Case Report: Case report: An unusual presentation of granulomatosis with polyangiitis.

Aim: We are reporting a case of an unusual presentation of granulomatosis with polyangiitis (GPA) with liver involvement. Case presentation: A 45-year-old male patient presented with erythematous plaques on the face and bilateral nasal obstruction. On physical examination, the patient had a ring-shaped squamous plaque on the face. The laboratory findings revealed an accelerated erythrocyte sedimentation rate at 100 mm/h, an elevated C-reactive protein at 66 mg/L, hyper gamma globulinemia 16 g/L and an elevated alkaline phosphatase (twice the upper normal limit). The craniofacial and thoracoabdominal computed tomography (CT) -scans showed ethmoid and maxillary sinusitis, low facial bone density, multiple mediastinal and hilar lymphadenopathy, diffuse small pulmonary nodules, and hepatomegaly. A cutaneous lesion biopsy, the nasal mucosa, and the liver showed a chronic inflammatory granulomatosis process with necrosis. Serum anti-neutrophil cytoplasmic antibody (ANCA) against PR3 was positive. The clinical, biological, radiological, and histological findings substantiated the diagnosis of GPA. The patient received systemic steroids combined with cyclophosphamide pulses on days 1, 14 and 28 and then he was lost to follow-up. Two-years later, he presented with a cardiac failure and skin ulcer in the right lower limb. A nasal endoscopic exam showed nasal septum cartilage perforation with resorption of the middle and inferior nasal concha. Two weeks later, he developed a diffuse alveolar hemorrhage and was therefore transferred to the intensive care unit but died of respiratory failure 3 days later. Conclusion: Clinicians should be aware of GPA atypical clinical manifestations.

Developing learning objectives in postgraduate medical education for family medicine (Tunisia).

Introduction-Aim: The third cycle of medical studies (TCMS) lasts 3 years for the specialty of family medicine (FM) in Tunisia. The members of the FM committee of the Faculty of Medicine of Monastir (FMM) aimed to detail the learning objectives (LO) of residents in FM. METHOD: We used the Delphi method in 2 rounds including a group of experts called FM Learning Objectives Writing Group (FMLOWG) at the FMM. The FMLOWG included 74 university hospital physicians and FM internship supervisors. These members actively participated in the 10 meetings held during the month of March 2022. Three points were discussed: the identification of LOs; the development of training titles and the proposal of the teaching methods to be adopted. The writing was subdivided into 5 domains of LO: transversal, public health, typical population and by system. RESULTS: We identified 1359 LOs for FM residency, for which 552 were LOs per system (40.5%). The learning included 618 training session titles. Residents will have an academic training day every 3 weeks during 9 months for each TCMS year. CONCLUSION: A detailed, MF-specific consensus has been developed by majority of medical specialties. It will be a learning base for learners, a reference for supervisors and TCMS teachers.

Case Report: Recurrent retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient.

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the revealing manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

[Corticosurrenaloma: an exceptional cause of primary hyperaldosteronism]. / Le corticosurrénalome: une cause exceptionnelle d'hyperaldostéronisme primaire.

Corticosurrenaloma is a rare malignant tumor of the adrenal gland that often secretes corticosteroids, sex steroids and precursors. Aldosterone-producing corticosurrenaloma is very rare, accounting for 1 case/10million inhabitants. We report the case of a 38-year old man presenting with severe arterial hypertension associated with deep hypokalaemia (2.2 mmol/L). Exploration showed primary hyperaldosteronism (aldosterone = 2645 pmol/l, aldosterone/renin ratio = 327 pmol/MUI), with hypersecretion of glucocorticoids. Abdominopelvic CT scan revealed left poorly differentiated and heterogeneous adrenal mass measuring 9cm, infiltrating into the surrounding adipose tissue and the diaphragm, extending into the left renal vein, with regional adenopathy and hepatic nodule measuring 4cm. The patient underwent radical nephrectomy followed by right hepatectomy two months after resulting in remission. A year after the patient developed lung metastases. This study highlights that corticosurrenaloma should be suspected in patients with primary aldosteronism despite its rarity.

[Late-onset pituitary stalk interruption syndrome (PSIS)]. / Syndrome d'interruption de la tige pituitaire à révélation tardive.

Pituitary stalk interruption syndrome is a fairly common cause of the deficiency of Growth Hormone and hypopituitarism often revealed in the neonatal period and childhood. This observation illustrates the peculiarities of a late clinical onset of this syndrome. We report a case of a 17-year-old patient hospitalized for primary amenorrhoea and impuberism. She had no history of neonatal incident. Clinical examination revealed severe growth retardation Hypophysiogramme showed complete hypopituitarism without diabetes insipidus. Magnetic resonance imaging revealed pituitary stalk interruption and an ectopic posterior pituitary gland. Kidney malformation was objectified, which is in favour of a congenital malformative origin of this syndrome. Hormone replacement was administered to this patient. This late-onset form emphasizes the need for early diagnosis of impuberism and/or stunting, revealing a potentially very serious pathology.

Obstructive sleep apnea presenting as pseudopheochromocytoma.

A 52-year-old female with a history of poorly controlled resistant hypertension was admitted to our hospital with severe hypertension. She had a history of fatigue and intermittent episodes of palpitations. Laboratory evaluation was significant for elevated 24-h urinary catecholamine levels (3,5 times the upper normal levels). This case was presenting with a clinical and biochemical picture indistinguishable from that of pheochromocytoma. However, neither computed tomography nor meta-iodo-benzyl-guanidine scintigraphy detected any catecholamine-producing tumor in or outside the adrenal glands. Our patient was screened with full polysomnography because of heavy snoring, daytime somnolence and obesity. It revealed severe obstructive sleep apnea syndrome. After three months of continuous positive airway pressure therapy, the patient experienced resolution of his presenting symptoms, improved blood pressure control and normalization of his urinary catecholamine levels. This case highlights sleep disordered breathing as a potentially reversible cause of pseudo-pheochromocytoma.

An acute adrenal insufficiency revealing pituitary metastases of lung cancer in an elderly patient.

Metastases of solid tumors to the pituitary gland are often asymptomatic or appereas as with diabetes insipid us. Pituitary metastases more commonly affect the posterior lobe and the infundibulum than the anterior lobe. The presentation with an acute adrenal insufficiency is a rare event. A 69-year-old men presented with vomiting, low blood pressure and hypoglycemia. Hormonal exploration confirmed a hypopituitarism. Appropriate therapy was initiated urgently. The hypothalamic-pituitary MRI showed a pituitary hypertrophy, a nodular thickening of the pituitary stalk. The chest X Rays revealed pulmonary opacity. Computed tomography scan of the chest showed a multiples tumors with mediastinal lymphadenopathy. Bronchoscopy and biopsy demonstrated a pulmonary adenocarcinoma. Hence we concluded to a lung cancer with multiple pituitary and adrenal gland metastases. This case emphasizes the need for an etiological investigation of acute adrenal insufficiency after treatment of acute phase.