[Adult onset Still's disease and lymphoma: a rare association]. / Maladie de Still de l'adulte et lymphome: une association rare.

Adult onset Still's disease (AOSD) and lymphomas are diseases characterized by very similar clinical and histopathological manifestations. The association between these diseases has rarely been reported in the literature. We here report the case of a 26-year old patient diagnosed with adult onset Still's disease. She had been previously treated for large B-cell Non-Hodgkin lymphoma (LBCNHL) with chemotherapy and haemopoietic stem-cell autograft with complete remission. The association between AOSD and lymphoma is rare and in all cases AOSD has been diagnosed before lymphoma. The peculiarity of this study lies in the succession LBCNHL- AOSD and raises several hypotheses on the association between these two diseases. The association between non-autoimmune diseases and lymphoid hemopathies have been widely demonstrated, whether it is the progression of autoimmune diseases vs lymphoma or non-autoimmune disease manifestations occurring in patients with this disease. This study highlights the difficult distinction between these diseases.

[Ruptured renal artery aneurysms in microscopic polyangiitis]. / Rupture d'anévrismes des artères rénales dans la polyangéite microscopique.

INTRODUCTION: Renal artery aneurysms are unusual manifestations of microscopic polyangiitis. OBSERVATION: We report the case of a 55 years old male patient, with a microscopic polyangiitis, which was revealed by a demyelinating polyradiculoneuropathy, the diagnosis was made with association of alveolar hemorrhage, peripheral neuropathy and glomerulonephritis with positive antineutrophil cytoplasmic antibodies (antimyeloperoxidase antibodies). The evolution was marked by a state of shock by rupture of aneurysms of intrarenal arteries. CONCLUSION: This case emphasizes the importance of looking for complications of unusual type of intrarenal aneurysms in patients with microscopic polyngiitis.

[Behcet syndrome: thirty comments with lung and vascular injury of peripheral vessels]. / Maladie de Behçet: trente observations avec atteintes vasculaires pulmonaires et des vaisseaux péripheriques.

INTRODUCTION: Behcet's disease is a systematic vasculitis of unknown cause, characterized essentially by eye, cutaneous, articular, neurological and vascular manifestations. METHODS: We retrospectively analysed the Behcet's disease cases that were followed up in our ward from January 2000 to January 2009. The inclusion criteria were those of International Study Group on Behçet's disease (aphthosis mouth was required). Data were retrieved and analysed with two softwares (Access(®) and Epi Info(®)). RESULTS: We observed 30 cases with vascular lesions on a series of 92 patients with Behcet's disease. Most patients were male, with an average age around 40. The venous manifestations, concerning essentially the lower limbs (deep and superficial thrombosis) were found at 27 patients (90 %), and the average of age during the appearance of the venous lesions was 40 years. Arterial lesions appear more late in 13 patients (43 %) (average of age 43 years). We noted, on the other hand, 11 cases of aneurysms and five cases of arterial thrombosis. The use of corticosteroids was necessary in all cases in association with the others drugs (anticoagulants, colchicine, immunosuppressors). Among the patients having had aneurysms, six were treated surgically. The outcome was favorable for most patients. Two patients had pulmonary embolism and two post-surgery complications. One patient died in the consequences of an intragastric break of an aneurysm of the abdominal aorta. CONCLUSION: The vascular involvement in Behcet's disease is manifested primarily by thrombophlebitis. Achieving blood pressure, less common, is problematic therapeutic because of the recurrent and life threatening.

[Antagonists of interleukin-6 (tocilizumab), in adult refractory still disease]. / Les antagonistes de l'interleukine-6 (tocilizumab) dans la maladie de still réfractaire de l'adulte.

INTRODUCTION: The pathogenesis of Still's disease is best elucidated for the better recognition of the involvement of Many pro-inflammatory cytokines in the genesis of this condition. Publications have reported the contribution beneficial for certain biotherapeutics, such as anti-TNFa, the anti-CD20 or antagonists of interleukine1 (IL-1) tested successfully in the treatment of systemic Juvenile idiopathic arthritis (Still's disease the child), the tocilizumab is a humanized monoclonal antibody directed against the receptor for interleukin-6 and is beginning to be reported as effective in some refractory cases of Still's disease in adults. PATIENTS: We report two young patients with Still's disease in adults with refractory early and prolonged remission after the first infusion tocilizumab. CONCLUSION: The tocilizumab can be used in patients MSA with refractory after failure or intolerance conventional treatments.

