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Purpose: Portal cavernoma follows a chronic occlusion of the portal vein. The long-term consequences of portal cavernoma are not well known. The objective of this study was to report the aetiology of the portal cavernoma and its natural course after excluding liver diseases causes., Methodology: A single centre retrospective study based on the data collected from the radiology department of the Clermont-Ferrand hospital was conducted from 2000 to 2011. All the patients for whom an imagery found a portal cavernoma have been looked for excluding the patients having a liver disease whatever the aetiology and the Budd-Chiari syndrome., Results: Thirty-two cases (18 women and 14 men) were selected. The mean age at diagnosis was 54.2 years and the mean follow-up period was 5.4 years. The discovery of a portal cavernoma was incidental for 8 cases. An aetiology was found for 24 cases: it was an haematological aetiology in 15 cases (10 myeloproliferative syndromes, 2 antiphospholid syndromes, 1 thalassemia major, 1 hyperhomocysteinemia, 1 prothrombin gene mutation), a general aetiology in 2 cases (1 coeliac disease, 1 pancreatic neoplasia), and a local inflammation in 7 cases. A dysmorphic aspect of the liver was noticed on medical imaging for 11 out of the 32 cases. A liver biopsy was performed in 4 patients and was normal for all of them. Sixteen patients developed oesophageal varices, 4 patients developed ascites, 3 developed asymptomatic biliary compression by the portal cavernoma, and the patient who had been followed for the longest time (15 years) developed an encephalopathy., Conclusion: In addition to its underlying etiology, the prognosis of portal is mainly related to the occurrence of oesophageal varices that may develop during the follow-up of the patients., (Copyright © 2015 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Mesenteric panniculitis is a nonspecific inflammatory process affecting the fatty tissue at the root of the mesentery. This term is also used to describe the clinical and imaging findings in this disorder. Mesenteric panniculitis can be a misleading term: it is commonly misused to design an increased density of the mesentery without prejudice regarding the etiology. Pain is the main clinical symptom. Half of the patients are asymptomatic. There is a palpable mass in half of cases. Laboratory tests sometimes reveal an acute phase reaction of varying intensity. Mesenteric panniculitis is suspected when CT scan shows increased density of the mesenteric fat. Nevertheless, only histological examination could establish the diagnosis. Histologic examination may reveal various stages: lipodystrophy (the first stage when fat necrosis is predominant), mesenteric panniculitis (a majority of infiltrating lymphocytes), sclerosing mesenteritis (the end stage when fibrosis is predominant). Histopathologic differential diagnoses are lymphomas, lipomas, liposarcomas that can mimic mesenteric panniculitis on CT scan. Mesenteric panniculitis is associated with various diseases, especially with intra-abdominal inflammatory process. It also can be idiopathic. Rare complications can occur with vascular or digestive tract compressions. Empirical treatment is only useful in symptomatic patients. Colchicine, corticosteroids or immunosuppressive agents can be used. The only interest of surgery is the histological confirmation of the diagnosis. A better understanding of the pathophysiology of the immunoregulatory functions of adipose tissue will improve mesenteric panniculitis management., (Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

IgG4 related disease (IgG4 RD) was first reported as autoimmune pancreatitis then it was established as a systemic disorder characterised by high blood level of IgG4 and fibrosis with rich plasmocytes IgG4+ in almost all organs. IgG4 RD is very sensitive to corticosteroid therapy. IgG4 RD has a high prevalence in eastern countries. Numerous articles on this topic are published and new diagnostic criteria are regularly established. The autoimmune or allergic mechanism of IgG4 RD is still a matter of debate. Interestingly, IgG4 subclass of antibody has anti-inflammatory features. IgG4 RD is not yet very well characterised in western countries. Whether IgG4 is involved in IgG4 RD, pathophysiology is to be defined. IgG4 RD spontaneously regresses in some cases so indications of treatment are not already well clear., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Gastrointestinal manifestations of Henoch-Schönlein purpura (HSP) in adults may be severe. Data about treatment are controversial and the outcome is seldom described., Methods: Twenty-three patients with gastrointestinal manifestations of HSP (ACR criteria) were retrospectively studied. We proposed to use clinical and radiological (CT scan) severity scores to assess the usefulness and the efficacy of corticosteroid therapy., Results: Age at onset ranged from 16 to 80 years (median 39). Gastrointestinal manifestations included abdominal pain (96%), vomiting (52%), gastrointestinal bleeding (39%), diarrhoea (13%) and intestinal obstruction (9%). Scores of disease clinical severity were calculated in 21 patients. Fourteen, three and four had severe, intermediate and mild disease, respectively. Ten patients in the group with severe clinical involvement underwent CT scan that showed severe radiological involvement (parietal thickening of several bowel loops of the same segment or several segments with ileal involvement). Eleven patients out of 14 with clinically severe disease were given corticosteroids. Two patients out of three with intermediate and three patients out of four with mild scores also received corticosteroids. The others received supportive care. In-patients with severe clinical scores, gastrointestinal symptoms improved within 2 days when they were given corticosteroids and within 12.3 days without corticosteroids (p<0.0002). No side effect was observed with steroid therapy., Conclusion: These results suggest that corticosteroids may reduce abdominal symptoms of HSP in adults with clinically severe disease. They are safe when CT scan is performed.

