[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.

[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.

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.

[Management of emphysematous pyelonephritis based on a series of 21 cases]. / La prise en charge des pyélonéphrites emphysémateuses. A propos de 21 cas.

OBJECTIVE: To define the clinical, laboratory and morphological features of emphysematous pyelonephritis, as well as the treatment modalities, with particular emphasis on the need for urgent treatment. MATERIAL AND METHODS: Between 1987 and 2004, 21 patients were treated for emphysematous pyelonephritis. Epidemiological, clinical, laboratory and radiological data, treatments and clinical outcome were retrospectively collected for all patients. RESULTS: This series comprised 15 women and six men with a mean age of 54.6 years. All were diabetic. Upper urinary tract obstruction was demonstrated in 47.6% of cases. The left kidney was affected in 14 patients and the right kidney was affected in six patients. Only one patient had bilateral pyelonephritis. The diagnosis was established by CT in every case. All patients received appropriate intensive care. Treatment was purely medical in one case. Emergency nephrectomy was performed in 12 patients, emergency surgical drainage was performed in three patients, percutaneous drainage was performed in two cases and ureteric catheter drainage was performed in three patients. The mortality rate in this series was 23.8%. CONCLUSION: Emphysematous pyelonephritis is a serious infection. Early diagnosis is essential, particularly in diabetic patients. The positive diagnosis is based on computed tomography and treatment is now increasingly conservative.

False penile fracture: report of 16 cases.

We determined the value of diagnostic and therapeutic approaches of false penile fractures and the outcome of treatment. We retrospectively reviewed 16 cases of presumed penile fracture with a negative surgical exploration. Clinical presentation, technique of treatment and outcome were noted. The mean age was 39 years (17-64). Nine patients were injured during sexual intercourse. All the patients presented with the presumptive diagnosis of penile fracture. False penile fracture was evoked in one patient presenting a new erection. Surgical penile exploration was carried out for all the patients without any radiological explorations. It revealed nonspecific dartos bleeding in 10 cases and avulsed superficial dorsal vein in six cases requiring venous ends ligation. All the patients regained penile appearance and potency. We can hardly distinguish false penile fracture from 'true' penile fracture with certainty either clinically or radiologically, thus, surgical exploration is mostly necessary. The prognosis is excellent.

Cystic nephroma in the adult: pathological aspects and therapeutic implications.

Cystic nephroma is a benign renal neoplasm. Since its initial description, there has been much debate regarding its origin. Preoperative diagnosis of Cystic nephroma is difficult to achieve. The differential diagnoses of Cystic nephroma are recently described mixed epithelial and stromal tumours of the kidney and cystic renal cell carcinoma. The Authors report three cases of Cystic nephroma and illustrate the clinical, radiological and histological features of this renal neoplasm.