[Wegener's granulomatosis with clinical manifestations of temporal arteritis]. / Une maladie de Wegener avec des signes d'artérite temporale.

A 51-year-old woman presented with crusting rhinitis, bilateral serous otitis, inflammatory arthralgias, fever, weight loss and signs of temporal arteritis. Temporal arteries were increased in size, painful, with inflammatory signs. There was microscopic hematuria and inflammatory parameters were increased. The renal function was normal. Anticytoplasmic neutrophils antibodies were detected (anti-PR3). Temporal artery biopsy did not show signs of giant cell arteritis. A diagnostic of Wegener's granulomatosis was established and steroid treatment allowed disappearance of clinical and biologic features.

Increased exposure to statins in patients developing chronic muscle diseases: a 2-year retrospective study.

OBJECTIVE: Case reports have suggested that lipid-lowering drugs (LLDs), especially statins, could induce or reveal chronic muscle diseases. We conducted a study to evaluate the association between chronic muscle diseases and prior exposure to LLDs. METHOD: This was a retrospective study of chronic primary muscle disease cases newly diagnosed at the Toulouse University Hospitals between January 2003 and December 2004 among patients living in the Midi-Pyrénées area, France. All patients remained symptomatic for more than 1 year after drug withdrawal, or required drugs for inflammatory myopathy. Data on the patient's exposure to LLDs and to other drugs were compared with that of matched controls (5/1) selected through the Midi-Pyrénées Health Insurance System database. RESULTS: A total of 37 patients were included in the study. Of those, 21 (56.8%) suffered from dermatomyositis (DM) or polymyositis (PM), 12 (32.4%) from genetic myopathy, and 4 (10.8%) from an unclassified disease. The prevalence of exposure to statins was 40.5% in patients and 20% in controls (odds ratio (OR) 2.73, 95% confidence interval (CI) 1.21-6.14; p<0.01). There was a significant positive interaction between statins and proton pump inhibitors exposure (weighted OR 3.3, 95% CI 1.37-7.54; p = 0.02). Statin exposure rate was 47.6% among patients with DM/PM (OR 3.86, 95% CI 1.30-11.57; p<0.01). There was no difference between patients and controls for exposure to fibrates. CONCLUSION: Patients who developed chronic muscle diseases after the age of 50, including DM/PM, had a higher than expected frequency of prior exposure to statins. Further studies are needed to confirm this association and the role of proton pump inhibitors.

[Choroidal metastasis as a presenting feature of breast carcinoma]. / Métastase choroïdienne révélant un cancer du sein.

Choroidal metastasis is a rare presenting feature of breast carcinoma. A 48-year-old woman presented with blurred vision of the right eye related to choroidal metastasis. Diagnostic work-up disclosed breast carcinoma with multiple metastases of the liver and lungs. Initial cerebral computed tomography scan was normal. During the follow-up, generalized seizure leaded to the diagnosis of multiple calcified cerebral metastasis. In 15 to 30 percent of cases, choroidal metastasis reveals a solid tumor, usually of the lung or the breast. Cerebral metastasis are common in breast cancer, but rarely calcified.

[Angiosarcoma of the aorta]. / Angiosarcome épithélioïde de l'aorte.

Primary aortic tumors are extremely rare. A 73-year-old woman presented with a 8 kg weight loss associated with abdominal pain. Physical examination was normal. Laboratory tests disclosed increased acute phase reactants. Thoracic and abdominal CT scan showed diffuse splenic and renal hypodense lesions with thrombotic feature of the thoracic aorta extending on 9 cm length. Transesophageal echocardiography showed a large and heterogeneous floating mass advocating a thrombus developed on atheroma. Because of the high risk of embolism the patient underwent surgical replacement of the thoracic aorta. Histopathology revealed an epithelioid angiosarcoma of the aorta. A primary tumor of the aorta should be suspected in the presence of an intra-aortic process presenting features of thrombosis.

Pulmonary involvement in Sweet's syndrome: a case report and review of the literature.

Pulmonary involvement in Sweet's syndrome (SS) is rare. We report a case of SS with severe respiratory involvement responding to corticosteroid therapy. A 82-year-old man presented fever of 39 degrees C associated with cough and dyspnea, and crackles in the left lung. The infection work-up was negative. Chest X-ray showed cardiomegaly and left lower lobe pulmonary infiltrates. Pulmonary signs did not improve on treatment with antibiotics, and after 1 week maculopapular lesions appeared, localized on the knees, the periombilical area and the back. The antibiotics were changed without improvement. A skin biopsy revealed infiltration by neutrophilic granulocytes and marked edema in the dermis, consistent with SS. The patient's condition progressively worsened, requiring high oxygenotherapy, and he was transferred to an intensive care unit. Chest X-ray revealed an important alveolar and interstitial syndrome. Bronchoalveolar lavage found 170 leukocytes with 30% neutrophils (N < 5%), 7% lymphocytes and 63% macrophages. A search for bacteria, viruses or parasites in bronchoalveolar lavage was negative. The patient was treated with antibiotics, a high dose of furosemide and steroids for 4 days. Because the patient improved dramatically within 5 days, with a negative infection work-up and a dramatic decrease of C-reactive protein, the antibiotics were stopped. Steroids were secondarily tapered very slowly. A chest computed tomography (CT) scan showed a substantial improvement of pulmonary lesions. We also review the 22 cases of pulmonary involvement of SS reported in the literature.

Sweet's syndrome revealing relapsing polychondritis.

Sweet's syndrome is extremely rare in relapsing polychondritis. We report a rare case of Sweet's syndrome revealing relapsing polychondritis. A 77-year-old man presented with fever, associated with respiratory symptoms and non pruritic, painful, erythematous, papules and plaques mainly on the arms and legs. Skin biopsy of the arm found a neutrophilic dermal infiltrate consistent with Sweet's syndrome. A month and a half after admission, the patient presented with ear chondritis and dysphonia. Biopsy of the ear cartilage found a significant perichondral inflammatory cells infiltrate. The diagnosis of relapsing polychondritis was established and a steroid treatment was instituted with a complete regression of the cutaneous signs and chondritis.

Spondylodiscitis due to Aerococcus urinae: a first report.

Aerococcus urinae is an uncommon urinary tract pathogen which causes infections predominantly in elderly persons with local and general predisposing conditions. There are few case reports of severe infections caused by Aerococcus urinae among which only 14 cases of endocarditis caused by Aerococcus urinae have been reported. We report the first case of Aerococcus urinae spondylodiscitis. The patient responded to 4 weeks of amoxicillin and clindamycin, and to 5 months of amoxicillin alone.

[Association of acquired Willebrand's disease, monoclonal gammopathy and angiodysplasia of the small bowel: a rare indication of high-dose intravenous immunoglobulins]. / Association maladie de Willebrand acquise--gammapathie monoclonale--angiodysplasie du grêle: une indication rare des immunoglobulines intraveineuses á forte dose.

Acquired von Willebrand's disease associated with a monoclonal gammopathy and angiodysplasia of the gut is a rare disorder. It is sometimes complicated by chronic intestinal bleeding and severe anemia, that is poorly responsive to usual treatments. We report such a new case that has been revealed by anemia, and characterised by the absence of the high-molecular weight multimers. The correction of the hemostasis defect and of anemia were related to the reappearance of the high-molecular weight multimers, that was achieved only after high-dose intravenous immunoglobulin courses. The perfusions were performed every 3 weeks for 2 years without loss of efficiency, that could be explained by the dissociation of immunoglobulin-von Willebrand's factor complex.