[Characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis]. / Caractéristiques de l'atteinte cardiaque au cours de la granulomatose éosinophilique avec polyangéite.

AIM OF THE STUDY: Our study aimed to identify the characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: We conducted a retrospective analytic study including EGPA cases diagnosed between 2000 and 2019 in an internal medicine department. Diagnosis was made according to the 1990 American College of Rheumatology criteria and the 2012 Chapel Hill Concensus. RESULTS: Eleven EGPA cases were included, 64% of patients were female. Median age at diagnosis was 52 years [42-58]. Heart damage revealed EGPA in 55% of cases with a significant predominance of women (p=0.015). The main cardiac manifestations were myocarditis, ischemic cardiomyopathy due to small vessel vasculitis, cardiac tamponade and intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) mainly showed subendocardial hyposignal in early infusion and late enhancement in the same areas, nodular by locations, associated with impaired left ventricle function and micro-infarctions by distal vasculitis. Cardiac damage was associated to ANCA negativity in 83.3% of cases. The median Birmingham Vasculitis Activity Score version3 (BVAS v3) was 16 [10-17]. Under conventional treatment, no relapses had occurred. The median vasculitis damage index (VDI) was 2 [1-2.3] and the mortality rate was zero after a mean follow-up of 43 months. CONCLUSION: Cardiomyopathy is a frequent revealing mode of EGPA. A late onset asthma and hypereosinophilia should guide the diagnosis. As ANCA research often turns out to be negative, histological evidence is recommended in this context. The contribution of cardiac MRI in the diagnosis of EGPA remains to be defined.

[A rare cause of pulmonary arterial hypertension: Thoracic amyloidosis]. / Une cause rare d'hypertension artérielle pulmonaire : l'amylose thoracique.

Isolated thoracic involvement in amyloidosis is a rare and serious condition. Its association with pulmonary arterial hypertension (PAH) usually weakens the prognosis. We report the case of a 40-year-old man with a smoking history, hospitalized for chest pain, abdominal pain and acute respiratory distress. The cardiac ultrasound revealed a circumferential pericardial effusion as well as a pulmonary artery systolic pressure (PAPS) at 80mmHg. Chest imaging (computed tomography scan and magnetic resonance imaging) showed a tissue process developed in the pericardial sheath (60×45mm) sheathing the ascending aorta and infiltrating the trunk of the pulmonary artery and its right branch. Anatomopathological and immunohistochemical study of the process revealed AL amyloidosis. Note that the patient had no signs of extrathoracic amyloidosis. Blood and urine electrophoresis and immunoelectrophoresis as well as bone marrow mylogram and biopsy were normal. The patient was put on oral anticoagulant as he presented with PAH. A therapeutic protocol with thalidomide and dexamethasone has been initiated. The course of the disease was marked by total regression of the clinical signs, a marked decrease in the amyloid process on imaging and a normalization of the PAPS; our follow-up being three years.

Pheochromocytoma presenting as an authentic small vessel vasculitis and complicated with pulmonary embolism: An original presentation.

Diagnosis of pheochromocytoma can be simple when classic manifestations are present. It can also be challenging and complicated in some cases because of its wide array of faces and presentations. We present a case of a 30-year-old female patient who came with acute respiratory distress, chest pain, hemoptysis, asthenia, anorexia, weight loss of 20kg, and paresthesia in her lower limbs. Clinical examination found high blood pressure, accelerated heart and respiratory rates, signs of acute right heart failure with jugular venous distention and ankle edema, reticularis livedo in the four limbs, ulcers in both knees and in the 3rd metacarpo-phalangeal articulations and necrotic lesions in both calcaneal tendons and in the right toes. Further investigations concluded on myocarditis associated with alveolar hemorrhage, pericardic and pleuritic effusions and a segmental pulmonary embolism of the right inferior lobe. Neuro-muscular biopsy was suggestive of myositis. Cutaneous biopsy found nonspecific chronic dermatitis. ANCA antibodies were tested twice and were negative. Cryoglobulinemia was also negative. Thoraco-abdomino-pelvic scan was performed showing a large right adrenal mass suggestive of pheochromocytoma. Diagnosis of right adrenal pheochromocytoma was confirmed by MIBG-I123 hyperfixation findings and urinary normetanephrin levels. The patient was treated surgically. Postoperative outcomes were remarkably favorable with a complete regression of the cutaneous lesions and normalization of the blood pressure. Paresthesia significantly decreased. Control echocardiography at 3 months showed an improved heart function with a persistent apical and septal akinesis.

[Assessment of insomnia among Tunisian patients with lung cancer]. / Évaluation de l'insomnie chez les patients tunisiens atteints de cancers bronchopulmonaires primitifs.

