[Pericarditis as the presenting manifestation of giant cell arteritis]. / Péricardite inaugurale d'une maladie de Horton.

INTRODUCTION: Pericarditis is rarely the presenting manifestation of giant cell arteritis. We report two additional patients. CASE REPORTS: Two patients aged over 70 years presented with acute chest pain. Echocardiography evidenced a pericarditis. Laboratory features showed increased level of acute phase reactants. On questioning, both patients had cephalic symptoms related to giant cell arteritis. Temporal artery biopsy histopathology was characteristic of giant cell arteritis in both. Oral prednisone therapy (20mg/D) led to a complete remission with no clinical relapse of pericarditis after a follow-up of one year and three years, respectively. CONCLUSION: Giant cell arteritis must be evoked in elderly patients with pericarditis because corticosteroids are necessary to avoid ischemic complications of the disease. However, fortuitous association can also be considered. No severe complication of pericarditis has been reported in the literature on corticosteroid therapy.

[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.

[Anti-Ku antibodies. Study of prévalence and of clinical meaning]. / Auto-immunité anti-Ku. Etude de prévalence et de signification clinique.

PURPOSE: Auto-immunity against Ku nuclear antigens is rare and clinical meaning remains badly estimated. Our study is for purposes: to appreciate the prévalence of antibodies anti-Ku within the framework of the search for antinuclear antibodies and to clarify clinical and biological relations associated to this auto-immunity. METHODS: A retrospective study of a series of 10,000 searches for antinuclear antibodies studies the prévalence of the auto-immunity anti-Ku and a retrospective analysis of the data found at the patients bearers of an anti-Ku identifies clinical and biological signs associated with this antibody. RESULTS: Prévalence anti-Ku is low (1/3493 case of antinuclear antibodies) and association is possible with in a myositic process through variable auto-immune contexts (overlap syndrome) of relative good preview. CONCLUSION: Auto-immunity anti-Ku is so characterized with its weak prévalence, a possible observation during different auto-immune diseases with an obvious frequency of the overlap syndrome often concerning a process myositic. Finally a weak évolutivité seems to characterize the auto-immune diseases of the patients with anti-Ku antibodies.

Disseminated reticulate hypomelanosis developing during primary biliary cirrhosis.

We report on a 50-year-old woman with disseminated reticulate hypomelanosis developing on the limbs and abdomen during primary biliary cirrhosis (PBC). Histopathological examination showed vacuolar basal cells, dyskeratosis and large multinucleated epidermal cells. Diagnosis of lichen sclerosus et atrophicus and autoimmune disease are discussed. The similarity between cutaneous changes in PBC and a graft-versus-host reaction is outlined.

[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.

[Corynebacterium (JK group) endocarditis. Apropos of a case]. / Endocardites à Corynebactérie du "groupe JK". A propos d'une observation.

The authors report a case of Corynebacterium (group JK) endocarditis. The main characteristics of the causative pathogen are specified: diagnostic bacteriological criteria, pathogenic capability after cardiac surgery, therapeutic problems related to resistance to antibiotics.