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Objectives: The aim of the study was to compare clinical/imaging findings and outcome in patients with idiopathic (isolated aortitis, IA) and with giant cell arteritis (GCA)-related aortitis., Methods: Patients from 11 French internal medicine departments were retrospectively included. Aortitis was defined by aortic wall thickening >2mm and/or an aortic aneurysm on CT-scan, associated to inflammatory syndrome. Patients with GCA had at least 3 ACR criteria. Aortic events (aneurysm, dissection, aortic surgeries) were reported, and free of aortic events-survival were compared., Results: Among 191 patients with non-infectious aortitis, 73 with GCA and 44 with IA were included. Patients with IA were younger (65 vs 70 years, p=0.003) and comprised more past/current smokers (43 vs 15%, p=0.0007). Aortic aneurisms were more frequent (38% vs 20%, p=0.03), and aortic wall thickening was more pronounced in IA. During follow-up (median=34 months), subsequent development of aortic aneurysm was significantly lower in GCA when compared to IA (p=0.009). GCA patients required significantly less aortic surgery during follow-up than IA patients (p=0.02). Mean age, sex ratio, inflammatory parameters, and free of aortic aneurism survival were equivalent in patients with IA ≥ 60 years when compared to patients with GCA-related aortitis., Conclusions: IA is more severe than aortitis related to GCA, with higher proportions of aortic aneurism at diagnosis and during follow-up. IA is a heterogeneous disease and its prognosis is worse in younger patients <60 years. Most patients with IA ≥ 60 years share many features with GCA-related aortitis., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Purpose: Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided., Methods: A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line., Results: Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n=93), with a mean age of 46 years, graduated from Nantes University (65%, n=86), practicing ambulatory general medicine (69%, n=164) in Loire-Atlantique department area (82%, n=176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n=166) concerning clinical issues (57%, n=155). A sole telephone advice was the main type of answer provided (56%, n=147) and a visit to the emergency room was prevented for 17% of calls., Conclusion: The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5years (range: 1.6-35). Two patients developed Waldenström's macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown., (Copyright © 2014 Elsevier B.V. All rights reserved.)

POEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment. All but one of the patients responded: clinical improvements were noted in neuropathies (16/20) organomegaly (13/13), peripheral edema (14/15), and pulmonary hypertension (5/5). At least a very good partial response was noted in 68% of patients, with partial responses in 26%. Serum VEGF levels fell markedly in all 17 patients with available values. Twelve patients had 18-FDG-PET/CT at diagnosis (11 with positive findings), and nine patients during follow-up. The number of lesions fell markedly in five cases and remained stable in two cases, while two patients became negative. During a median follow-up of 22 months, four patients relapsed. Toxicity, predominantly hematological, was mild and manageable. Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses., (Copyright © 2012 Wiley Periodicals, Inc.)

Unlabelled: Anticholinergic medications are responsible for most frequent adverse drug effects. Two scales have been elaborated as tools for prescribers: the Anticholinergic Drug Scale (ADS) of Carnahan et al., and the Anticholinergic Risk Scale (ARS) of Rudolph et al. The objective of this study was to analyze the diagnostic performance of both scales for predicting signs related to an anticholinergic effect., Method: Medical records of 1379 patients aged 75 years or older hospitalized in a geriatric acute care unit between 2002 and 2005 were studied. The analyze was made retrospectively, but data were collected prospectively., Results: Risk of appearance of total anticholinergic signs (ADS : OR 1,45, CI 95% [1,03-2,03], p=0,037 and ARS : OR 1,98, CI 95% [1,19-3,28] p<0,01) and peripheral signs (ADS: OR 1,66, CI 95% [1,22-2,26], p<0,01 and ARS : OR 1,81, CI 95% [1,19-2,75], p<0,01) increased when score was ≥ 3 with both scales, which wasn't the case for central signs., Conclusion: Both scales permitted to detect an increased risk of appearance of total and peripheral anticholinergic signs, but not the centrals as delirium. Interest of total anticholinergic burden remains to be demonstrated, especially for delirium risk assessment.

