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L’ipotensione ortostatica (IO) e definita da una riduzione della pressione arteriosa sistolica di 20 mmHg o di quella diastolica di 10 mmHg, al momento del passaggio in posizione eretta. L’IO e spesso associata a un’ipertensione arteriosa (ITA) e rappresenta, d’altra parte, un fattore di rischio cardiovascolare indipendente. L’arco baroriflesso, la rete venosa e il sistema renina-angiotensina sono i principali attori della regolazione. Le eziologie sono numerose, ma l’iter diagnostico si fonda sul carattere simpaticotonico o meno dell’IO, permettendo di distinguere le malattie che alterano o meno il funzionamento del sistema simpatico. Sono da ricercare alcune eziologie curabili e, particolarmente, la iatrogenia. Viceversa, le affezioni croniche come il diabete o le malattie neurodegenerative (Parkinson, atrofia multisistemica) sono spesso di diagnosi evidente, ma con una prognosi piu infausta. Il trattamento associa l’educazione del paziente, le misure fisiche e i mezzi farmacologici, al primo posto dei quali vi sono il fludrocortisone e la midodrina. I farmaci di seconda linea sono l’eritropoietina e gli analoghi dell’octreotide. Le difficolta del trattamento risiedono nella frequenza degli effetti collaterali, ma il fattore limitante principale e la comparsa di un’ipertensione da decubito.

This review focuses on auto-immune diseases which frequently affect women and can have interactions with pregnancy: lupus erythematosus (LES), antiphospholipide syndrome (SAPL), Sjogren's syndrome (GS), rheumatoid arthritis (PR) and auto-immune thyroiditis. LES may flare at the end of a pregnancy and during post-partum. Its biological monitoring during pregnancy is well established. SAPL is at risk of sterility, prematurity, Hellp syndrome, eclampsia and retroplacental hematoma. The main risk, actually risk is foetal loss by placental ischemia, which has to be well known as 2 randomised studies have proven the efficacy of treatments with aspirin +/- subcutaneous heparin. LES, GS and PR can both be associated with anti SS-A +/- anti SS-B antibodies linked to a risk of congenital auriculo-ventricular block, which is fortunately low (less than 5% of the cases). Auto-immune thyroiditis are often revealed during pregnancy and may be enhanced during the six first months of post-partum.

Reported here is the case of a patient who spontaneously recovered from hemophagocytic syndrome associated with acute B19 infection and concomitant Epstein-Barr virus reactivation. The previously healthy 37-year-old-man was hospitalized after 10 days of high fever, arthralgia and arthritis and was determined to have hemophagocytic syndrome. Immunoglobulin (Ig) M antibodies to Epstein-Barr virus (EBV) capsid antigen, early antigen and parvovirus B19 (B19) were found. B19 DNA and low-level EBV DNA were detected in bone marrow, serum and peripheral blood mononuclear cells. The patient recovered spontaneously without any treatment. Two months later anti-B19 IgG antibodies were detected, while at 9-month follow-up, anti-B19 IgM antibodies were no longer detectable and B19 DNA had disappeared from serum. To the best of our knowledge, this is the first report of spontaneous resolution of hemophagocytic syndrome associated with acute B19 infection and concomitant EBV reactivation in an otherwise healthy adult.

Recurrent fetal losses indicate screening for antiphospholipid antibodies, especially after the third consecutive fetal loss, or when they occur after 12 weeks gestation or when the mother presents with thrombosis or other ailments of antiphospholipid syndrome. Fetal loss may be caused by thromboses of placental vasculature. There is no agreement concerning the mechanism of thromboses: protein C pathway and/or annexin V are the best candidates. When fetal loss occurs early during gestation, murine models suggest that antiphospholipid antibodies can also act on trophoblasts by inhibiting syncytia formation. Among the high risk patients with more than two fetal losses, an association of aspirin and heparin given early during gestation is successful in 70–80% of cases.

Resume La bronchiolite obliterante est exceptionnelle au cours du lupus. Il n'existe pas de schema therapeutique et son pronostic reste sombre. Nous rapportons le cas d'une jeune fille de 18 ans, atteinte d'une bronchiolite obliterante (pas d'histologie specifique) pour laquelle plusieurs traitements ont ete tentes sans succes : corticotherapie orale, plasmaphereses, bolus de cyclophsophamide. Seuls les bolus de methylprednisolone ont permis une nette amelioration de son etat respiratoire.

