Fibrose(s) rétropéritonéale(s) : stratégie diagnostique,pathologies associées et suivi à long terme d’une cohorte française

Purpose

Retroperitoneal fibrosis (RPF) is a rare disease with an expanding etiologic spectrum. We aimed to analyze non-invasive diagnosis strategy, associated disorders, monitoring, treatment and prognosis.

Methods

Retrospective cohort study in a single tertiary center.

Results

Eighteen RPF cases (11 males) followed between 1996 and 2009 were reviewed. Blood CRP level was high in all cases before treatment. CT scan, associated or not with MRI or 18-FDG PET-scan, confirmed the diagnosis in 15 patients. Histological analysis of a surgical biopsy specimen was performed in only three cases. Ten patients suffered retroperitoneal fibrosis secondary to systemic vasculitis (granulomatosis with polyangeitis, n = 1, Takayasu aortitis, n = 2), systemic fibrosis with Riedel thyroiditis (n = 1) and atheromatous periaortitis (n = 6). Fifteen patients were treated with corticosteroids with a mean treatment duration of 60 months (12–228). Dependency to corticosteroids was recorded in ten patients. Patients with fibrosis related to vasculitis were younger, had a higher CRP level, more frequent corticosteroid dependency and a higher relapse rate. Relapses were successfully treated with steroids. Immunosuppressive treatment was only prescribed in the setting of systemic vasculitis. No patient died, after a 6 ± 2 years follow-up. Late relapses could occur, sometimes years after steroid therapy cessation.

Conclusion

In our study, RPF occurred as a secondary disorder in 60% of the cases. Disease extension, relapse rate and treatment response varied according to the underlying cause of RPF, pleading for an extensive and systematic initial assessment. Since no death or end-stage renal insufficiency was observed, RPF might be considered as a steroid-sensitive and benign disorder.     

Maladie de Takayasu et fibrose rétropéritonéale : une nouvelle observation

The authors report a 38-year-old young Moroccan woman with retroperitoneal fibrosis (RPF) associated with Takayasu's disease. The RPF was diagnosed in the presence of an acute renal failure requiring placement of double J catheters while an abdominal CT scan was suggestive of RPF. The diagnosis of Takayasu's disease was suspected three years later when the patient presented with right upper extremity dysesthesia. Aortic angiography showed evidence of typical inflammatory arteritis involvement with bilateral regular and concentric stenosis of axillary arteries and a left, smoothly narrowed primitive carotid artery, and left renal artery partial occlusion. The patient was treated with prednisone (0.5 mg/kg per day) with a marked improvement. The association between RPF and Takayasu's disease is very rare, and only five cases have been reported in the literature. These two diseases share similarities in some of the etiologic factors and anatomic localizations.     

Admissions en réanimation des femmes en cours de grossesse ou en post-partum : circonstances et pronostic. Une série rétrospective de 96 cas

Purpose

Very few studies had been published about admission of obstetric patients in French intensive care units (ICU).

Patients and methods

Files of women who had been admitted during pregnancy or the postpartum period to ICU of an academic hospital between January 1st 1997 and 31st December 2006 were analyzed. Diagnosis at admission, severity, main treatments and outcome were studied during two successive periods of 5 years.

Results

There were 96 admissions (0.95% of all admissions to ICU). They included 34 complications due to arterial hypertension (35%), among them 10 cases of pre-eclampsia, eight of eclampsia, seven of Hemolysis-Liver Enzymes-Low Platelet (HELLP) syndrome and two of retroplacental hematoma ; 26 postpartum haemorrhages (27%); and 36 miscellaneous diagnosis including two sepsis, four acute pulmonary oedema, four cardiomyopathy, three pulmonary embolism, and three acute liver steatosis. Mean Simplified Acute Physiologic Score (SAPS II) was 18.5 ± 11.2 and mean Sequential Organ Failure Assessment (SOFA) 2.1 ± 2.3. The main treatments were mechanical ventilation (50% of all cases) and transfusion (32%). The average ICU stay was 5.7 ± 5.4 days. Two maternal deaths were observed (aortic dissection and peripartum cardiomyopathy). The number of patients admitted for postpartum haemorrhage increased from five to 21 over the two successive periods of the study.

