Atteinte osseuse axiale de la sarcoïdose.

INTRODUCTION: Sarcoidosis is a multi-systemic disease characterized by non-caseating granulomas. Bone involvement initially considered as rare and described as a peripheral osteitis of the hands and feet, has recently been reported on the axial skeleton. CASE REPORTS: We report 4 clinical observations of sarcoidosis (3 women, 1 man) with axial bone involvement located to the spine (n = 4), pelvic bone (n = 2), scapular bone (n = 2), sternum (n = 1), mandible (n = 1). Sarcoidosis was already diagnosed in 3 cases. Bone pain was the main symptom, related in 3 cases. Magnetic resonance imaging appeared to be the best imaging test Histological bone analysis revealed typical granulomatous lesions (n = 2). Treatment included corticosteroids (n = 4), hydroxychloroquine (n = 2), and methotrexate (n = 2), with a good efficacy on bone pain in symptomatic patients. CONCLUSION: These 4 cases, as well as recent literature, illustrate bone involvement of sarcoidosis on the axial skeleton. It is symptomatic in around 50% of cases but may be a source of significant disability. Differential diagnosis with neoplasm may require bone histological analysis. This condition appears to be responsive to usual treatments for sarcoidosis.

Cholangiopathy in critically ill patients surviving beyond the intensive care period: a multicentre survey in liver units.

BACKGROUND: The outcome of cholangiopathy developing in intensive care unit (ICU) is not known in patients surviving their ICU stay. AIM: To perform a survey in liver units, in order to clarify the course of cholangiopathy after surviving ICU stay. METHODS: The files of the liver units affiliated to the French network for vascular liver disease were screened for cases of ICU cholangiopathy developing in patients with normal liver function tests on ICU admission, and no prior history of liver disease. RESULTS: Between 2005 and 2015, 16 cases were retrieved. Extensive burns were the cause for admission to ICU in 11 patients. Serum alkaline phosphatase levels increased from day 11 (2-46) to a peak of 15 (4-32) × ULN on day 81 (12-511). Magnetic resonance cholangiography showed irregularities or frank stenosis of the intrahepatic ducts, and proximal extrahepatic ducts contrasting with a normal aspect of the distal common bile duct. Follow-up duration was 20.6 (4.7-71.8) months. Three patients were lost to follow-up; 2 patients died from liver failure and no patient was transplanted. One patient had worsening strictures of the intrahepatic bile ducts with jaundice. Nine patients had persistent but minor strictures of the intrahepatic bile ducts on MR cholangiography, and persistent cholestasis without jaundice. One patient had normal liver function tests. CONCLUSIONS: In patients surviving their ICU stay, ICU cholangiopathy is not uniformly fatal in the short term or clinically symptomatic in the medium term. Preservation of the distal common bile duct appears to be a finding differentiating ICU cholangiopathy from other diffuse cholangiopathies.

[Abdominal pain in progressive encephalomyelitis with rigidity]. / Encéphalomyélite progressive avec rigidité révélée par une douleur abdominale.

INTRODUCTION: Stiff-person syndrome is rare neurological disease, associating trunk rigidity and painful muscular spasms. A clinical variant of stiff person syndrome is the progressive encephalomyelitis with rigidity and myoclonus (PERM), which includes neurological cognitive disturbances. CASE REPORT: We report a 73-year-old woman initially addressed for abdominal pain, anorexia and severe weight-loss, for whom diagnosis of PERM was made. CONCLUSION: Because of its various clinical presentations, sometimes without evidence for neurological disease, the diagnosis of PERM is delayed. The presence of antineuropile antibodies associated with muscular spasms at electromyogram are strong evidence for this diagnosis.

[Granulomatosis with polyangiitis (previously Wegener's granulomatosis) mimicking malingering]. / Forme pseudo-pathomimique de granulomatose avec polyangéite (anciennement maladie de Wegener).

INTRODUCTION: ANCA vasculitis may involve the skin and develop slowly without specific histology, and without autoantibodies. CASE REPORT: We report a 50-year-old woman who experienced bilateral mastectomy because of ulcero-necrotic, non-specific inflammatory cutaneous lesions of the breasts. First considered by others as a malinger patient, she developed oto-neurological lesions leading to the diagnosis of Wegener's granulomatosis. Five years later, specific antibodies of the disease were present. CONCLUSION: Cutaneous involvement by ANCA vasculitis can be isolated for a long time. Physicians must have a high degree of suspicion to avoid diagnostic delay of ANCA vasculitis.

[Ischemic cholangitis in intensive care unit: favourable outcome with ursodesoxycholic acid and fenofibrate]. / Cholangite sclérosante de réanimation : évolution favorable sous acide ursodésoxycholique et fénofibrate.

INTRODUCTION: Ischemic cholangitis in intensive care unit is a recently reported liver disease in patients who have had a prolonged mechanical ventilation and vasopressive drug support for multiple organ deficiency. Prognosis is usually poor and the only life-saving therapy is liver transplantation despite ursodesoxycholic acid treatment. CASE REPORT: We report a 63-year-old man who presented with a sclerosis cholangitis after a month in intensive care unit, effectively treated with fenofibrate and ursodesoxycholic acid. Recent reports underline fenofibrate efficacy in the treatment of primary biliary cirrhosis, especially in association with ursodesoxycholic acid. This treatment has prevented liver transplantation for our patient with a correct quality of life. CONCLUSION: The addition of fibrate to ursodesoxycholic acid improves persistent cholestasis in sclerosing cholangitis.

[Fungal sinusitis: report of two cases of indolent form of fungus usually invasive, up treatment and care]. / Sinusites fongiques: à propos de deux cas de forme indolente de mycose habituellement invasive, place du traitement et prise en charge.

OBJECTIVES: Unlike Aspergillus sinusitis, sinus infections by mucormycosis are often invasive and have a lethal potential. We report two cases of chronic sinusitis with unilateral uncomplicated diagnosis of mucormycosis confirmed by pathology or mycology and discuss the therapeutic strategy. PRESENTATION: Case 1) An immunocompetent patient with a right chronically sphenoid sinusitis uncomplicated underwent endonasal surgery in our institution. Rhizopus Oryzae was found on the surgical mycological specimen. The patient received no adjuvant medical therapy after surgery, because there were no invasive clinical or radiological criteria and no histopathological confirmation. Clinical, radiological and mycological follow-up was done. Case 2) A patient with a chronic maxillary sinusitis underwent endonasal surgery in our institution. Histological analysis found filaments in favor of mucormycosis without vascular emboli. We decided in agreement with the national reference center for mycology not to treat medically. CONCLUSION: A follow up of more than 3 years for both cases tends to confirm this collective decision.

[Postexercise duplex ultrasound to diagnose external iliac endofibrosis]. / Endofibrose de l'artère iliaque externe diagnostiquée par écho-doppler post-effort.

Arterial endofibrosis is a disease of recent discovery which concerns high-performance athletes, predominantly competitive cyclists. The preferential location is the external iliac artery. The symptoms are diverse (pain, edema, paresthesia), always linked to an effort. The diagnosis may be delayed due to atypical symptoms in athletes. Complementary tests are measure of the systolic pressure index after exercise, duplex ultrasound, CT angiography, MR angiography and arteriography. We report a case of endofibrosis where late diagnosis was established with postexercise duplex ultrasound, while CT angiography and arteriography failed to reveal characteristic abnormalities.