Septic arthritis of the pubic symphysis is uncommon, and usually occurs in patients with predisposing conditions (female incontinence surgery, sports). Staphylococcus aureus and Pseudomonas aeruginosa are the main bacteria responsible of these infections. Streptococcal infections of the pubic symphysis are uncommon. We report three cases of streptococcal infections of the pubic symphysis that occurred in the absence of predisposing condition such as surgery or endocarditis. The diagnosis of septic arthritis was difficult, particularly in one patient who underwent an orchidopexy for a suspected of spermatic cord torsion before diagnosis was corrected. All three patients had a favourable outcome after an antibiotic treatment combining amoxicillin and rifampicin. Septic arthritis of the pubic symphysis should be suspected in patients with sudden groin pain, pubic tenderness and fever to avoid traumatic treatments and useless investigations.

Among diagnostic progress over the last three years in internal medicine, Antisynthetase Syndrome is now more easily recognised with the diffusion of laboratory tests for research of antibodies against tRNA synthetases (Anti JO1, anti PL7, Anti PL12). In two third of cases, these antibodies are found despite absence of antinuclear antibodies. Hence, we have to search them specifically in patients with polyarthritis associated with myositis, cutaneous manifestations (Raynaud phenomenom and "mechanic'hands") and interstitial lung disease. Discovery of asymptomatic mutation in the L ferritin coding sequence help us to better understand the "unexplained" hyperferritinemia. Initially described by japonese gastroenterologists, auto immune pancreatitis in fact a part of a systemic sclerosing disease with a biochemical hallmark: in crease of a subclass of immunoglobulins G (IgG4). A new pediatric disease due to a deficiency of the interleukin1 receptor antagonist (multifocal aseptic osteitis, periostitis, stomatitis, disseminated pustulosis) help us to better understand unexplained auto inflammatory diseases. The therapeutic progress is primarily due to an explosion of biological therapies, particularly four of them very useful for internists (in an off label use) : Interleukin 1 inhibitors (anakinra, Canakinumab) to treat some auto inflammatory diseases (cryopirin associated periodic syndromes and deficency of interleukin 1 receptor antagonist), monoclonal antibody against interleukin 5 (mepolizumab) to treat some hypereosinophilic syndromes and Churg and Strauss angiitis, interleukin 6 inhibitiors to treat multifocal Castleman's disease and adult Still disease, a monoclonal antibody against vascular endothelial growth factor (Bevacizumab) to treat hereditary hemorrhagic telangiectasia.

A retrospective study was undertaken of 120 children with systemic lupus erythematosus (SLE) seen in Paris and its immediate suburbs who fulfilled at least four of the American College of Rheumatology diagnostic criteria for SLE, and in whom the disease was diagnosed before the age of 16 and between January 1975 and December 1987. Eleven of these children (eight girls and three boys) all more than 10 years of age (mean follow up 8.1 years; range 3-13) had thrombotic episodes (9%). Thrombosis was one of the presenting signs in seven patients; in five it was associated with typical symptoms of SLE, and in the other two the thrombotic episode was isolated and diagnosis of SLE was delayed one and three years. Of a total of 16 thrombotic episodes (six of which were recurrent), 14 involved the leg veins, and in four there was associated pulmonary embolism. There were two episodes that affected cerebral arteries. The American College of Rheumatology diagnostic criteria for SLE as well as the incidence of lupus anticoagulant, positive direct Coombs test, and vasculitis in this group of patients was compared with the incidence in patients with SLE but no thrombosis. Only lupus anticoagulant was significantly associated with thrombotic episodes: eight of 11 (73%) of patients with SLE and thrombotic (arterial or venous) episodes had lupus anticoagulant compared with only 10 of 74 patients (14%) with no history of thrombotic events in the same age group.

The hypereosinophilic syndromes (HES), defined by an unexplained and sustained hypereosinophilia, can be associated with heterogeneous hematological conditions. Several molecular mechanisms underlying the eosinophilia, which remained indeterminate for a long time, have been recently identified. These recent advances allowed a better classification of the various forms of HES and the development of targeted therapies. The role of tyrosine kinases, especially PDGFRA, and the efficacy of tyrosine kinases inhibitors dramatically improved the diagnosis and the treatment of myeloproliferative variant of HES. On the other side, eosinophilia can be driven by IL-5 secreting abnormal and often clonal T cell subsets (lymphocytic variant of HES). The crucial role of this cytokine in eosinophil development, activation and survival leads to the assessment of anti-IL-5 monoclonal antibodies which have recently shown to provide a significant corticosteroid sparing effect in FIP1L1-PDGFRA negative HES patients. Despite these major advances, half of HES remains unexplained (idiopathic HES). Some FIPL1-PDGFRA negative patients respond to imatinib, suggesting the role of other tyrosine kinases (or other partners than FIP1L1 in a fusion gene implicating PDGFRA). Development of new biomarkers is needed to help physicians in the diagnosis, classification of HES and in the choice of a targeted therapy.

