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Background: Primary Sjögren's syndrome is associated in 6 to 25% of cases with kidney involvement Most often it is a chronic interstitial nephritis, generally asymptomatic but sometimes responsible for distal tubular acidosis which can be complicated by severe hypokalemia., Case Report: A 48-year-old woman had hypokalemic quadriplegia, distal renal tubular acidosis and chronic interstitial nephritis at kidney biopsy. Primary Sjögren's syndrome was diagnosed., Discussion: We discuss briefly the clinical, biological and immunological features of 18 similar cases reported in the literature.

Introduction: Necrobiotic xanthogranulomatosis is a rare cutaneous disorder usually associated with monoclonal gammopathy. Most frequently, cutaneous lesions involve the face (periorbital region) and the trunk, and are characterized by indurated xanthomatous plaques and nodules., Exegesis: We describe a patient presenting with necrobiotic xanthogranulomatosis that was unusual, as it was associated with IgM kappa monoclonal gammopathy of undetermined clinical significance and because no periorbital involvement was observed., Conclusions: Necrobiotic xanthogranulomatosis should be distinguished from other cutaneous manifestations associated with plasma cell dyscrasias, such as normolipemic plane xanthoma. It can be associated with IgM gammopathy.

Background: The occurrence of gastric carcinoid tumors during the cure of pernicious anemia has been reported in the literature. Concerning a new case, we review physiopathological data of this infrequent association., Case Report: We report the case of a 47-year-old women with pernicious anemia in which gastroscopy found two small tumors developed from enterochromaffin-like cells., Conclusion: These tumors are usually latent and developed from enterochromaffin-like cells. The pathologic and histologic diagnosis is based on positive chromogranin A immunomarking. The physiopathological mechanism studies are in favour of the predominant part of hypergastrinemia following achlorhydria. Usually, evolution is benign and surgical or endoscopic resection recommended.

Medical treatment of systemic idiopathic fibrosis is first based on steroid therapy, of which efficacy is well known for nearly 75% of cases of retroperitoneal fibrosis, but les certain for the other localisations of the disease. Studies on the immuno-pathological mechanism of systemic fibrosis have led to immunosuppressive drug use, such as azathioprin, cyclophosphamide and methotrexate. Tamoxifen has been tried because of its efficacy on desmoid tumors and seems to be of great interest. Attention is focused on fibrosing cervicitis which may also be treated with immunosuppressive drugs.

Porphyria cutanea tarda is a metabolic disorder caused by reduced hepatic uroporphyrinogen decarboxylase activity characterized by skin lesions and liver damage. The high frequency of liver histological damage in patients with porphyria cutanea tarda led us to study hepatitis B and C viral infection which has a striking prevalence in southern Europe. We attempt here to expose different pathophysiological hypotheses: is viral hepatitis a triggering factor precipiting latent decreased uroporphyrinogen decarboxylase activity or does it directly induce the enzyme deficiency?

We report a case of the uncommon association of dermatomyositis and primary biliary cirrhosis in a causasion male of 48-year-old. Diagnosis of dematomyositis was made because of muscle weakness, loss of weight, skin telangiectasia, elevated serum concentration of creatine kinase, polyphasic low amplitudes waves on electromyography and histologic confirmation on muscle biopsy. Diagnosis of primary biliary cirrhosis was made because of elevated values of alcaline phosphatase and gamma glutamyl transferase, elevated values of type II mitochondrial antibody and compatible histological lesions on liver biopsy. We found only ten case reports associating polymyositis and primary biliary cirrhosis. We hypothesize that hepatic and muscle mitochondrial dysfonction may be involved.

Two cases of lupus dementia presented many points of particular interest: 1) the progressive installation of intellectual deterioration, inaugural for the first observation; 2) the diagnostic difficulties of neurolupus with the ARA criteria; 3) the appearance of cerebral magnetic resonance imaging with confluent hypersignals of the periventricular white matter on T2-weighted images; 4) the patholophysiological hypotheses: vascular disease? immunologic disease?; 5) the clinical improvement and SPECT amelioration for the second patient with corticosteroids.

