Giant cell arteritis-related aortitis with positive or negative temporal artery biopsy: a French multicentre study.

Objective: To compare the clinical presentation and outcome of giant cell arteritis (GCA)-related aortitis according to the results of temporal artery biopsy (TAB).Method: Patients with GCA-related aortitis diagnosed between 2000 and 2017, who underwent TAB, were retrospectively included from a French multicentre database. They all met at least three American College of Rheumatology criteria for the diagnosis of GCA. Aortitis was defined by aortic wall thickening > 2 mm on computed tomography scan and/or an aortic aneurysm, associated with an inflammatory syndrome. Patients were divided into two groups [positive and negative TAB (TAB+, TAB-)], which were compared regarding aortic imaging characteristics and aortic events, at aortitis diagnosis and during follow-up.Results: We included 56 patients with TAB+ (70%) and 24 with TAB- (30%). At aortitis diagnosis, patients with TAB- were significantly younger than those with TAB+ (67.7 ± 9 vs 72.3 ± 7 years, p = 0.022). Initial clinical signs of GCA, inflammatory parameters, and glucocorticoid therapy were similar in both groups. Coronary artery disease and/or lower limb peripheral arterial disease was more frequent in TAB- patients (25% vs 5.3%, p = 0.018). Aortic wall thickness and type of aortic involvement were not significantly different between groups. Diffuse arterial involvement from the aortic arch was more frequent in TAB- patients (29.1 vs 8.9%, p = 0.03). There were no differences between the groups regarding overall, aneurism-free, relapse-free, and aortic event-free survival.Conclusion: Among patients with GCA-related aortitis, those with TAB- are characterized by younger age and increased frequency of diffuse arterial involvement from the aortic arch compared to those with TAB+, without significant differences in terms of prognosis.

A case of Q fever with erythema nodosum.

BACKGROUND: Acute Q fever is asymptomatic in 60% of the patients, while the reminder may present with fever, pneumoniae, and hepatitis. Skin manifestations are uncommon including transient punctiform rashes, purpuric, or maculopapular eruptions. Erythema nodosum have seldom been reported. CASE PRESENTATION: A 37-year-old female presented with fever for 1 month and skin lesions consists of erythematous painful nodule of the legs. Serological testing for Coxiella burnetii was positive. Treatment consisted with doxycycline for 2 weeks. Evolution was favorable. The patient completely recovered and had no evidence of skin lesion 1 month later. CONCLUSION: Because of its nonspecific clinical presentation, Q fever with erythema nodosum is probably underestimated. Q fever should be evocated when facing unexplained erythema nodosum even if there is not other typical clinical manifestation of Q fever.

[Inflammatory myopathy following acute meningoccemia in a properdin-deficient patient: A case report]. / Myopathie inflammatoire post-méningococcémie aiguë chez un patient présentant un déficit en properdine : à propos d'un cas.

INTRODUCTION: Myalgia is a classical sign in invasive meningococcal diseases (IMD), but severe and persistent myalgia following an IMD have never been reported to date. CASE REPORT: A 20-year-old man presented with purpura fulminans and meningitis caused by Neisseria meningitidis serogroup Y, revealing properdin deficiency. Although meningitis symptoms improved after antibiotherapy, initial myalgia of the lower limbs increased, associated with mild rhabdomyolysis. Magnetic resonance imaging (MRI) revealed an increased STIR (Short TI inversion recovery) signal of both quadriceps muscles, without abscess. After exclusion of other causes of myopathy, a post-infectious myositis was diagnosed. A four-week course of corticosteroids led to dramatic improvement. CONCLUSION: Post-infectious inflammatory myopathy should be suspected in case of severe and persistent myalgia associated with rhabdomyolysis following an IMD, after exclusion of pyomyositis especially. A short course of corticosteroids seems to be effective.

[Surgical treatment of an auto-immune hemolytic anemia]. / Traitement chirurgical d'une anémie hémolytique auto-immune.

