C. Toledano

Localized scleroderma: a series of 52 patients.

BACKGROUND Localized scleroderma also called morphea is a skin disorder of undetermined cause. The widely recognized Mayo Clinic Classification identifies 5 main morphea types: plaque, generalized, bullous, linear and deep. Whether each of these distinct types has a particular clinical course or is associated with some patient-related features is still unclear. METHODS We report here a retrospective series of patients with localized scleroderma with an attempt to identify features related to the type of lesion involved. The medical records of all patients with a diagnosis of localized scleroderma were reviewed by skilled practitioners. Lesions were classified according to the Mayo Clinic Classification. The relationship between each lesion type and various clinical features was tested by non-parametrical methods. RESULTS The sample of 52 patients included 43 females and 9 males. Median age at onset was 30 y (range 1-76). Frequencies of patients according to morphea types were: plaque morphea 41 (78.8%) (including morphea en plaque 30 (57.7%) and atrophoderma of Pasini-Pierini 11 (21.1%)), linear scleroderma 14 (26.9%). Nine patients (17.3%) had both types of localized scleroderma. Median age at onset was lower in patients with linear scleroderma (8 y (range 3-44)) than in others (36 y (range 1-77)) (p=0.0003). Head involvement was more common in patients with linear scleroderma (37.5%) than in other subtypes (11.1%) (p=0.05). Atrophoderma of Pasini-Pierini was never located at the head. Systemic symptoms, antinuclear antibodies and the rheumatic factor were not associated with localized scleroderma types or subtypes. CONCLUSION These results suggest that morphea types, in adults are not associated with distinct patient features except for age at disease onset (lower) and the localization on the head (more frequent), in patients with lesions of the linear type.

Aldolase predicts subsequent myopathy occurrence in systemic sclerosis

IntroductionMyopathy related to systemic sclerosis (Myo-SSc) is a disabling and unpredictable complication of SSc. We assessed the predictive value of serum aldolase, creatine kinase (CK), alanine transaminase (ALT), aspartate transaminase (AST) and C-reactive protein (CRP) to estimate the risk of developing Myo-SSc.MethodsWe enrolled 137 SSc patients without proximal muscle weakness in a prospective monocentric study to follow them longitudinally over a four-year period. The risk of occurrence of Myo-SSc was ascertained according to the European NeuroMuscular Centre criteria and was analyzed according to levels of plasma aldolase, CK, transaminase enzymes and CRP at inclusion. Performance of each parameter to predict Myo-SSc occurrence was assessed and compared with the others.ResultsThe area under the receiver operating characteristic curves (ROC) of plasma aldolase for Myo-SSc occurrence prediction was 0.80 (95% CI: 0.67 to 0.94, P < 0.001), which was higher than that of plasma CK (0.75, P = 0.01), and that of ALT (0.63, P = 0.04). AST and CRP had no predictive value for Myo-SSc occurrence. The best cut-off of aldolase for prediction of Myo-SSc occurrence within three years after inclusion was 9 U/L and higher than the upper normality limit (7 U/L), unlike that of CK and ALT. Myo-SSc occurred more frequently in patients whose plasma aldolase was higher than 9 U/L. Adjusted Hazard Ratio for patients with aldolase > 9 U/L was 10.3 (95% CI: 2.3 to 45.5), P < 0.001.ConclusionsIncreased plasma aldolase level accurately identified SSc patients with high risk to develop subsequent Myo-SSc. This could help initiate appropriate treatment when the disabling muscle damage is still in a reversible stage.

Digital photography as an operational tool for assessing corticosteroid-induced lipodystrophy

BACKGROUND Corticosteroid-induced lipodystrophy (CIL) is exclusively diagnosed in a subjective manner. OBJECTIVE To evaluate the reliability of digital photographs in the diagnosis of CIL. METHODS All consecutive patients starting long-term, high dosage corticosteroid therapy were photographed at baseline and after 3 months of therapy. At the end of the study, 3 physicians with expertise in corticosteroids classified patients as lipodystrophic yes/no/unclassifiable. Photographs analyses performed by 9 medical readers and evaluation of CIL using visual analog scale (VAS) performed during the M3 visit were compared to this classification. RESULTS Eighty-eight patients were monitored. Fifty of them were classified by the 3 experts as lipodystrophic and 30 as not lipodystrophic (8 were unclassifiable). Their intra- and inter-observer agreements were moderate or fair (kappa coefficient<or=0.57) when month 3 photographs were analysed alone and substantial or near perfect (kappa coefficient>or=0.75) when M3 photographs were analysed beside baseline ones. By comparison with expert consensus, only 3 out of 4 patients were correctly classified using VAS. The AUROC curve and inter-observer agreement significantly improved with experience for the 9 non-experts. CONCLUSION The use of digital photographs do better than VAS to evaluate CIL. The accuracy of diagnosis improves with experience. Morphological changes are more important than morphological phenotype.

