupus erythematosus (SLE) with systemic sclerosis (SS) and with polymyositis defined as overlap syndromes. The prevalence of pulmonary hypertension is unknown in SS-SLE overlap syndrome because of its rarity. The aim of our study was to precise clinical, paraclinical and evolutive features of pulmonary hypertension in patients with systemic sclerosis-systemic lupus erythematosus (SS-SLE) overlap syndrome. METHODS: Sixteen cases of SS-SLE overlap syndrome were retrospectively studied in a period of 16 years (2000-2015). SS-SLE overlap syndrome was diagnosed in the presence of at least 4 criteria of the American College of Rheumatology (ACR) for the diagnosis of SLE and a major criterion or 2 minor criteria of ACR of SS classification. Pulmonary arterial pressure (PAP) was estimated with doppler echocardiography. Pulmonary hypertension (PAH) was defined by a PAP superior than 30mmHg. We distributed groups according to the existence (Group 1) or not (Group 2) of a PAH. Epidemiological, clinical and evolutive features were compared between the two groups with bilateral fisher test (P significant if inferior at 0.05) RESULTS: Sixteen cases of female patients with SS-SLE overlap syndrome with a middle-age of 39 years, extreme (29-58 years) were studied. PAH complicated the evolution of SS-SLE overlap syndrome in six cases with a middle-age of 41 years. Ten patients of the group 2 had an average age of 40 years. The average age of the beginning of the disease was 28 years in the group 1 and 31 years in the group 2. SS preceded SLE in 6 among 16 cases (Group 1: 2/6, Group 2: 4/10). SS was revealed most frequently by Raynauw's Syndrome in both groups (Group 1: 4/6, Group 2: 7/10). Cutaneous and articular involvements were the most frequent observed manifestations of SLE (Group 1: 5/6, Group 2: 6/10). In the group 1, the PAH was discovered approximatively11 years after the beginning of the SS-SLE overlap syndrome. The average PAP was 52mmHg, extreme (32-80mmHg). A right cardiac insufficiency complicated the evolution of the PAH in 3 cases. The PAH was primitive in 3 cases. There was no significant difference concerning the SS-SLE overlap syndrome onset disease symptoms, the frequency of lung involvement and esophageal, neurological, articular and trophic manifestations. PAH was not associated with lupic proliferative renal disease, neither with cutaneous proximal sclerosis nor with anti-Scl70 positivity. Patients were treated with vasodilator treatment in eleven cases: prostacyclin derivates in five cases and endothelin receptor antagonist in six cases. Two patients received corticosteroids and boli of cyclophosphamide for renal involvement and neurologic involvement in each case. Stabilization of PAP was observed in these two cases. Mean follow-up disease was 67 months, extreme (4-124 months) Cutaneous sclerosis evolution was not significantly different between both groups. Global cardiac insufficiency secondary to PAH caused death in one case. CONCLUSION: According to the results of our study, SS-SLE overlap syndrome complicated with PAH seems to be associated more frequently with limited and distal cutaneous manifestations. Patients that have developed lupus nephropathy and/or had positive anti-Scl70 seem to be protected from appearance of PAH during the SS-SLE overlap syndrome.

[Cerebral amyloid angiopathy revealed or hypertension-related cerebral small vessel diseases: A clinical challenge]. / Angiopathie amyloïde cérébrale ou atteinte des microvaisseaux cérébraux en rapport avec l'HTA : un challenge clinique.

Cerebral amyloid angiopathy (CAA) is a entity characterized by degenerative Amyloïd deposits in the walls of the meningeal and cortical vessels. It is considered as the second cause of primitives cerebral hemorrhage in elderly. The differential diagnosis between AAC and hypertension-related cerebral small vessel diseases is difficult and represent a true challenge for the clinician. We report two cases of cerebral small vessel diseases revealed by malignant hypertension.

[Epidemiological, clinical and evolutionary peculiarities of interstitial lung disease in systemic sclerosis]. / Particularités épidémiologiques, cliniques et évolutives des pneumopathies interstitielles au cours de la sclérodermie systémique.

Pulmonary involvement during systemic sclerosis (SS) is dominated by interstitial lung disease and arterial pulmonary hypertension. It is about a retrospective study analyzing 65 cases of SS over a period of 13 years. We compared cases with and without interstitial lung disease. The diagnosis of SS was retained according to American College of Rheumatology (ACR)/EULAR 2013 criteria. The diagnosis of interstitial lung disease was retained in TDM and EFR. Pulmonary hypertension is defined by a pulmonary arterial pression higher than 25 mmHg. The mean delay of diagnosis of interstitial lung disease and the diagnosis was of 48 months (extremes 0-78 months). The comparison between both groups according to average age of the patients, prevalence of pulmonary hypertension, frequency of Raynaud phenomenon and trophic disorders did not find any significant difference. Lung involvement was associated with an esophageal involvement in 71% of the cases (P=0.059). Antibodies anti-Scl 70 were noted more frequently in patient's with interstitial lung disease (79% of the cases, P=0.001). Patients were treated with colchicine and vitamin E. A corticotherapy had been indicated at a single patient. The evolution of SS was marked by the stabilisation of the restrictive syndrome in 71.8% of the cases and a worsening in 25% of the cases. Early and appropriate diagnosis of SS and screening of lung involvement are essential for a early care.

[Incidence of venous thromboembolism in men admitted to a pneumology unit for acute respiratory disease]. / Incidence de la maladie thromboembolique veineuse chez des hommes admis dans un service de pneumologie pour affection respiratoire aiguë

BACKGROUND: Venous thromboembolism (VTE) is a major source of morbidity and mortality all over the world. It is one of the complications arising in a hospital environment. The main aim of this study is to estimate the incidence of VTE in respiratory inpatients. METHOD: The authors carried out a prospective study on acute respiratory disease inpatients. A medical analytical index card was used. An echo-venous Doppler of lower limbs was practiced on D1 and D10 of hospitalization. The Wells scored was used to estimate the clinical probability of the occurrence of VTE. RESULTS: Seven of 100 patients studied developed VTE (95% CI : 2-12%), four of which presented a pulmonary embolism. The patients with VTE are 60-years-old±11.67. The incidence of VTE includes 20% (95% CI : 12.2-27.8%) of the patients hospitalized for bronchial cancer, 7.14% (95% CI : 2.1-12.18) of the patients presenting pulmonary tuberculosis and 4.54% (95% CI : 0.46-8.62%) of the patients hospitalized for exacerbation of chronic obstructive pulmonary disease (COPD). The duration of hospitalization was prolonged for the treatment of VTE (21±4.41 days for these patients compared with 12±5 days in the absence of VTE (P<0.001)). The analysis of the various risk factors shows that a Performance Status>2 (P=0.005) and lung cancer (P=0.028) are the factors most incriminated in the occurrence of VTE. It is associated with a mortality of 2%. CONCLUSION: VTE is a reality which is necessary to prevent in respiratory inpatients in situations at risk, especially in patients with lung cancer and with a PS>2.