Management of giant cell arteritis: Recommendations of the French Study Group for Large Vessel Vasculitis (GEFA).

PURPOSE: Management of giant cell arteritis (GCA, Horton's disease) involves many uncertainties. This work was undertaken to establish French recommendations for GCA management. METHODS: Recommendations were developed by a multidisciplinary panel of 33 physicians, members of the French Study Group for Large Vessel Vasculitis (Groupe d'étude français des artérites des gros vaisseaux [GEFA]). The topics to be addressed, selected from proposals by group members, were assigned to subgroups to summarize the available literature and draft recommendations. Following an iterative consensus-seeking process that yielded consensus recommendations, the degree of agreement among panel members was evaluated with a 5-point Likert scale. A recommendation was approved when ≥ 80% of the voters agreed or strongly agreed. RESULTS: The 15 retained topics resulted in 31 consensus recommendations focusing on GCA nomenclature and classification, the role of temporal artery biopsy and medical imaging in the diagnosis, indications and search modalities for involvement of the aorta and its branches, the glucocorticoid regimen to prescribe, treatment of complicated GCA, indications for use of immunosuppressants or targeted biologic therapies, adjunctive treatment measures, and management of relapse and recurrence. CONCLUSIONS: The recommendations, which will be updated regularly, are intended to guide and harmonize the standards of GCA management.

Imatinib mesylate in scleroderma-associated diffuse skin fibrosis: a phase II multicentre randomized double-blinded controlled trial.

BACKGROUND: Imatinib mesylate is a potent inhibitor of platelet-derived growth factor and transforming growth factor-ß signalling pathways which may play a role in systemic sclerosis (SSc)-associated skin changes. OBJECTIVES: We aimed primarily at assessing the efficacy of imatinib mesylate in scleroderma skin fibrosis. METHODS: We performed a phase II double-blinded trial on patients with scleroderma with either morphoea involving > 20% of body surface area or SSc with extensive skin involvement: modified Rodnan Skin Score (mRSS) ≥ 20/51. Each patient was randomized to receive either imatinib mesylate 400 mg or placebo daily for a total of 6 months, and then was followed up 6 months after therapy discontinuation. Skin fibrosis was assessed by mRSS and measurement of the dermal thickness using skin biopsies performed at inclusion and at 6 months of treatment. In addition, quality of life (Dermatology Life Quality Index and modified Health Assessment Questionnaire for Scleroderma) was recorded at each visit, and pulmonary function before and after intervention. RESULTS: Twenty-eight patients were included in the study with a mean age of 48·9 years (range 30-71): 25 had a diagnosis of a SSc and three of diffuse cutaneous scleroderma. Demographic data, frequency of organ involvement of SSc and mRSS were comparable between groups. At 6 months, the proportion of variation of mRSS from inclusion was not statistically significantly different between the two groups (median +0·10 in imatinib group vs. -0·16 in placebo group, P = 0·098). Similarly, changes in dermal thickness, quality of life and diffusion capacity for carbon monoxide were not significantly different between groups. CONCLUSIONS: This study failed to demonstrate the efficacy of imatinib 400 mg daily to improve skin fibrosis of diffuse scleroderma after 6 months of treatment based on validated outcome measurements.

Thrombotic events during long-term follow-up of obstetric antiphospholipid syndrome patients.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thromboses and/or pregnancy-associated morbidity. Some patients develop only obstetric complications (obstetric APS), but data on the frequency of thrombotic events during the follow-up of these patients are scarce. This study was undertaken to evaluate the rate of thrombotic events after obstetric APS diagnosis according to the 2006 revised criteria. In total, 32 obstetric APS patients were retrospectively studied, with mean follow-up of 50 ± 37 months. After delivery, aspirin was prescribed to all patients as primary thrombosis prevention. The thrombosis rate was 3.3/100 patient-years and was 4.6, 4.5 and 10/100 patient-years when we considered at least two antiphospholipid antibody positivities (among lupus anticoagulant, anticardiolipin and anti-ß2-glycoprotein-I), antinuclear antibody positivity or systemic lupus erythematosus-associated APS patients, respectively. The thrombosis rate was high after obstetric APS diagnosis, even for patients taking aspirin. Larger, prospective studies are needed to confirm this high frequency and determine the associated risk factors.

[Digital ischemia related to bromocriptin]. / Ischémie digitale sous bromocriptine.

INTRODUCTION: Bromocriptin has been associated with stroke and myocardial infarction in the postpartum period. We report on the case of a patient who developed digital ischemia while receiving this drug. EXEGESIS: Mrs D, 28 years old presented with digital ischemia occurring five days after the introduction of bromocriptin. Magnetic resonance imaging also displayed stroke in the area of the right posterior cerebellar artery. The course was favourable after discontinuation of the drug. DISCUSSION: Bromocriptin is an ergot derivative with dopaminergic agonist properties. A paradoxical vasoconstriction is rarely associated with vascular ischemic complications, including digital ischemia.

[Central retinal artery occlusion in Wegener'granulomatosis]. / Occlusion de l'artère centrale de la rétine au cours de la granulomatose de Wegener.