Aortite inflammatoire au cours de la maladie de Horton

A sub-clinical inflammatory aortitis is very frequent in patients with giant cell arteritis, and can be the only localization of the disease. In most patients, this aortitis is asymptomatic and is of no consequence on the patients survival. The relative risk of developing an aortic dissection or aneurysm is 17.3. Evolution towards an aneurysm or an aortic dissection is unpredictable and rare; and seems independent of the disease activity and the associated vascular risk factors. Isolated aortitis treatment is not consensual, but often similar to the treatment of giant cell arteritis and adapted to clinical and biological markers of disease activity. Screening for sub-clinical aortitis with FDG-PET should not be prescribed in patients with typical presentation of giant cell arteritis. A systematic screening of aortic complications in giant cell arteritis patients could be done with a chest X-ray and an abdominal ultrasound possibly completed with an aortic CT-scan at time of diagnosis, in order to look for aneurysms with possible surgical indication.

Fièvre prolongée associée à une éruption cutanée diffuse révélant une méningococcémie chronique

INTRODUCTION Chronic meningococcemia is an unusual clinical presentation within the spectrum of infections due to Neisseria meningitidis. CASE REPORT We report a 32-year-old man who presented with a 15-day history of fever and maculopapular skin rash, in the absence of meningeal irritation or severe sepsis manifestation. Blood culture identified N. meningitidis. Clinical course was uneventful after antibiotic treatment was initiated. CONCLUSION Early diagnosis of chronic meningococcemia is crucial for optimal management of the patient and his/her contacts. Such a diagnosis should be suspected in the presence of the characteristic clinical triad (recurrent fever, skin rash and arthralgia), and this clinical presentation should be distinguished from systemic vasculitis as inadequate prescription of corticosteroids may be deleterious.

Augmentation de CXCL10 dans le sérum au cours de la pneumopathie interstitielle de la sclérodermie systémique

INTRODUCTION CXCL10, a gamma-interferon-induced chemokine seems to play a relevant role in lung involvement that occurs in systemic sclerosis (SSc). The objective of this study was to assess the serum level of CXCL10 in interstitial lung disease (ILD) associated with SSc. METHODS Serum level of CXCL10 was assayed in 23 healthy volunteers (60.0 years; 58.0-67.3) and 29 SSc patients (63.1 years; 60.1-69.4) by ELISA method. Pulmonary function tests (PFTs), lung CT-scan and echocardiogram were also performed in the patients. Serum levels from patients and healthy controls were compared and a comparison among SSc patients between those with and without ILD, as documented by lung CT-scan, was also performed. RESULTS Median CXCL10 level from patients with SSc was significantly higher than that from healthy volunteers (110.0 pg/ml; 60.8-223.8 versus 52.0; 41.3-65.8; p<0.001). Fifteen out of the 29 patients had ILD on lung CT-scan; the median CXCL10 level from SSc patients with ILD was significantly higher than that from SSc patients without ILD (210.0 pg/ml; 115.0-307.5 versus 76.0; 55.0-110.0; p=0.02). CONCLUSION Our findings suggest that CXCL10 is specifically increased in the lung involvement of SSc and plays a role in scleroderma lung disease.