We report the case of a patient with mammary implants for 42 years. She presented for three years a change of the general state with clinicobiological demonstrations of indefinite origin, and a progressive bilateral increase of her mammary volume until it became exceptional. The surgery of explantation allowed the retreat of two masses of almost 6kg. The analysis did not put in evidence any malignant process but confirmed that it was about prostheses in polyethylene. In one year of recession, a considerable improvement of her general state was observed., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Background: Classic giant cell arteritis affects older adults who are aged >50 years. Temporal arteritis is uncommon in young adults but juvenile temporal vasculitis (JTV) is the most frequent form found in young people. Clinical presentation is usually poor, with localized temporal inflammatory changes without consistent systemic manifestations. Generally, the patients have a benign clinical course, without ophthalmic or ischemic manifestations. In these rare JTVs, excision of the involved section of temporal artery is often curative and corticosteroid therapy is not required., Method: The present study reports a case of JTV in a middle-aged woman., Results: A 44-year-old woman complained of violent temporal headache, with a slight inflammatory syndrome. She had no vascular systemic manifestation and no cause of secondary vasculitis. Doppler ultrasonography suggested a localized inflammatory arteritis. Temporal biopsy was performed. Histologic findings were compatible with JTV (nongranulomatous panarteritis with mononuclear cells and eosinophils). All the symptoms disappeared after excision. One year later, she remains well and reports neither systemic manifestation nor recurrence., Conclusion: Vasculitis of the temporal arteries in young people is uncommon and JTV is rare in middle-aged people. It is necessary to search for systemic or secondary vasculitis. In contrast to giant cell arteritis, steroids are not required., (Copyright © 2011 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved.)

Germline mutations in CYBB, the human gene encoding the gp91(phox) subunit of the phagocyte NADPH oxidase, impair the respiratory burst of all types of phagocytes and result in X-linked chronic granulomatous disease (CGD). We report here two kindreds in which otherwise healthy male adults developed X-linked recessive Mendelian susceptibility to mycobacterial disease (MSMD) syndromes. These patients had previously unknown mutations in CYBB that resulted in an impaired respiratory burst in monocyte-derived macrophages but not in monocytes or granulocytes. The macrophage-specific functional consequences of the germline mutation resulted from cell-specific impairment in the assembly of the NADPH oxidase. This 'experiment of nature' indicates that CYBB is associated with MSMD and demonstrates that the respiratory burst in human macrophages is a crucial mechanism for protective immunity to tuberculous mycobacteria.

Objective: The prevalence of the involvement of large vessels in giant cell arteritis (GCA) is 3-13%. Aortitis is the most serious complication of GCA. Computed tomodensitometric (CT) scan allows analysis of both the aortic wall and endoluminal part of the aorta. Therefore, we conducted a study using CT scan to analyze aortic abnormalities in patients with recent-onset GCA., Methods: This prospective controlled study compared patients with biopsy-proven GCA with a matched control group based on sex, age, and cardiovascular risk factors. During the 4-week period following diagnosis of GCA, patients underwent an aortic CT scan. The aortic imaging results were blindly compared between both groups., Results: From January 5, 1998 to January 11, 1999, 22 patients and 22 controls were screened by CT scan for aortic involvement. Thickening of the aortic wall was more frequent among patients than controls (45.4% versus 13.6%; P = 0.02). Aortic thickening (mean 3.3 mm) was located on the ascending part of the thoracic aorta in 22.7% of the patients, with no evidence of thickening in the controls (P = 0.05). Thickening of the abdominal aortic wall was noted in 27.3% of the patients and none of the controls (P = 0.02)., Conclusion: This study suggests that inflammatory aortic thickening, detected by CT scan, occurs frequently at the time of diagnosis of GCA, and that this condition predominantly occurs on the ascending part of the aorta.

Background: Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis., Patients and Methods: This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta., Results: Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm., Conclusion: Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

Background: Rare cases of vasculitis restricted to the lower limbs have been reported, but the characteristics, outcome and response to treatment of this entity are not well known., Objective: To describe the clinical, complementary examinations and response to treatment of this rare entity in the first retrospective series, and to compare data with historical pooled cases., Methods: Retrospective analysis of all biopsy-proven cases observed over a 10-year period in four French tertiary medical units. Diagnosis of vasculitis restricted to the lower limb required the absence of any clinical symptom and complementary test finding, suggesting major extramuscular visceral involvement., Results: 11 patients were included. Vasculitis restricted to the lower limb was associated with disabling muscle pain of the calves. Fever was present in 50% of cases; ankle arthralgia in 50% and skin involvement in 40%. MRI was the cornerstone of the diagnosis, showing hyperintense signal in T2 weight and in T1 weight after gadolinium injection. MRI findings correlated well with clinical outcome and were useful in guiding biopsy. Muscle biopsy was consistent with a polyarteritis nodosa-type vasculitis in only 40% cases, whereas a leucocytoclastic vasculitis was seen for all other cases. Treatment with corticosteroids was effective in all cases, but there were relapses requiring immunosuppressive agents in 54% of cases., Conclusion: Vasculitis of the calf muscles must be considered for patients with calf pain and with a biological inflammatory syndrome.