This descriptive and epidemiological study was conducted in Mars 2002 in Internal Medicine in order to (1) participate in elaborating a White Book about the speciality, (2) analyse the post-university formation needs of the specialists in Internal Medicine.A questionnaire was sent to all specialists in Internal Medicine listed on the ADELI file (n = 2155). For the first three patients seen in consultation and during hospital stay, questioned specialists had to mention the age, sex, origin, motive of the visit, nature of symptoms, complexity of the problem and the nature of the required abilities. They also had to precise the main diagnosis of all patients seen in the same day.Three hundred and sixty answers have been received. Three hundred and thirty two were exploitable. Five thousand six hundred and eleven main diagnosis were listed. Fifteen percent of the questioned specialists did practise in other specialities than Internal Medicine. Orphaned diseases were the most common pathologies carried out in consultation (17%). Patients seen during their hospital stay suffered more frequently from infectious, haematological and malignant diseases. In 55% of the cases, patients were seen in second or third line after a visit to a general practitioner or another specialist. The abilities of the Internal Medicine specialist alone were sufficient in 70% of the cases to solve the problem. Complexity of the problem was evaluated by the specialists themselves at about 45/100 on an analogical scale.This study inform the medical community about the type of patients treated by the specialists in Internal Medicine, precise the exact nature of their professional exercise and their real need in medical post-university formation.

This review focuses on auto-immune diseases which frequently affect women and can have interactions with pregnancy: lupus erythematosus (LES), antiphospholipide syndrome (SAPL), Sjögren's syndrome (GS), rheumatoid arthritis (PR) and auto-immune thyroiditis. LES may flare at the end of a pregnancy and during post-partum. Its biological monitoring during pregnancy is well established. SAPL is at risk of sterility, prematurity, Hellp syndrome, eclampsia and retroplacental hematoma. The main risk, actually risk is foetal loss by placental ischemia, which has to be well known as 2 randomised studies have proven the efficacy of treatments with aspirin +/- subcutaneous heparin. LES, GS and PR can both be associated with anti SS-A +/- anti SS-B antibodies linked to a risk of congenital auriculo-ventricular block, which is fortunately low (less than 5% of the cases). Auto-immune thyroiditis are often revealed during pregnancy and may be enhanced during the six first months of post-partum.

Crowned dens syndrome is due to a microcrystalline infringement (hydroxyapatite or calcium pyrophosphate) of the retro-odontoidal ligament of atlas, often leading to the erroneous diagnosis of meningitis or spondylitis. We report on three new cases diagnosed from 1996 to 1999.The patients complained of cervicalgies, headaches or fever. The initially evoked diagnoses were meningitis, spondylodiscitis or endocarditis. Clinical exam found meningism and an inflammatory syndrome in all patients. Analysis of the cerebro-spinal fluid realised in two cases was normal. The diagnosis of crowned dens syndrome was assessed in two cases by cervical CT scan of C1/C2. In the third case, chondrocalcinosis of a wrist allowed this diagnosis. We report a probably non fortuitous case of crowned dens syndrome associated with genetic hemochromatosis. A non steroidal anti-inflammatory treatment allowed a dramatic regression of clinical symptoms.This entity should be better known; it can mimick numerous diagnosis and be responsible for fever in the long course.

CT is a noninvasive technique that has a major role at each stage of the management of patients with suspected or known lung cancer: in diagnosis; in staging the extension of cancer before surgery or medical treatment; in evaluating the response to the treatment, and in detecting complications [1,2].

The antifibrotic action of interferon alpha on the liver is now established in hepatitis C (cf. article by T. Poynard). Numerous in vitro experimental evidences may lead people into believing in the efficacy of interferon gamma in patients with mucous membrane and pulmonary fibrosis.A randomized study has confirmed the efficacy of interferon gamma in the idiopathic pulmonary fibrosis, probably by an anti TGF beta action. Several open studies have shown the efficacy of interferon gamma in patients with post-radiation cutaneous fibrosis (Chernobyl survivors, sequelae of radiotherapy for cancer). In systemic sclerosis, several teams have shown that interferon gamma reduced collagen synthesis by sclerodermic fibroblasts in vitro. Four open studies have also confirmed the efficacy of interferon gamma in systemic sclerosis. In our experience concerning 20 patients with diffuse systemic sclerosis, the five years survival with long term therapy was 85% and we observed an improvement in the cutaneous suppleness in 40% of these cases. There was no serious side effect.These encouraging results need to be confirmed by large randomized studies in pulmonary fibrosis and systemic sclerosis. Other indications should be assessed, particularly extra-cutaneous radiation sequelae and in systemic fibrosis (retroperitoneal, mediastinal and cervical).