Type IV Ehlers-Danlos syndrome, a rare disease caused by abnormal synthesis of type III collagen, often leads to vascular fragility. We report 9 cases (6 men and 3 women, mean age 35 years). For 7 of the patients, the inaugural signs were arterial complications including haemoperitoneum in 2 patients with multiple aneurysmal dystrophy of the abdominal arteries, one case of ruptured subclavian artery, two dissections of the renal artery, one case with rupture of a cerebral aneurysm, one rupture of the mesenteric artery and a haematoma after arterial puncture. Other vascular manifestations were acrosyndrome (n = 4), varicose veins (n = 3), and prolapsus of the mitral valve (n = 2). In addition, 8 of the 9 patients presented extravascular signs. There was a history of familial disease in 5 cases. Pregnancy was completed to term in three patients: a cesarean section was required in one case and intra-uterine growth retardation was seen in 2. Morbidity was important with hemiparesia, blindness and paraparesis sequellae. One patient died from haemorrhage. This series of patients with type IV Ehlers-Danlos syndrome illustrates the severity of this disease whose prevalence is often underestimated. The disease is transmitted by autosomal dominant inheritance, underscoring the importance of familial testing for early diagnosis. Clinicians should be aware of the vascular manifestations and avoid invasive punctures or operations except in exceptional indications.

Objective: A prospective study of deep vein thrombosis was conducted to compare diagnosis with venous echo-Doppler and phlebography in 101 hospitalized patients with symptomatic lower limbs., Methods: Phlebography was used as the reference examination to evaluate the sensitivity and specificity of echo-Doppler for lesions at all levels of the limb., Results: Overall sensitivity was 90% and specificity 72%. The prevalence of venous thrombosis was 50% symptomatic patients and the positive predictive value of echo-Doppler was 76%. The negative predictive value was 88%. For patients with proximal signs, there were no false negatives with echo-Doppler and for those with distal signs, particularly in patients who had recently undergone surgery, were obese or had a past history of deep vein thrombosis echo-Doppler gave less satisfactory results., Conclusion: Echo-Doppler can be proposed as a first intention examination for the search for deep vein thrombosis in patients with clinical signs in the proximal area of the leg, while phlebography is still required for more distal areas.

Simultaneous elevation of thyreostimulin and thyroid hormones values, when associated with clinical hyperthyroidism, raises a dual problem of diagnosis and treatment. We report a case of hyperthyroidism with elevated thyreostimulin in a young adult man. The values of free triiodothyronin and free thyroxin were elevated and the thyroxin binding globulin was normal. A normal pituitary tomodensitometry, the normal values of the alpha sub-unit of thyreostimulin and the dynamic tests of thyreostimulin secretion allowed us to rule out the hypothesis of a pituitary adenoma. Non-tumoral inappropriate secretions of thyreostimulin are at present regarded as syndromes of resistance to thyroid hormones. Their biological translation is a simultaneous elevation of thyreostimulin and thyroid hormones. Peripheral resistance, which is rarely complete, finds its expression in clinical hyperthyroidism or in normal clinical condition, whereas pituitary resistance, which causes the lack of feedback on the secretion of thyreostimulin expresses itself by clinical hyperthyroidism, sometimes with goitre owing to the trophic action of thyreostimulin on the thyroid. Generalized syndromes of resistance are the most common. Our patient had a selective pituitary resistance to thyroid hormones and less than thirty cases are reported in the literature. Treatment with a beta-blocker remains indicated when facing clinical evidence for hyperthyroidism and oral triiodothyronin seems to be able to slow down the inappropriate secretion of thyreostimulin. Regarding our patient who experienced a clinical and biological relapse after the stopping of the treatment, we join many authors who recommend a prolonged treatment.

10% of young male healthy volunteers have a total bilirubin value over 20 mumol/l; thus such a value appears not relevant as screening cut off point in clinical pharmacology. This study was intended to confirm if a 27 mumol/l cut off point previously defined by the authors does not support a risk. This study dealt with 487 subjects who had together measurements of total bilirubin value and lab. tests of liver cytolysis, cholestasis or hemolysis during the selection process. 48 subjects (9.8%) had a total bilirubin value over 20 mumol/l. Correlation tests do not provide arguments of cytolysis, cholestasis or hemolysis and there was no argument in favor of Gilbert's syndrome. Out of 48 hyperbilirubinemic subjects only 22 were included in clinical pharmacology studies. In more than 60%, the total bilirubin value returned to normal spontaneously and in no case appeared a significant clinical, biological, pharmacokinetic or dynamic abnormality. Except a possible increase of slow acetylor frequency, the medical literature analysis does not show any relevant modification in metabolism, pharmacokinetics or pharmacodynamics until a 40 mumol/l value of total bilirubin. Thus, the 27 mumol/l value of total bilirubin previously proposed is confirmed as a useful limit that does not lead to an additional risk.