Purposes: Ten to fifteen percent of granulomatous hepatitis are idiopathic. If symptoms like prolonged fever are present, empirical treatment is discussed. The goal of this study is to describe the empirical treatment proposed in this situation by French specialists of internal medicine., Methods: We conducted a practice investigation among the French national society of internal medicine (SNFMI), using an anonymous questionnaire that related a case of idiopathic granulomatous hepatitis. This questionnaire was proposed to all French internists present at the SNFMI congress in June and December 2004. French specialists of internal medicine had to answer if they would prescribe an empirical treatment and if so, to specify this treatment., Results: Thirty-six French specialists of internal medicine answered to the questionnaire. In the proposed situation, 89% of them initiate an empirical treatment. In 18/36 cases (50%), a first-line anti-tuberculosis empirical treatment is proposed (quadritherapy in 11 cases). In 7 cases (19%), an empirical treatment with prednisone, 0.4 mg/kg/d (N=1) and 1 mg/kg/d (N=6), would be prescribed. Seven internists (19%) would prescribe an empirical treatment with cyclins at the dose of 100 to 400 mg/d. Median duration of the empirical treatment would be 28 days (range: 8-252d). The evaluation parameters mentionned are: fever (69%), weight (59%), seric level of C-reactive protein (59%), and liver biology (53%). In case of failure of first-line empirical treatments, 69% of all questionned internists prescribe a second-line treatments: prednisone at the dose of 0.4 to 2 mg/kg/d (72%), anti-tuberculosis treatments (16%), cyclins 200 mg/d (12%), with a median duration of 28 days. Seven internists (19%) propose to combine two empirical treatments., Discussion: Faced with a problem of idiopathic granulomatous hepatitis, French internists questionned propose four therapeutics options: no treatment, anti-tuberculosis treatment, cyclins or steroids treatment. First-line anti-tuberculosis treatment is a coherent proposition regarding to the high prevalence of tuberculosis. There are only few data available concerning empirical treatment with steroids or cyclins. Specific proposition of such empirical treatments should be defined., Conclusions: The management of idiopathic granulomatous hepatitis is difficult. Our study shows that therapeutics practices of French internists are heterogenous. The main proposition consists in a first-line anti-tuberculosis empirical treatment, that has to be evaluated after four weeks, and switched with steroids (prednisone, 1 mg/Kg/d) in case of failure. This study is not an expert proposition but contributes to suggest clinical practice guidelines for a rare, complex, heterogenous, and typically internist situation.

Study Objective: This study investigated the clinical features and disease course of sarcoidosis diagnosed in patients > 70 years of age., Methods: A retrospective analysis was made of cases treated at the University Hospital in Nantes, France, between 1986 and 2000. The diagnosis of sarcoidosis was confirmed histopathologically. Cases involving progressive cancer and active tuberculosis were excluded., Results: Thirty white patients with sarcoidosis diagnosed after age 70 years (mean, 74 years) were included. An alteration of general health (asthenia and/or anorexia and/or weight loss) was frequent (53%) and characteristic of the systemic form of the disease. Dyspnea was a fairly common sign (23%). The intrathoracic form of sarcoidosis was most frequent (43.3%). Diagnosis was difficult and lengthy, and symptomatology was atypical. Accessory salivary gland biopsy was an important contributing factor to diagnosis (70.6% were positive). Oral corticosteroid therapy was often required (60.7%). The disease course was satisfactory overall (81.8% of cases), but only for 50% of patients in intrathoracic stage IV., Conclusions: The clinical presentation of sarcoidosis in elderly subjects is mainly characterized by an alteration of general health. Diagnosis is difficult and should include accessory salivary gland biopsy. Therapy frequently involves corticosteroids. Overall prognosis is similar to that for young subjects.