Castleman's disease is a polyclonal lymphoplasmacytic and vascular proliferation prominant in lymphoid tissues. It is associated with lymph node enlargement, hepatosplenomegaly and fever. This manifestations could be secondary to hyperproduction of interleukin 6. The prognosis is poor. The opportunistic infections which are characteristic of severe HIV infection worsen the prognosis. Prolonged monochemotherapy with vinblastine or etoposide can control Castleman's disease.Recent advances in human herpesvirus 8 (HHV8) knowledge and its predominance in the forms which are linked to the HIV seropositivity have partly explained the clinical manifestations of Castleman's disease. Indeed, HHV8 produce an homologous interleukin 6, the vIL-6, responsible for lymphoplasmacytic proliferation. The presence of other homologues of human cytokines produced by HHV8 could contribute to lymphoplasmacytosis and to endothelial proliferation.Taking into account this viral origin, alpha interferon could be an alternative in forms which are less progressive. However, antiviral therapy against HHV8 or HIV and the immunitary restoration do not have any influence on the evolution of Castleman's disease, contrary to opportunistic infections.

Fabry disease is an X-linked recessive abnormality of glycosphingolipid metabolism that is due to deficiency of the lysosomal enzyme alpha-galactosidase A.A majority of hemizygous men develop severe multisystemic disease (classic form), dominated by renal failure, progressive neurological and cardiac involvement. Nevertheless, some affected men retain sufficient enzyme activity and long remain asymptomatic (atypical form); their main manifestation is hypertrophic cardiomyopathy. Female heterozygous carriers are usually asymptomatic; 15% of them, however, have severe involvement of one or several organs. Laboratory, histologic and molecular diagnosis identifies 100% of hemizygous and over 80% of heterozygous subjects.With developments in molecular genetics, it is now possible to produce the human recombinant enzyme alpha-galactosidase A. Two recent studies had proven that this therapeutic approach was able to be clinically and histologically effective in men. In addition, the results of a trial of gene therapy in a Fabry gene knocked-out mouse appear promising.

We describe a 50-year-old man with relapsing polychondritis (RP) involving auricular cartilage, uveitis and hearing loss, who had an aneurysm of the anterior cerebral artery. Intracranial aneurysm is a rare manifestation of RP.

Antiphospholipid syndrome is the most frequent cause of acquired thrombophilia. Aspirin may have some indications. CURRENT KONWLEDGE AND KEY POINTS: The usefulness of low doses of aspirin is now well demonstrated in the prevention of obstetric complications associated with antiphospholipid antibodies (especially pregnancy loss). When heparin is combined with low-dose aspirin, the recurrent rate of fetal loss is lower than 30%. In patients with arterial or venous thrombosis, there is a high rate of recurrence during the two first years except if high-dose warfarin was used (i.e., INRor = 3). The association warfarin-aspirin in secondary prevention of thrombosis may be evaluated in prospective studies. It is not so clear in the literature and in our experience that warfarin is superior to aspirin in stroke recurrence prevention in patients with antiphospholipid antibodies, except in Sneddon's syndrome. There are no guidelines in primary thrombosis prevention in patients with antiphospholipid antibodies. In lupus patients, aspirin may not be sufficient after many years of follow-up in preventing a first episode of thrombosis. Prospective studies may be undertaken. Atherosclerotic patients with antiphospholipid antibodies are particularly exposed to the risk of thrombosis after revascularisation or angioplasty and stent implantation. Aspirin may have a place in those patients but these must be evaluated. FUTUR PROSPECTS AND PROJECTS: Except in prevention of obstetric complications, the usefulness of aspirin in patients with antiphospholipid antibodies must be evaluated in prospective studies.

