Pulmonary hypertension on systemic sclerosis-lupus erythematosus overlap syndrome

PurposeThere 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.MethodsSixteen 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 30 mmHg. 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)ResultsSixteen 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 52 mmHg, extreme (32–80 mmHg). 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.ConclusionAccording 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.     

Hypertension artérielle du sujet noir âgé de 50 ans et plus a Lomé : aspects épidémiologiques et évaluation du risque cardiovasculaire (Étude prospective et longitudinale de 1485 patients)

Introduction

High blood pressure is public health problems whose assumption of responsibility especially remains difficult at the old subject. Generally it is associated with the other cardiovascular risk factors. We assigned for objectives to determine the prevalence of High blood pressure in the old subject in specialized milieu and to evaluate the cardiovascular risk among these patients.

Methodology

It is about a longitudinal exploratory study undertaken on 1485 hypertensive of 50 years old and more, selected among 1999 patients received in three health professional trainings of the commune of Lomé, of June 1, 2004 to June 30, 2007. Information had been collected using a card of investigation. Classifications of high blood pressure were those of the JVCVII and the European Society of Cardiology. The data analysis had been made by computer tools.

Results

The prevalence high blood pressure was of 74.29%. We had noted a female prevalence (63.8%) with a sex ratio of 0,57 and one middle age of 62.08 ± 9.3 years. Dyspnea (45.9%), chest pains (16.2%) and palpitations (13.2%) were the principal found symptoms. The various listed risk factors were: the dyslipidemy (58.1%), obesity (36.12%), alcoholism (16.7%) and the diabetes (10.6%). The complications were of order cardiac (87.81%), eyepieces (79.8%), renal (19.86%), neurological (4.92%) and arterial (0.99%). The cardiovascular risk was very high at 58.05% of the patients. The rate of lethality was of 1.9%.

Conclusion

High blood pressure is the cardiovascular risk factor most frequent in our country as from 50 years. Its assumption of responsibility passes by information, the education of the population and requires the mobilization of all the social components.     

Beneficial effect of sildenafil following surgery for mitral stenosis complicated by pre-capillary pulmonary hypertension: report of two cases

Pulmonary hypertension is a serious disorder, difficult to treat especially in the severe forms. The treatment consists mainly of calcium channel blockers, anti-coagulation, intravenous epoprostenol, inhaled nitric oxide and recent agents as bosentan and sildenafil. Sildenafil, a phosphodiesterase 5 specific inhibitor, has been largely evaluated in primary pulmonary hypertension, and in some cases of secondary pulmonary hypertension including parenchymal and thromboembolic diseases; it has not yet been evaluated in severe pulmonary hypertension with elevated pre-capillary resistance in operated mitral stenosis. We report the cases of two patients operated from mitral valve replacement for severe mitral stenosis with elevated pre-capillary resistance, where oral sildenafil, introduced empirically immediately after the surgical procedure at the dose of 50 mg/d, permitted a significant decrease in pulmonary pressures and resistances, allowing a rapid withdrawal of nitric oxide and reducing therefore hospitalization time in the intensive care unit. We think that this simple treatment, with or without association to nitric oxide, should be generalized to persistent pulmonary hypertension following cardiac surgery.