Despite its increasing incidence giant cell arteritis is not well detected in the elderly. Response to corticosteroid treatment is the same before and after the age of 75, but there are many steroid-induced side effects, particularly bone fractures, in the elderly. Therefore, it is important to reduce the corticosteroid load in elderly and frail people. In this cases, 0.3 to 0.5mg/kg, or 15 to 25mg daily prednisone-equivalent dose at start seems to be enough to prevent blindness in simple forms. This dose has to be rapidly reduced whenever the C-reactive protein remains moderately elevated. Moreover, an anti-agregant or anticoagulant treatment must be associated at the beginning of steroid treatment to prevent ischemic complications, as well as biphosphonates, which could prevent corticosteroid-induced osteoporosis.

The coexistence of giant cell arteritis (GCA) and autoimmune thyroid diseases has been suggested. In our prospective study of 39 patients with GCA, we measured autoantibodies and thyroid function and compared the results to those of a control group. No statistical difference was found. Our conclusions differ from those of other authors.

Endocrine function was prospectively evaluated in 98 patients (73 men and 25 women) infected by HIV in various stages of illness: Centers for Disease Control groups II (19), III (20), IVA and IVC2 (27), IVC1 and IVD (32). Testing included baseline and post-stimulation evaluation of gonadal, thyroidal, and adrenal axes. Although adrenal function was within normal values in most cases, with no differences between patient groups, nine out of 98 patients had either a low baseline or post-stimulation serum cortisol, cytomegalovirus adrenalitis being suspected in two cases. Mineralocorticoid response was normal in all individuals. The main abnormalities were sick euthyroid syndrome with low tri-idothyronine and/or thyroxine in 16% of patients and hypotestosteronemia in 29% of men with AIDS. These abnormalities, related to a functional deficiency of the hypothalamic-pituitary axis, were highly correlated with the degree of illness, i.e. weight loss and low CD4+ cell count. It was concluded that endocrine dysfunction in HIV-infected patients is rarely of clinical significance, that it is related more to cachexia and advanced disease than to HIV or opportunistic infections, and that it could serve as a prognostic marker.

We evaluated the efficacy and toxicity of piperacillin-ofloxacin as an empiric treatment of fever in patients with neutropenia. 24 febrile episodes occurring in 21 patients (mean neutropenia: 204/mm3) were treated. The neutropenia was due to an hematologic malignancy in 6 cases and to chemotherapy in 15 cases. Fever was related to septicemia in 4 cases, urinary tract infection in 1 case, other infectious sites without microbiological documentation in 11 cases, and was of unknown origin in 8 cases. Empirical therapy was started within 24 hours of the occurrence of fever greater than 38.5 degrees C with the combination of intravenous piperacillin (12 g/day in 3 divided doses) and oral ofloxacin (400 mg/day in 2 doses). The overall response rate was 86% (19/22) of evaluable cases, with an immediate success rate (apyrexia within 48 hours) of 46%. Of the 3 failures, one was bacteriologically documented and was due to a multiply resistant strain of Staphylococcus haemolyticus (to which both piperacillin and ofloxacin were resistant). The therapy was clinically well tolerated in all except 3 patients, in whom intolerance to intravenous piperacillin was observed, leading to discontinuation of the drug in 2 cases. More extensive and comparative trials should better determine the place of this piperacillin-ofloxacin combination as first-line treatment of febrile episodes in patients with neutropenia.

In two patients "granulomatous hepatitis" was diagnosed upon examination of liver biopsy specimens taken because of prolonged fever with poor general condition. Observed lesions were small nodules without necrosis: follicular adentitis was also found in one patient. Diagnosis of Whipple disease was established by examination of a biopsy specimen of the intestinal mucosa. Study of the hepatic lesions after Gram and PAS staining, done in one patient, did not demonstrate the suggestive intrahistiocytic particles. Confrontation of clinical evidence and histologic findings in lymph node and intestinal biopsy specimens is required for diagnosis.

A case of tricuspid valve endocarditis with spinal epidural abscess caused by Actinobacillus actinomycetemcomitans is reported in a 74-year-old male with an endocardial pacemaker. Despite antibiotic treatment, removal of the endocardial wire was necessary for recovery.