INTRODUCTION: Sleep disorders are relatively common in oncology. However, they have not been well studied and are often treated insufficiently. AIM: To assess the prevalence and severity of insomnia in lung cancer patients and evaluate the relationship between insomnia and certain clinical parameters. METHODS: A cross-sectional study was undertaken of patients in Tunis with primary lung cancer. Socio-demographic and clinical data were obtained from the medical records Patients were then asked to answer questionnaires related to insomnia (ISI), depression-anxiety (HAD) and quality of life (QLQ-C30). RESULTS: Fifty patients with lung cancer were included (46 men, 4 women). The average age was 59±9 years. Insomnia was found in 24 patients (48%) and 60% of patients had depression. HAD was significantly higher in the insomniac patients (21.54±8.96 vs. 9.81±5.28, P<0.0001). Similarly, the QLQ-C30 was significantly lower in these patients (41.24±12.55 vs. 56±16.88, P<0.01). ISI was significantly correlated with HAD and QLQ-C30. CONCLUSION: Insomnia is common in patients with lung cancer. It is responsible for impaired quality of life and psychological distress. Diagnosis and management of insomnia in patients with lung cancer is therefore mandatory.

[Home non-invasive ventilation for chronic obstructive pulmonary disease]. / Ventilation non invasive à domicile au cours de la broncho-pneumopathie chronique obstructive.

INTRODUCTION: The benefits of long-term non-invasive ventilation (NIV) in the management of chronic obstructive pulmonary disease (COPD) patients remain controversial. AIM: To analyze the characteristics of COPD patients under home NIV and to evaluate its impact among this population. METHODS: We carried out a retrospective study between January 2002 and April 2016 of COPD patients under long-term NIV at "la Rabta" and the Military Hospital. RESULTS: There were 27 patients with an average age of 64 and a sex ratio (M/F) of 0.92. Active smoking was reported in 96.3%. A persistent hypercapnia following an acute exacerbation of COPD with failure to wean the NIV was the main indication of long-term NIV. We noted a reduction in hospital admissions in the first year of 60% and in intensive care of 83.3% (P<10-3). There was no non-significant decrease of PaCO2 (4.5mmHg). There was no modification in FEV 1 and in FVC (P>0.05). The survival rate was 96.3% at 1 year, 83.3% at 2 years and a median survival of 24 months. CONCLUSIONS: Our study suggests that home NIV contributes to the stabilization of some COPD patients by reducing the hospitalizations rates for exacerbation. More prospective studies are needed to better assess the impact of NIV on survival and quality of life and to better define the COPD patients who require NIV.

[Tuberculous pseudotumour, a challenging diagnosis]. / La tuberculose pseudotumorale, un diagnostic difficile.

INTRODUCTION: The pseudotumorous form of tuberculosis is a rare entity. Whatever its location, it can simulate neoplasia by its radiological and/or endoscopic appearances. We highlight the diagnostic difficulties associated with this type of presentation. METHODS: We performed a retrospective study of inpatient records from 2003 to 2016 in the pneumology department of La Rabta Hospital to identify cases of thoracic tuberculous pseudo-tumor. RESULTS: Seventeen patients were identified. The median age was 41 years and their symptomatology was dominated by cough and general debility. All had abnormal radiology with 10 cases of suspect lesions. Fibre-optic bronchoscopy revealed endobronchial abnormalities in 11 cases. The median overall diagnostic delay was 97 days. The diagnosis was confirmed bacteriologically in five cases, histologically in 14 cases and based on clinical presumption in one case. The progression was favourable: 13 patients have been declared cured and four patients are still undergoing treatment. CONCLUSION: Making a positive diagnosis of thoracic tuberculous pseudotumour can be difficult, as bacteriological samples are often negative. This can lead to a significant delay in diagnosis and treatment.

RET fusion genes are associated with chronic myelomonocytic leukemia and enhance monocytic differentiation.

Myeloproliferative neoplasms are frequently associated with aberrant constitutive tyrosine kinase (TK) activity resulting from chimaeric fusion genes or point mutations such as BCR-ABL1 or JAK2 V617F. We report here the cloning and functional characterization of two novel fusion genes BCR-RET and FGFR1OP-RET in chronic myelomonocytic leukemia (CMML) cases generated by two balanced translocations t(10;22)(q11;q11) and t(6;10)(q27;q11), respectively. The two RET fusion genes leading to the aberrant activation of RET, are able to transform hematopoietic cells and skew the hematopoietic differentiation program towards the monocytic/macrophage lineage. The RET fusion genes seem to constitutively mimic the same signaling pathway as RAS mutations frequently involved in CMML. One patient was treated with Sorafenib, a specific inhibitor of the RET TK function, and demonstrated cytological and clinical remissions.

[Hydatid cyst of the chest wall mimicking metastatic colon cancer]. / Kyste hydatique de la paroi thoracique mimant une métastase de cancer colique.

Chest wall hydatidosis, rare even in the endemic countries, represents 0.09 to 0.3 % of all cases of thoracic echinococcosis. The authors report the case of a 76 year-old man presenting a chest wall mass 4 years after surgery for colic carcinoma. The mass presented both hypoechoic and hyperechoic structures in the ultrasound chest echography. Therefore, metastastic colon cancer was suspected. The pathological study of the mass revealed hydatid membranes. Thoracic tomodensitometry supported the diagnosis of costovertebral and soft tissue hydatid cysts. The patient underwent the surgical resection of two rib arches, a transverse apophysis and the neighbouring soft tissue associated with pre- and post-surgical albendazole. No clinical manifestations were noted in the follow-up after 1 year.