Conclusion

The number of women admitted to ICU during pregnancy or the postpartum period is low. Admissions for hemorrhage have increased. Regular monitoring of maternal morbidity and mortality gives relevant clues for assessing the quality of obstetrical care.     

Apport de l’IRM cardiaque dans l’évaluation initiale et le suivi des myocardites mimant un syndrome coronaire aigu : à propos d’une série de 43 patients

Aims

The diagnosis of acute myocarditis is complex, especially when the clinical presentation mimics an acute coronary syndrome. This condition may promote the progression to dilated cardiomyopathy and the occurrence of severe arrhythmias. A reassessment integrating a cardiac MRI at three months after the acute episode could help identify patients with a poor prognosis.

Patients and results

This prospective series of 43 consecutive patients hospitalised for acute myocarditis included 36 men and seven women, with a mean age of 32 years, with no indication of heart failure. All patients presented elevated levels of troponin I. Echocardiography showed moderate left ventricular dysfunction in six cases and segmental wall motion abnormalities in 22 cases. After gadolinium injection, a subepicardial late enhancement was observed in 39 cases. Three months after the acute episode, all patients were asymptomatic. The echocardiography and laboratory tests were normal. In 23 cases, the MRI showed persistence of the late enhancement without segmental wall motion abnormality. After a mean follow-up of three years, one patient was lost to follow-up and only one suffered a heart failure revealing a dilated cardiomyopathy complicated by ventricular arrhythmias.

Conclusion

On admission, the subepicardial localisation of late enhancement in the cardiac MRI is reliable criteria for the diagnosis of acute myocarditis, enabling to rule out an acute coronary syndrome. During follow-up, the persistence of late enhancement has no impact on prognosis. In this series, after a mean follow-up of three years, it was not associated with clinical or paraclinical abnormalities, except in one patient.     

Résultats à court et long terme du remplacement valvulaire mitral par prothèse mécanique à bille et à ailettes (à propos d’une série de 236 patients consécutifs avec un suivi moyen de 11 ans)

Objectives

To study the early and late results of mitral valve replacement (MVR) by Starr-Edwards caged-ball and bileaflet mechanical prosthesis.

Material and methods

We retrospectively analyzed 236 MVR performed in 236 patients: 127 by Starr-Edwards prosthesis (group 1) and 109 by bileaflet prosthesis (group 2).

Results

During the early period (30 days), the mortality rate was higher in group 1 (6.3 % vs 1.8 %; p = 0.0001), while hemorrhagic, thromboembolic and infectious complications were comparable in the two groups. In the late period (> 30 days) and with an average follow-up of 11.5 ± 5.7 years, mortality was higher in group 1 (9.4 % vs 4.6 %; p < 0.0001). The same was true for thromboembolic complications (20.8 % vs 6.4 %; p < 0.0001), hemorrhagic complications (13.4 % vs 7.3 %; p = 0.02), infectious complications (3.1 % vs 0.9 %; p = 0.02) and cardiac complications that were not due to the prosthesis (32.3 % vs 14.7 %; p = 0.02). The hemodynamic profile of the bileaflet prostheses was better than that of the Starr-Edwards prostheses (average functional prosthetic surface area was 2.37 ± 0.44 cm² and average pressure gradient was 5.6 ± 1.1 mmHg vs 2.04 ± 0.52 cm² and 7.6 ± 4.9 mmHg).

Conclusion

Our work confirms the superiority of bileaflet mechanical prostheses, with rates of early and late mortality, thromboembolic and hemorrhagic complications lower than those of the Starr-Edwards prostheses in more than 11 years of follow-up. However, one should not forget that the prevention of infective endocarditis, good observance of oral anticoagulant treatment and early surgery before left ventricular dysfunction occurs remain the best guarantee a good result of the MVR.     