Lung findings on thoracic high-resolution computed tomography in patients with ankylosing spondylitis. Correlations with disease duration, clinical findings and pulmonary function testing.

The aim of this study was to identify the spectrum of abnormalities revealed on high-resolution computerized tomography (HRCT) in patients with ankylosing spondylitis (AS), to compare findings with those of plain radiography and pulmonary function testing (PFT), and to look for correlations between lung involvement and AS severity. We prospectively studied 55 consecutive patients with a diagnosis of AS according to the modified New York criteria who attended our department over a period of 2 years. All patients had a detailed rheumatological examination and underwent plain chest radiography, chest HRCT and PFT. HRCT revealed abnormalities in 29 patients (52.7%), whereas plain chest radiography was abnormal in only 2. Abnormalities consisted of interstitial lung disease (ILD) ( n=4), apical fibrosis ( n=5), emphysema ( n=5), bronchiectasis ( n=4), ground glass attenuation ( n=2), and non-specific interstitial abnormalities ( n=26). Only apical fibrosis and bronchiectasis were statistically more frequent with increasing disease duration (significant trend chi(2)test, p=0.0029 and 0.028, respectively). PFT showed a restrictive process in 19 patients (34.5%). No correlation was noted between HRCT and PFT, nor with AS symptomatic and structural severity parameters. However, there was a statistically significant correlation between PFT and AS symptomatic and structural severity parameters. In conclusion,: this study confirms that the chest HRCT of patients with AS showed a great number of abnormalities undetectable by standard X-rays. The high incidence of lung abnormalities emphasizes the importance of excluding such a diagnosis in patients with AS even without respiratory symptoms.

[Gastrointestinal bleeding revealing polycythemia. A case report]. / Hémorragie digestive révélatrice d'une polyglobulie. A propos d'une observation.

PURPOSE: Gastrointestinal bleeding, an uncommon complication of polycythemia can be the inaugural sign in exceptional cases. CASE REPORT: A 35-year-old patient was hospitalized for upper gastrointestinal bleeding. Physical examination and laboratory tests led to the diagnosis of polycythemia (Vaquez disease). Gastroscopy showed an hemorrhagic bulber ulcer. Outcome was favorable after chemotherapy and antinuclear treatment. CONCLUSION: Based on this observation and data in the literature we propose that this unusual presentation would suggest a relationship between polycythemia and ulcer disease.

[Sarcoidosis and ankylosing spondylitis. A case report and review of the literature]. / Sarcoïdose et spondylarthrite ankylosante. A propos d'un cas et revue de la littérature.

UNLABELLED: Ankylosing spondylitis is a very uncommon finding in patients with sarcoidosis. Thirteen cases have been reported in the literature. We report a new case. Observation. - A 40-year-old man had inflammatory low back pain since 1983 which formerly responded to nonsteroidal inflammatory drugs (NSAIDs). He developed dyspnea and skin rash in 1993. Physical exam found cervical and lumbar spine stiffness and violaceous and circular lesions on the forehead, nose and right cheek. Laboratory tests showed: ESR at 50 mm, increased serum angiotensin-1-converting enzyme, and negative HLA B27. X-rays showed sacroiliac ankylosis, cervical and lumbar syndesmophytes and cervical facet joint ankylosis. The plain chest x-ray showed an interstitial syndrome. Chest CT scan showed mediastinal adenopathies. Skin biopsy disclosed non-caseating epitheliod and giant-cell granuloma. Outcome was good with steroid therapy but back pain was only improved by NSAIDs. DISCUSSION: - This association raises a diagnostical problem because spine involvement in sarcoidosis can mimic ankylosing spondylitis. It also suggests the hypothesis of a pathophysiological link between the two diseases.