Isolated pulmonary involvement in Goodpasture's syndrome is exceptionally described. We report a 36-year-old woman with pulmonary haemorrhage and review 28 additional cases of the literature. In fact, these patients had often mild urine abnormalities and constant glomerular lesions. Antiglomerular basement membrane antibodies testing should be systematically ordered in patients presenting with alveolar haemorrhage. Goodpasture's syndrome without renal abnormality could be an early stage of the disease with a better prognosis.

Introduction: If abnormal thyroid function indices have been reported in patients with nephrotic syndrome, hypothyroidism is exceptional., Exegesis: We report three adult patients (1, 2, 3) with hypothyroidism associated with nephrotic syndrome (minimal change glomerulonephritis [1], idiopathic membranous nephropathy stage I [2], stage II [3]). Glomerulopathy treatment and thyroid hormone replacement therapy were both initiated. Low replacement (1, 2) was sufficient when proteinuria decreased. It was higher when nephrotic syndrome was uncontrolled (3)., Conclusion: Excessive thyroxine-binding protein and thyroxine urinary loss generate low rate of free thyroxine and elevated TSH. Systematic thyroid hormonal test is necessary if nephrotic syndrome is severe and prolonged.

Introduction: Nitrofurantoin is commonly used in the treatment of urinary tract infection and may cause a potential severe complication: interstitial lung diseases., Case Report: A 78-year-old and an 87-year-old woman treated with nitrofurantoin since respectively 10 months and 6 years developed cough and dyspnea. Antibiotics were ineffective and interstitial lung disease was found. Nitrofurantoin's stopping allowed a clinical and radiological improvement., Conclusion: A good medical supervision is important when nitrofurantoin is prescribed for a long time. The treatment has to be stopped when respiratory symptoms appear to allow an improvement of the symptoms.

Introduction: Pancreatic involvement in the course of wegener's granulomatosis is rarely reported., Exegesis: We report a case presenting as a pancreatic tumorlike lesion. We also discuss six further cases from the medical literature. Two of them had a tumorlike lesion, three had acute pancreatitis and the last had exocrine pancreatic insufficiency., Conclusion: Treatment, association of corticoid and cyclophosphamide, is efficient if it is started quickly. So we think that this unusual manifestation of Wegener's granulomatosis should be recognized.

Purpose: Study of characteristics of ocular involvement in systemic vasculitis., Methods: We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature., Results: Our patients suffered from Wegener's granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis., Conclusion: Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.

Purpose: Mesenteric venous thrombosis is an uncommon entity whose diagnosis is difficult because the symptoms are nonspecific. Yet early recognition of this disease helps avoid the evolution towards mesenteric infarction., Methods: We describe clinical symptoms and hypercoagulable states discovered in six patients with superior mesenteric venous thrombosis., Results: Five of six patients were admitted to hospital for abdominal pain whose mean duration was 9 days before the diagnosis of venous thrombosis was made. Three of them had a fever. Two patients had a past history of deep venous thrombosis. All patients had a hypercoagulable state and three of them had two associated disorders of coagulation. In all cases, abdominal CT scan had permitted the diagnosis of mesenteric venous thrombosis. An anticoagulant therapy was conducted successfully in five of six patients., Conclusion: A CT abdominal scan done early in the case of nonspecific abdominal pain, since the patients had a previous history of venous thrombosis, may permit a nonsurgical treatment of mesenteric venous thrombosis. A screening for thrombophilia must always be carried out since coagulation disorders are very frequently the recognised causes in mesenteric venous thrombosis.