Prévalence et valeur diagnostique des anticorps antinucléaires de spécificité antigénique indéterminée : étude rétrospective à propos d’une série de 90 patients

Purpose

The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique.

Patients and methods

Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis.

Results

Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n = 6), scleroderma (n = 5), Sjögren's syndrome (n = 3), SLE (n = 4), rheumatoid arthritis (n = 6) and antiphospholipid syndrome (n = 4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation.

Conclusion

Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as “gold standard” test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.     

Infarctus du myocarde chez le sujet fumeur de moins de 50 ans traité par angioplastie coronaire. Évolution hospitalière et sur le long terme d’une série consécutive de 93 patients

Aims of the study

The study evaluated in-hospital and long-term outcome of patients less than 50 years old with myocardial infarction within 12 hours after symptom onset treated by coronary angioplasty.

Patients and method

This is a retrospective study with survival analysis by Kaplan-Meier method in patients included from December 2003 to February 2008.

Results

We included 93 patients aged 42,8 ± 5,2 years old with smoking estimated at 27,7 ± 12,7 pack-years. Thirty-one patients (33,3%) were dyslipidemic and 36 patients had family history of coronary artery disease. Thirty patients (32,3%) had an anterior myocardial infarction and four patients (4.4%) had Killip greater than 2. Coronary angioplasty was performed within 4.5 ± 3.0 hours after symptom onset with TIMI 3 final flow in the culprit vessel in 96.8%. One patient died from cardiogenic shock. With a follow-up of 85 patients during 20.0 ± 15.6 months, the survival without death was 98.2% and survival without major cardiac complication was 87.9% at 24 months. Seventy-two patients (85.7%) were taking a betablocker, 81 patients (96.4%) aspirin, 75 patients (89.3%) a statin and 64 patients (76.2%) an angiotensin-converting inhibitor. Only 50 patients (58.8%) were nonsmokers.

Conclusion

Thus, young smokers with acute MI treated by coronary angioplasty have a good prognosis during in-hospital stay and long-term outcome. Secondary medical treatment prevention is well followed but there is a low rate of smoking cessation.     

Influence de l'hypertension artérielle sur le profil clinique et le pronostic des patients hospitalisés pour COVID-19 dans la ville de Bukavu,en République Démocratique du Congo : étude de cohorte prospective

ProblemThere are no studies on the association between high blood pressure and COVID-19 in South Kivu.Objectiveto determine the influence of arterial hypertension on the clinical characteristics and prognosis of COVID-19 patients hospitalized in the city of Bukavu.MethodologyBetween June 2020 and June 2022, an open cohort of hypertensive and non-hypertensive COVID-19 patients admitted to two clinics in the city of Bukavu was formed. The primary endpoint was the occurrence of death. Thus, a prospective modeling of mortality by the Kaplan-Meier estimator was carried out.ResultsAmong the 178 admissions for COVID-19, 68 (38.2%) patients were hypertensive. Compared to non-hypertensives, hypertensive patients were significantly older [61.0 (56.0–71.0) vs. 48.0 (32.7–64.0); p < 0,0001). During the observation period of 1059 patient-days, the incidence of death (3.2/100 patient-days) was non-significantly higher in hypertensive patients (4.3/100 patient-days) (p = 0.06). On the other hand, the independent predictors of death were Sepsis [adjusted HR = 3.7 (1.5–8.7)], CRP > 100 mg/L [adjusted HR = 3.0 (1.2–7, 0)] and SaO₂ < 90 % [adjusted HR = 3.9 (1.3–11.8)].ConclusionThis study shows that hypertension was very common in patients admitted for COVID-19 in the city of Bukavu but did not influence the vital prognosis of the latter, thus confirming the finds of most authors who have addressed the question.