We report four cases of the side effects of minocycline seen during the last two years in our department. There was one case of drug-related lupus and three cases of hypersensitivity reactions, including one eosinophilic pneumopathy with pericarditis, one nephropathy and one severe, pseudo-infectious episode of high fever, rash, lympadenopathy, hepatitis and eosinophilia. Minocycline is a tetracycline agent widely used for acne therapy in France and all over the world. During the last few years, there has been an increasing number of reports concerning systemic adverse reactions to minocycline, with on the one hand auto-immune disorders (lupus, autoimmune hepatitis, vascularitis with ANCA), occurring after a prolonged course of therapy and reported recently in the last few years, and on the other hand, hypersensitivity reactions (eosinophilic pneumopathies, hepatitis, nephropathies, myocarditis, serum sickness or pseudo-infectious reactions), occurring precociously in the course of therapy, and potentially severe. Although these side effects are uncommon in the context of the high number of patients who have been prescribed the drug, the first-line antibiotic therapy in acne must probably be reconsidered.

We describe 3 patients in whom the onset of systemic scleroderma occurred shortly after ionizing irradiation for nasopharyngeal or breast carcinoma. This relationship has been described rarely as has the exacerbation of a preexisting scleroderma after irradiation. This gives indications for direction of studies.

The antiphospholipid syndrome was individualized 12 years ago. Treatment was initially based on steroids, immunosuppressive drugs and intravenous immunoglobulin therapy. More recently, several retrospective studies have established that in most clinical conditions therapeutic doses of oral vitamin K antagonists (INRor = 3) are sufficient to control the disease.However, high dose immunoglobulin therapy is still indicated in a few cases, especially in life-threatening immune peripheral thrombocytopenia, and in recurrent foetal loss: in the latter indication, immunoglobulin therapy alone is efficient in 80% of cases.Prospective studies are needed to assess the efficacy of intravenous immunoglobulin therapy in neurological complications occurring in spite of anticoagulant therapy, and in the context of repeated foetal losses when antithrombotic therapy with aspirin and subcutaneous heparin has failed.

OBJECTIVE: Pulmonary artery involvement in Takayasu's arteritis is suggestive of the disease. Our aim was to use electron beam CT to study pulmonary artery changes in patients with Takayasu's arteritis. CONCLUSION: Diffuse wall thickening and dilatation or stenosis were significantly more frequent on CT in patients with Takayasu's arteritis than in the control subjects. Knowledge of these findings may be of value when assessing pulmonary artery involvement and thus may be helpful for the diagnosis of Takayasu's arteritis.

Up to 15% of patients with temporal arteritis, may have large vessel involvement. The ascending aorta, aortic arch, subclavian and axillary arteries are most frequently involved. These localisations may be the presenting feature but usually, temporal arteritis is known or symptomatic at the time of large vessel involvement discovering. When corticosteroids are given in adequate doses, the response is favorable in most patients with limbs localisation but aortic localisation is associated with a markedly increased risk for the development of aneurysm and dissection which may cause death.

Decreased susceptibility to penicillin G of pneumococcal strains is continuously increasing in France.We assessed effect of resistance to penicillin on therapeutic management and mortality in adults with pneumococcal pneumonia in our hospital.This one-year retrospective study (1995) included patients with proven pneumococcal infection (positive blood culture, pleural fluid, or specimens from the lower respiratory tract). Strains of Streptococcus pneumoniae were screened for susceptibility to antimicrobial agents. Resistance to penicillin G was defined as a minimal inhibitory concentrationor = 0.12 microgram/ml. Age immune and nosocomial status, first and second line antibiotherapy and death were compared according to the strains susceptibility to penicillin G. A p value below 0.05 was statistically significant.In 15 cases a pneumococcal strain susceptible to penicillin G was isolated while 23 patients were infected with a strain with a decreased susceptibility to penicillin G. Age was significantly higher in the latest group (61.6 versus 54.7 years) while no difference was noted between the 2 groups according to immune and nosocomial status, therapeutic management and death.Resistance to penicillin did neither appear to increase mortality nor to influence therapeutic management in patients with pneumococcal infection.

Aortic arch dissection may be sometimes misdiagnosed due to the lack of mild to moderate chest pain. Definite diagnosis is often made while dissection has already occurred more than 15 days ago, being thereafter considered as chronic. Aortic dissection may then present as a prolonged febrile illness with fever and/or inflammation as main symptoms, with little or no pain.We retrospectively reviewed cases of chronic aortic dissections seen in a department of internal medicine and a department of neurology between 1975 and 1992.We report six cases of patients presenting with aortic dissection and describe their outcome and treatments after the diagnosis was made based on either thoracic computerized tomography or trans-esophageal echocardiography evidence. Four patients had surgical aortic arch repair while one patient was treated with beta-blockers.Chronic aortic dissection has rarely been reported to cause fever or increased sedimentation rate. Treatment has to be discussed between medical and surgical teams involved in the therapeutical management of these unusual patients.