INTRODUCTION: Almost 50% of patients with Wegener's granulomatosis (WG) develop ocular features, leading to visual loss in 8%. However, central retinal artery occlusion (CRAO) has exceptionally been reported. EXEGESIS: We report 2 patients with CRAO and WG according to ACR classification criteria. A literature search indicates that CRAO with WG has only been reported in 15 patients. We analyse the 17 cases to discuss systemic manifestations, ocular prognosis and treatment. CONCLUSION: CRAO is a rare manifestation of WG. Nevertheless, CRAO seems to be associated with vasculitic type of WG. So, a prompt diagnosis could lead to early aggressive regimen to improve visual and general prognosis.

[Cystic lung disease associated with Sjögren's syndrome: 2 cases]. / Maladie kystique pulmonaire compliquant le syndrome de Gougerot-Sjögren: deux observations.

INTRODUCTION: Cystic lung disease is characterised on chest iconography by foci of decreased lung density with definable and thinned walls (wall thickness<4 mm) and with length's diameter superior at 1 cm. Cystic lung disease is exceptionally associated with the Sjögren's syndrome; very few cases have been described. EXEGESIS: We report two cases of cystic lung disease associated with Sjögren's syndrome, one occurring in a Lupus-Sjögren's overlapping syndrome, and another revealing primary Sjögren's syndrome. CONCLUSION: The Sjögren's syndrome should be recognised as could be associated with Cystic lung disease; and latent Sjögren's syndrome should be researched in presence of cystic lung lesions.

[18FDG PET: a new criterion for disease activity in Takayasu arteritis]. / La TEP au 18FDG au cours de l'artérite de Takayasu: un marqueur d'activité de la maladie.

INTRODUCTION: Takayasu arteritis (TA) is an inflammatory arteritis affecting large vessels, predominantly the aorta, its main branches, and the pulmonary arteries. Up to now, arteriography was considered as the "gold standard". But others exams are emerging in the management of TA: vascular ultrasound, angio-scanner, magnetic resonance imaging and 18FDG positron emission tomography (18FDG PET). Such investigations allow a study of the lumen but also of the arterial walls. However, at the time, no biological or radiological test is able to determine the activity of TA. 18FDG PET could be effective to estimate the disease activity. EXEGESIS: We report the case of a young woman for who 18FDG PET permit to assert a relapse of TA. CONCLUSION: 18FDG PET could be effective to estimate the disease activity.

Thrombotic microangiopathy in adult Still's disease.

Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti-beta2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.

[Ofuji's papuloerythroderma: two cases treated with azathioprine]. / Papulo-érythrodermie d'Ofuji.

BACKGROUND: Ofuji's papuloerythroderma is a rare disorder, characterized by a generalized pruriginous eruption, sparing the folds. It predominates in the elderly. The pathology is still unknown but associations with lymphoma have been described. Various therapeutic approaches have been tried, most often including local and general corticosteroids and PUVA. OBSERVATION: Two patients aged 71 and 84 years presented red pruriginous macular rash sparing the abdominal folds. Eosinophilia and lymphopenia were observed. Cutaneous biopsies showed dermal lymphocytic and plasmocytic infiltrates with, in one case, eosinophil and neutrophil exocytosis. Clinical, biological and morphological investigations showed no association with other diseases such as cancer or lymphoma. Azathioprine permitted clinical and biological remission in both patients but had to be interrupted because of minor side effects (infection, gastroenterologic disorders) and corticosteroids were introduced in one case. DISCUSSION: We suggest that histological aspects, such as exocytosis, may represent a link between Ofuji's papuloerythroderma and lymphoma. Azathioprine led to clinical and biological improvement in our 2 patients. Because of its adverse effects, it could be proposed as second-line therapy in patients presenting resistance or intolerance to usual treatments.

[Rheumatoid purpura in adults and parvovirus B19 infection: fortuitous association or parvovirus B19-induced vasculitis?]. / Purpura rhumatoïde de l'adulte et infection à parvovirus B19: association fortuite ou vascularite induite par le parvovirus B19?

INTRODUCTION: Henoch-Schoenlein purpura has been reported to be associated with parvovirus B19 infection, particularly in children and rarely in adults. We report the case of a 42-year-old patient presenting with this association. EXEGESIS: A 42-year-old patient was admitted to our medical center because of lower limb purpura. Henoch-Schoenlein purpura diagnosis was confirmed on histological findings (kidney biopsy) and concomitantly parvovirus B19 infection was proved by serological test (IgM+). Association of Henoch-Schoenlein purpura and parvovirus B19 infection has already been described. However, none of the reported studies demonstrated clearly the link between these two diseases. With regard to this observation, we wonder about the systematic use of the parvovirus B19 serological test in patients presenting first Henoch-Schoenlein purpura. Indeed, parvovirus B19-induced vasculitis is habitually controlled with intravenous immunoglobulins. CONCLUSION: A prospective study should explore the link between Henoch-Schoenlein purpura and primary parvovirus B19 infection. Moreover, we should evaluate intravenous immunoglobulins' efficacy in Henoch-Schoenlein purpura associated with active parvovirus B19 infection in order to improve the prognosis of this disease.