Tuberculosis in patients with and without primary health coverage

BACKGROUND: The aim of this study was to determine whether tuberculosis (TB) treatment and follow-up is a feasible option for patients without primary health coverage when free access to health care and drug delivery is provided on an outpatient basis. METHODS: We conducted a retrospective analysis of patient characteristics, treatment modalities, and evolution up to 9 months of follow-up at Saint-Louis Hospital, Paris, France. RESULTS: TB patients without (n=44) compared to those with (n=49) primary health coverage at diagnosis tended to be younger and more frequently homeless (P<0.05) or had illegal status (P<0.001). They had similar TB presentation when starting treatment, but required free drug delivery more often (74 vs. 21%, P<0.001). After the same duration of follow-up, patients considered as cured or as having completed treatment (52.3 vs. 51%), dead (2.3 vs. 8.2%), still under treatment (13.2 vs. 6.1%), or lost to follow-up (31.8 vs. 34.7%) were similar in both groups. At the end of the follow-up period, 43% of the patients without primary health coverage had managed to obtain 70% or 100% free medical aid or social security. CONCLUSIONS: Free access to health care and drug delivery is worthwhile in TB patients without primary health coverage since it permits the same clinical results as in TB patients with primary health coverage. However, efforts should be made to improve the management and follow-up of TB patients and to lower the number of patients lost to follow-up, whatever their social status.

Des anomalies unguéales

Une femme de 38 ans consultait en avril 2008 dans le cadre u suivi annuel d’un phénomène de Raynaud évoluant depuis la n de l’adolescence. En avril 2000, elle avait eu un rash malaire on infiltré, une acrocyanose des extrémités ainsi qu’un livedo ctif des poignets et des genoux. Au cours de l’été 2000, elle était articulièrement gênée par son acrosyndrome et était traitée par ifédipine 30 mg/jour remplacé par du losartan 50 mg/jour en aison d’une mauvaise tolérance. À l’automne 2001, elle avait eu n panaris ainsi qu’une recrudescence du phénomène de Raynaud onduisant à la reprise du traitement par nifédipine 30 mg/jour. u cours de l’hiver 2004, elle eut un gonflement permanent et ndolore des doigts avec fissures. On constatait alors la présence e télangiectasies du nez. L’hypocomplémentémie, connue depuis lusieurs années, persistait sur la fraction C3 (0,66 g/l, N ≥ 0,9), la raction C4 étant à 0,13 g/l (N ≥ 0,10). En avril 2008, alors que le hénomène de Raynaud persistait depuis l’hiver, l’examen trouvait es anomalies des ongles des troisième, quatrième et cinquième oigts de la main droite (Fig. 1) et du cinquième doigt de la main auche. Selon la patiente, il s’agissait du premier épisode de ce type t les lésions étaient apparues au cours de l’hiver précédent. La apillaroscopie révélait quelques dystrophies mineures en caducée t tortueuses ainsi que de rares hémorragies. Il n’y avait aucun lément en faveur d’une microangiopathie organisée spécifique ’une sclérodermie, d’une polymyosite ou d’une maladie de Sharp.

Une thrombose veineuse profonde

A deep vein thrombosis (DVT) is a blood clot that forms in the deep veins of the leg. A deep vein thrombosis in the thigh carries a risk of pulmonary embolism (PE). This occurs when the clot loses its attachment to the inside of the vein, leaves the leg and lodges in the pulmonary artery, the main blood vessel to the lungs. If the clot is large enough, it can completely block that artery and cause death.

Des poumons asymétriques

Une femme de 84 ans consultait pour dyspnée avec orthopnée t œdèmes des membres inférieurs, évoluant depuis un mois. lle avait pour principaux antécédents une insuffisance rénale hronique, une polyarthrite rhumatoïde, une néphrostomie pour ithiase urinaire, et une hypokinésie de la paroi inférieure du yocarde, à fonction ventriculaire gauche conservée. Elle n’avait amais fumé et ne rapportait aucune exposition à des toxiques rofessionnels, dans les emplois successifs de couturière puis de onctionnaire à l’Éducation nationale. La dyspnée s’était installée rogressivement depuis un mois, initialement à l’effort, se majoant à la toux, à l’inspiration profonde et aux changements de osition, évoluant vers une orthopnée, avec apparition d’œdèmes es membres inférieurs dans les dernières semaines. Il n’était apporté ni toux, ni douleur thoracique, ni syndrome fébrile, ni ltération de l’état général. Elle était tachycarde et polypnéique, a saturation en oxygène en air ambiant était de 95 %, la pression rtérielle à 150/73 mmHg. Le murmure vésiculaire était bilatéral t asymétrique, diminué à gauche, sans bruit surajouté. On notait es œdèmes des membres inférieurs bilatéraux, volumineux, renant le godet jusqu’à mi-mollets et suintants. La radiographie e thorax de face, au lit, montrait une opacité hilaire droite, et une