Purpose: Chronic urticaria is a common skin disorder. The cause is rarely determined. Autoimmune diseases, particularly autoimmune thyroiditis, have been implicated in the occurrence of chronic urticaria., Methods: We reviewed clinical records of patients with Hashimoto's disease and chronic urticaria., Results: In our department, six patients had presented chronic urticaria associated with Hashimoto's thyroiditis: four patients, of which three treated with L-thyroxine were euthyroid, the other two were hypothyroid. Hashimoto's thyroiditis had been diagnosed for three patients during the investigation of chronic urticaria. Three patients developed chronic urticaria though they were treated with thyroid suppression for Hashimoto's disease. Two of them had a dramatic improvement with opotherapy. One patient who was euthyroid without treatment improved with hormonal therapy. The fourth patient had a partial remission with thyroid hormones and was cured with corticotherapy., Conclusion: The mechanism by which thyroid autoimmunity is associated with urticaria is poorly understood. A cross-linking of IgE receptors of mastocytes induced by antithyroid antibodies may be a cause of histamine release. Hormonal therapy may be a potent event for the clinical improvement by the suppression of chronic thyroid stimulation. Assay of thyroid hormone and antithyroid antibodies should be performed in patients with chronic urticaria. Discovery of Hashimoto's thyroiditis with chronic urticaria requires thyroid hormone replacement not only in hypothyroid but also euthyroid patients.

Purpose: Henoch-Schonlein purpura is a rare eventuality in adulthood. Abdominal involvement can worsen the short-term prognosis and justify medical treatment to avoid surgery., Methods: Abdominopelvic computed tomography (CT) was performed in six adult patients (four men and two women; age range, 19-74 years) with Henoch-Schonlein purpura before any treatment. Abdominal symptoms were concomitant with the purpuric rash in two cases and followed it in the other cases., Results: The small bowel was always involved with a parietal thickening, and the target sign was observed in three patients. The lesions were located in the duodenum in two patients, the jejunum in two and the ileum in five. Two segments were involved in one patient and three segments in another patient. A peritoneal effusion was always present. No colonic lesion was discovered on CT. Once the results were known, all patients received steroids with initially intravenous methylprednisolone in five patients at doses ranging from 80 to 1,000 mg/day. On follow-up, none of the patients underwent surgery., Conclusion: Abdominopelvic CT scan demonstrated signs suggestive of intestinal vasculitis. An underlying neoplasia or an eventual other cause of abdominal pain were explored and a steroid therapy was then began with a good outcome. Abdominal CT can be helpful in the diagnosis of abdominal involvement in Henoch-Schonlein purpura.

Introduction: Before the HIV infection era, plasmocyte tumor rarely occurred in patients younger than 40 years of age. Less frequent than lymphomas, the incidence of these blood diseases has however substantially increased in HIV-infected patients. In these patients, in addition to onset at earlier age, their clinical presentation is quite different and extramedullary plasmocytomas in unexpected locations are more common., Exegesis: We report the case of a 29-year-old HIV-infected female patient in whom were diagnosed occipital, parotidal, sphenoidal, epidural, and uterine plasmocytomas for which chemotherapy and subsequent radiotherapy were successful. The increase in the incidence of plasmocyte tumors in HIV-infected patients might be facilitated by Epstein Barr Virus (EBV) co-infection, HIV-related chronic antigenic stimulation, and secretion of interleukin 6 by infected lymphocytes., Conclusion: Plasmocyte tumors belong to neoplasia whose incidence is increased in HIV infection. Their currently poor diagnosis should be improved by highly active antiretroviral therapies allowing enhanced chemotherapy with possibility of autograft.

Background: Paraneoplastic digital ischemia is an uncommon complication of metastatic adenocarcinomas., Case Report: Two years after remission of an uterine adenocarcinoma, the patient developed an acrosyndrome involving all four limbs with digital ischemia. Recurrent carcinoma was evidenced by a very high antinuclear antibody titer. Chemotherapy improved the acrosyndrome., Discussion: Vasomotor disorders which developed in older subjects with no other signs of autoimmune disorders should suggest a neoplastic origin. Icshemia of the fingers would be caused by vasculitis. An elevated antinuclear antibody titer may be a supplementary argument suggesting a neoplastic etiology.

Background: Pheochromocytoma and primary hyperaldosteronism rarely occur simultaneously. Few cases have been reported in the literature., Case Report: A patient explored for hypertension was found to have hypokalemia related to primary hyperaldosteronism. Pathology examination of the ablated adrenal showed a co-existing pheochromocytoma suspected at history taking although urine catecholamines were normal., Discussion: Different pathogenic hypothesis have been proposed. Such dual tumors could be a simple coincidence, occur in a particular genetic setting, be related to direct contact between cortical and medullary tissue leading to reactional cortical hyperplasia, pheochromocytoma produced factors stimulating aldosterone synthesis, or factor X, a substance produced by cortical adenomas and favoring growth of the pheochromocytoma.