[Natural history of portal cavernoma without liver disease. A single centre retrospective study of 32 cases]. / Évolution naturelle des cavernomes portaux non liés à une hépatopathie. Étude rétrospective monocentrique de 32 observations.

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.

[Visceral larva migrans with cardiac manifestation: a case report and literature review]. / Syndrome de larva migrans viscéral avec atteinte cardiaque: une observation et revue de la littérature.

INTRODUCTION: Toxocariasis is a roundworm infection that may be associated with serious cardiac manifestations. We report one case and review another 12 cases in the literature. CASE REPORT: A 74-year-old man, presented with clinical features of myopericarditis. Eosinophil count was 20,000/mm(3). The ELISA and Western Blot were positive for Toxocara canis. The patient was treated with corticosteroids and antiparasite treatment. The outcome was rapidly favorable. DISCUSSION: In the other 12 cases, the cardiac manifestations were often severe: six myocarditis, three tamponades and three Loeffler's endocarditis have been published. Four therapeutic strategies have been reported: symptomatic treatment alone was associated with worse progressive; antiparasite treatment alone or companied with corticosteroids and corticosteroids alone had good results. CONCLUSION: The toxocariasis should be investigated systematically in case of eosinophilic cardiomyopathy. The treatment strategy is still controversial.

[Recurrent pericarditis related to primary pericardial malignant mesothelioma]. / Péricardite récidivante : traquer le mésotheliome péricardique primitif.

INTRODUCTION: Most of recurrent pericarditis are idiopathic and only 15 to 20% have a specific diagnosis. Primary pericardial mesothelioma is a rare cause of recurrent pericarditis. Diagnosis can be challenging and antedates patient's death in only 10 to 20% of cases. Histology of mesothelioma and immunohistochemistry are mandatory for the diagnosis. Median of survival before using pemetrexed was about 6 months after diagnosis. CASE REPORT: We report the history of a 64-year-old woman for which repeated biopsy for recurrent pericarditis was necessary to diagnose a primary pericardial mesothelioma. The first biopsy had only found mesothelial hyperplasia. CONCLUSION: This case report highlights the necessity of repeat pericardial biopsy in the case of adverse outcome.

[Stress and auto-immunity]. / Stress et auto-immunité

The etiology of auto-immune disorders is multifactorial. Stress is probably a participating factor. Indeed, a high proportion of patients with auto-immune diseases report uncommon stress before disease onset or disease flare. The biological consequences of stress are increasingly well understood. Glucocorticoids and catecholamines released by hypothalamic-pituitary-adrenal axis during stress will alter the balance Th1/Th2 and the balance Th17/Treg. Stress impairs cellular immunity, decreases immune tolerance and stimulates humoral immunity exposing individuals to autoimmune disease among others. The treatment for autoimmune disease should include stress management.

Is IgG4-Related Disease a Cause of Xerostomia? A Cohort Study of 60 Patients.

Objective. Immunoglobulin-G4-(IgG4-) related disease (IgG4 RD) is a fibrosing process characterized by a significant infiltration of IgG4-secreting plasma cells. IgG4 RD can affect almost all organs including salivary glands. Whether IgG4 RD plays a role in the development of sicca syndrome and particularly dry mouth syndrome remains to be investigated. Methods. We conducted a monocentric cohort study for two years to search for IgG4 RD features in patients with dry mouth syndrome using immunostainings of labial salivary gland specimens with anti-IgG4 antibody. Results. Among 60 patients presenting with dry mouth syndrome who underwent labial salivary gland biopsy, 18 showed positive immunostaining with the anti-IgG4 antibody including 4 patients with typical systemic IgG4 RD. Five also fulfilled criteria for Sjögren's syndrome. Conclusion. These findings suggest that clinical forms of IgG4 RD salivary involvement without salivary swelling may occur. This salivary involvement is probably overlooked in everyday practice and could represent a mild form of IgG4 RD.

[Mesenteric panniculitis]. / Panniculite mésentérique.

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.

[Abdominal manifestations of Henoch-Schönlein purpura in adults. A retrospective study of 23 cases]. / Atteintes digestives du purpura rhumatoïde de l'adulte. Etude d'une série rétrospective de 23 patients.

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.

[Pituitary metastasis presenting as diabetes insipidus: a report of four cases and literature review]. / Diabète insipide révélateur de métastases hypophysaires : quatre observations et revue de la littérature.