INTRODUCTION: Auto-immune hemolytic anemia (AIHA) is a rare cause of anemia, characterized by autoantibodies directed against self red blood cells. It can be primary or secondary, in particular due to lymphoproliferative diseases. CASE REPORT: We report the case of a 24-year-old woman who presented with a severe macrocytic anemia associated with an ovarian teratoma. CONCLUSION: Ovarian teratoma is a rare cause of secondary AIHA, with only few cases reported. Its treatment differs from primary AIHA as steroids may be ineffective. Indeed, complete response can only be achieved with surgical excision of the tumor.

[Association of systemic diseases and myelodysplastic syndromes. A retrospective study of 14 cases]. / Association maladies systémiques et syndromes myélodysplasiques. Etude rétrospective portant sur 14 observations.

CONTEXT: The association of a systemic disease (SD) and a myelodysplastic syndrome (MDS) may not be a coincidence. We report 14 cases. METHODS: A retrospective study was conducted in patients presenting with an MDS, hospitalised between 1989 and 1999, in the search for a concomitant systemic disease. RESULTS: Ninety-seven patients, 61 men and 36 women, with a mean age of 74 +/- 11 years suffered from an MDS and 14 of them a concomitant SD: one nodular periateritis, 2 systemic vascularitis, 2 cutaneous vascularitis, 2 atrophic polychondritis, 4 Gougerot-Sjogrën syndrome, 2 systemic lupus and one cutaneous lupus. The systemic disease did not appear to influence survival. CONCLUSION: It is possible that the association is not a coincidence and therefore an MDS should be searched for when confronted with an SD, so that treatment may be adapted appropriately.

[Imported malaria: prevention should strengthened]. / Paludisme d'importation: il faut renforcer le message de prévention.

INTRODUCTION: The risk of acquiring malaria infection can largely be prevented by the regular use of chemoprophylactic drugs combined with protective measures against mosquito bites. In a retrospective study we had for aim to evaluate the compliance to malaria chemoprophylaxis in patients presenting with malaria infection. METHODS: We analyzed the compliance to the recommended malaria chemoprophylaxis of French travelers hospitalized in a department of infectious diseases because of malaria infection, between January 1999 and December 2003. RESULTS: Eighty-five patients, with a mean age of 34.1 years (16-65) were treated for malaria infection. Seventy-seven were due to Plasmodium falciparum. The outcome was favorable for all patients, despite four severe accesses. Forty-six patients (54%) did not take any chemoprophylaxis (CP), 19 (22%) had an inadequate CP for the risk, 13 (15%) badly complied with intermittent intake of CP and seven (8%) complied well with the recommended malaria CP. Among the 85 patients, 27 (32%) had come to the travelers' consultation and been given recommendations and a recommended malaria CP prescription before traveling. CONCLUSION: These results confirm that the majority of imported malaria cases is a consequence of bad compliance to CP. Understanding user profiles and factors predicting non-compliance may help us to improve pretravel counseling, thereby reducing the risk for travelers to acquire malaria infection.

[Adherence to guidelines for the treatment of venous thromboembolism in cancer patients: a retrospective analysis of 145 cases]. / Application des recommandations dans le traitement de la maladie thromboembolique veineuse chez les patients atteints de cancer: étude rétrospective sur 145 cas.

OBJECTIVES: To assess adherence to French guidelines for curative treatment of thromboembolism in cancer patients, and to identify factors limiting their implementation. PATIENTS AND METHODS: Retrospective analysis of the medical files of cancer patients diagnosed with deep vein thrombosis (DVT) and/or pulmonary embolism (PE) in one site between January 1st, 2010 and June 30th, 2011. Central venous catheter thrombosis and superficial vein thrombosis were excluded. RESULTS: The series included 145 patients, among whom 113 (78%) had solid tumors (at a metastatic stage in 68% of cases) and 33 (22%) had hematologic malignancies. Low molecular weight heparin (LMWH) was prescribed as long-term treatment (>10 days) for 83 patients (57.2%) and a vitamin K antagonist (VKA) for 33 patients (22.7%). Bleeding required treatment modifications or discontinuation in 11 (7.5%) and 10 (6.8%) patients respectively. After 6 months, LMWH, VKA and fondaparinux were prescribed for 28, 27 and six (19.3%, 18.6% et 4.1%) patients respectively. Mean duration of anticoagulation was 176.8 days. Treatment was not affected by a history of venous thromboembolism, the presence of pulmonary embolism or proximal deep vein thrombosis but it was significantly shorter in case of thrombosis limited to muscular veins (115.5 vs 182.3 days, P<0.05). Overall, guidelines were fully implemented in only 68 (46.9%) patients, with regards to the choice of pharmacological class and duration of treatment. CONCLUSION: Adherence to national guidelines is insufficient and actions must be taken to improve the management of venous thromboembolism in cancer patients.

Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome.

BACKGROUND: The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). METHODS: The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. RESULTS: Anti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO<45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). CONCLUSION: Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO<45% at ILD diagnosis and UIP pattern on HRCT-scan.

Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome.

The aims of the present study were to: compare the characteristics between antisynthetase syndrome (ASS) patients with anti-Jo1 antibody and those with anti-PL7/PL12 antibody. The medical records of 95 consecutive patients with ASS were reviewed. Seventy-five of these patients had anti-Jo1 antibody; the other patients had anti-PL7 (n=15) or anti-PL12 (n=5) antibody. At ASS diagnosis, the prevalence of myalgia (p=0.007) and muscle weakness (p=0.02) was significantly lower in the group of anti-PL7/PL12-positive patients than in those with anti-Jo1 antibody; median value of CK (p=0.00003) was also lower in anti-PL7/PL12 patients. Anti-Jo1 positive patients developed more rarely myositis resolution (21.3% vs. 46.2%); in addition, the overall recurrence rate of myositis was higher in anti-Jo1 positive patients than in patients with anti-PL7/PL12 antibody (65.9% vs. 19.4%). Anti-Jo1-positive patients, compared with those with anti-PL7/PL12 antibody, more often experienced: joint involvement (63.3%vs. 40%) and cancer (13.3% vs. 5%). By contrast, anti-PL7/PL12 positive patients, compared with those with anti-Jo1 antibody, more commonly exhibited: ILD (90% vs. 68%); in anti-PL7/PL12 positive patients, ILD was more often symptomatic at diagnosis, and led more rarely to resolution of lung manifestations (5.6% vs. 29.4%). Finally, the group of anti-PL7/PL12 positive patients more commonly experienced gastrointestinal manifestations related to ASS (p=0.02). Taken together, although anti-Jo1 positive patients with ASS share some features with those with anti-PL7/PL12 antibody, they exhibit many differences regarding clinical phenotype and long-term outcome. Our study underscores that the presence of anti-Jo1 antibody results in more severe myositis, joint impairment and increased risk of cancer. On the other hand, the presence of anti-PL7/PL12 antibody is markedly associated with: early and severe ILD, and gastrointestinal complications. Thus, our study interestingly indicates that the finding for anti-Jo1 and anti-PL7/PL12 antibodies impacts both the long-term outcome and prognosis of patients with ASS.

[An erysipelas revealing infection by Dermatobia hominis]. / Un érysipèle révélant une infection àDermatobia hominis.

Myiases are parasitic infections by larvae of flies. The development of intercontinental travels increases the incidence of tropical myiasis in travellers. We report the case of a patient, having recently stayed in Peru, presenting with an inflammatory plate of the right shoulder, covered with small papules with a hole inside. The initial aspect seemed like an erysipelas. Considering the resistance to the antibiotic treatment, the diagnosis of myiase was suspected. The local application of petroleum jelly allowed the exit of nine larvae of Dermatobia hominis and a fast good outcome. In human beings, the number of larvae usually infecting the same individual varies from one to four. This observation is original because of the number of implied larvae, which explains the intensity and the extent of the local inflammatory signs, which first looked like erysipelas. This diagnosis must be suspected in cases of erysipelas resistant to antibiotics in patients back from an endemic area.