J. Magnant

18 FDG PET/CT contribution in occult giant-cell arteritis.

A72-year-old woman was admitted for asthenia and weight loss within the last 3 months, along with a rise in C-reactive protein and fibrinogen levels, at 90 mg/L and 8.34 g/L, respectively. Computed tomographic (CT) scan showed a circumferential thickening of the thoracic and abdominal aorta (Fig. 1). F-fluoro-desoxyglucose (FDG) positron emission tomography (PET)/CT showed an increased metabolic activity (maximum standardized uptake value = 2.9 g/mL; normal, 1.5Y2.0 mg/L) of the entire aorta (Fig. 2). A temporal artery biopsy confirmed the diagnosis of giant cell arteritis (GCA; Fig. 3) despite the fact that the patient’s temporal arteries were clinically normal, and she did not present any headache or other features of GCA. A good general condition was restored, and blood inflammatory markers were normalized after prednisone 0.7 mg/kg per day. Rarely, GCA presents as an isolated aortitis with fever, asthenia, or an unexplained inflammatory response. The American College of Rheumatology criteria for GCA do not take into account large artery involvement. At this stage, imaging may inform about disease activity and extent but also lead to diag-

Périartérite noueuse cutanée et maladie de Crohn : une association à ne pas méconnaître

INTRODUCTION Cutaneous polyarteritis nodosa (CPAN) is an entity which needs to be acknowledged, since it can have a spontaneously adverse outcome. We report two cases of CPAN associated with Crohns disease. CASE REPORTS The first patient was suffering from Crohns disease for 9 years when she was referred for a necrotic toe. A diagnosis of necrotizing angeitis was confirmed by histological examination of a skin biopsy. Despite systemic corticosteroids, the lesions became more severe, requiring immunosuppressive treatment. The second patient was a female patient referred with forefoot ischemia. Cutaneous histology confirmed the diagnosis of necrotizing angeitis that responded favourably to corticosteroid treatment. The patient had been diagnosed with Crohns disease 2 months previously. CONCLUSION CPAN differed from systemic PAN by the absence of visceral involvement. Its association with Crohns disease, although uncommon, must be recognized as it affects treatment and monitoring.

Late onset malnutrition from esophageal and phrenic dysfunction after radiotherapy for Hodgkin's lymphoma: A case report

Radiotherapy may lead to late-onset, rare, but sometimes life-threatening complications that need to be recognized for timely management. We report the case of a 39-year-old man who presented with a 20-kg weight loss with severe dysphagia and respiratory failure. His medical history was noticeable for Hodgkins lymphoma that was treated 20 y previously. The physical examination and electroneuromyography indicated vagal and phrenic neuropathies. We concluded that the patient had late-onset esophageal motor disorder and bilateral phrenic paralysis secondary to the radiotherapy received 20 y previously for the lymphoma. The patients management included long-term nutritional support. Although late-onset vagal and phrenic nerve injuries have been described separately after radiotherapy, we report the first case of paralysis at both sites. Another striking feature of this observation is the subsequent severe malnutrition that accompanied these paralyses.

Amyotrophie diabétique : intérêt de la corticothérapie ?

Diabetic amyotrophy or lombosacral radiculoplexus neuropathy is a rare complication associated with early-stage diabetes. Thigh pain, quadricipital amyotrophy, proximal weakness of lower limbs and weight loss are the main symptoms of the disease. As neurological damage is related to inflammatory microvasculitis, corticosteroid therapy may be considered as the first line therapy. We report a 54-year-old patient with type 2 diabetes affected with severe diabetic amyotrophy. Following intravenous corticosteroid therapy, the patient reported a rapid pain relief and gained muscle strength.Many neurologic and psychiatric manifestations have been associated with systemic lupus erythematosus. Narcolepsy, currently hypothesized as related to an autoimmune process, has been rarely associated with systemic lupus erythematosus. We report a 36-year-old woman who presented with narcolepsy and who subsequently developed systemic lupus erythematosus. Excessive daytime sleepiness resolved after the administration of four intravenous bolus of cyclophosphamide and methylprednisolone followed by maintenance therapy with hydroxychloroquine, aspirine and prednisone. Narcolepsy should be included in the neuropsychiatric manifestations of systemic lupus erythematosus and it may have a parallel clinical course to the activity of the lupus.

What is the best salivary gland ultrasonography scoring methods for the diagnosis of primary or secondary Sjögren's syndromes?

OBJECTIVE To evaluate the performance of salivary gland ultrasonography for the diagnosis of primary and secondary Sjögrens syndromes (pSS and sSS). METHOD Multicenter cross-sectional study on 97 patients with clinical sicca symptoms. The pSS (n = 22) met the American-European Consensus Group (AECG) classification criteria. The control patients (n = 36) with sicca symptoms did not fulfill the AECG criteria. Four scores were used to evaluate the 4 major salivary gland echostructure: the Salaffi score (0-16), Jousse-Joulin score (0-4), Hocevar score (0-48) and Milic score (0-12). RESULTS The medians of ultrasonographic (US) scores were higher in the pSS and sSS groups than in the control group (P < 0.001). The receiver-operating characteristic (ROC) curves and the positive likelihood ratio (LR+) of the four scores showed a good diagnostic performance for the US diagnosis of pSS and sSS. Respectively, for pSS and sSS, the AUC were 0.891 (95%CI 0.812-0.970) and 0.824 (95%CI 0.695-0.954) for Hocevar score, 0.885 (95%CI 0.804-0.965) and 0.808 (95%CI 0.673-0.943) for Milic score, 0.915 (95%CI 0.848-0.982) and 0.844 (95%CI 0.724-0.965) for Salaffi score, 0.897 (95%CI 0.821-0.973) and 0.851 (95%CI 0.735-0.968) for Jousse-Joulin score. This study showed an interesting inter-observer reproducibility (kappa = 0.714 ± 0.131) of the US evaluation with 85.7% agreement between reader to determine the pathological character of the salivary glands. CONCLUSION Salivary gland US is a simple, non-invasive and performant imaging procedure for the diagnosis of pSS and sSS, with Salaffi, Milic and Jousse-Joulin scores.