INTRODUCTION: Pituitary metastases are a rare cause of central diabetes insipidus and usually complicate advanced cancers. CASE REPORTS: We report four cases in which diabetes insipidus revealed a metastatic stage of a lung (two cases) or a breast (two cases) cancer. One patient presented with a panhypopituitarism, three had diabetes insipidus including one with corticotroph insufficiency. In one case, the cerebrospinal fluid analysis showed a lymphocytic meningitis. On brain magnetic resonance imaging there was an absence of high intensity signal in the posterior pituitary lobe (one case) or an infiltration of the posterior lobe (three cases). CONCLUSION: Breast and lung malignancies are the most frequent causes of metastasis of the pituitary gland. In most cases there are often other metastatic locations; however insipidus diabetes can reveal the metastatic stage of the cancer. A pituitary biopsy may be necessary to obtain the diagnosis.

[Intra-alveolar haemorrhage without renal damage as the initial presenting feature of Goodpasture's syndrome: case report and review of literature]. / Hémorragie intra-alvéolaire isolée révélant un syndrome de Goodpasture sans atteinte rénale : à propos d'une observation et revue de la littérature.

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.

[Nephrotic syndrome: don't forget to search for hypothyroidism]. / Syndrome néphrotique: penser à rechercher une hypothyroïdie associée.

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.

[Nitrofurantoin-induced lung disease: about two cases]. / Pneumopathie à la nitrofurantoïne: à propos de deux observations.

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.

[Lingual tuberculosis revealing disseminated tuberculosis]. / Tuberculose disséminée révélée par une localisation linguale.

INTRODUCTION: Tuberculous lesions of the oral cavity are uncommon. Most of cases are secondary to pulmonary disease and the primary form is rare. EXEGESIS: We report the case of a 64 year-old man, smoker, presenting a chronic ulcer of the tongue, with anorexia and important weight loss. The biopsy of this ulcer showed granulomatous inflammation and Langhans type giant cells, without necrosis. Ziehl-Nielsen stain was negative. Pulmonary lesions were subsequently detected (chest X-ray, CT-scan) and the disseminated tuberculosis was confirmed by a positive culture with acid-fast bacilli in urine, blood, and pulmonary sample. Antituberculosis treatment resulted in the complete resolution of the oral lesion. CONCLUSION: Biopsy for histopathological diagnosis, acid-fast stains and culture, is essential to determine the exact nature of chronic oral ulceration to distinguish between oral malignancy, infectious (syphilis), traumatic, or aphthous ulcers. Tuberculosis of the tongue is a difficult diagnosis. However it should be searched for because treatment usually results in a rapid recovery.

[Pancreatic involvement in Wegener's granulomatosis: a rare and difficult diagnosis]. / Les atteintes pancréatiques au cours de la granulomatose de Wegener: une localisation rare et un diagnostic difficile.

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.

Intractable hiccup as the initial presenting feature of systemic lupus erythematosus.

An infarctus of medulla oblongata was discovered in a 44-year old man with an intractable hiccup and 10, 11th and 12th right cranial nerves palsies. Systemic lupus erythematosus (SLE) with antiphospholipid syndrome was subsequently diagnosed. Hiccup has withdrew with corticosteroid therapy and low-dose aspirin. The other cases of literature and pathophysiologic hypotheses are briefly reported.

Can procalcitonin measurement help in differentiating between bacterial infection and other kinds of inflammatory processes?

OBJECTIVE: To study the levels of procalcitonin (PCT) in various inflammatory states seen in an internal medicine department and to evaluate the possible discriminative role of PCT in differentiating bacterial infection from other inflammatory processes. METHODS: PCT, C reactive protein (CRP), and white blood cell count (WBC) were measured in patients admitted to the department for fever or biological inflammatory syndrome, or both. The serum of 173 consecutive patients was analysed according to the aetiological diagnosis. The patients were divided into two groups: group I (n=60) with documented bacterial or fungal infection; group II (n=113) with abacterial inflammatory disease. RESULTS: PCT levels were >0.5 ng/ml in 39/60 (65%) patients in group I. In group II, three patients with a viral infection had slightly increased PCT levels (0.7, 0.8, and 1.1 ng/ml) as did two others, one with crystal arthritis and the other with vasculitis (0.7 ng/ml in both cases). All other patients in group II had PCT levels <0.5 ng/ml. In this study a value of PCT >0.5 ng/ml was taken as the marker of bacterial infection (sensitivity 65%, specificity 96%). PCT values were more discriminative than WBC and CRP in distinguishing a bacterial infection from another inflammatory process. CONCLUSION: PCT levels only rose significantly during bacterial infections. In this study PCT levels >1.2 ng/ml were always evidence of bacterial infection and the cue for starting antibiotic treatment.

[Mesenteric venous thrombosis: early diagnosis with abdominal CT scan and screening for coagulopathies. Six observations]. / Thromboses veineuses mésentériques: apports diagnostiques du scanner abdominal et de la recherche d'une coagulopathie. Six observations.

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.