Search

Background: The association of inflammatory myopathy and myasthenia gravis is a rarely described entity whose clinical presentation has always been intriguing because of the great clinical similarity between these two pathologies. The presence of a thymic pathology often explains this combination, whose mechanisms are very complex., Case Presentation: A 56-year-old woman of North African origin, was hospitalized to explore the Raynaud phenomenon associated with proximal muscle weakness, pain, and arthralgia. There was no rash, and neuromuscular examination had revealed proximal tetraparesis and mild neck weakness. Tendon reflexes were normal. There was no abnormal nail fold capillaroscopy. A significant titer of muscle enzymes had been shown on blood tests, and autoimmune screening for myositis-specific and myositis-associated autoantibodies was negative. Electromyography had shown a myopathic pattern, and muscle biopsy confirmed an inflammatory myopathy. Although steroids were introduced, the clinical course was unsatisfactory; ophthalmic and bulbar symptomatology appeared. The association of myasthenia gravis was confirmed by an elevated level of serum acetylcholine receptor. A chest computed tomography scan had identified a thymoma. Treated with prednisone, pyridostigmine, and thymectomy, the patient's clinical and biological evolution was favorable., Conclusion: This case illustrates an exceptional association of two entities and the difficulty encountered during their diagnosis and treatment. The management of these two diseases is different, so it is essential to recognize this concomitant presentation., (© 2021. The Author(s).)

Introduction: Bronchopulmonary sequestration is a rare congenital abnormality characterized by the presence of a non-functioning mass of lung tissue which receives an aberrant arterial supply from systemic circulation., Case Report: We describe the case of a 30-year-old man who presented with recurrent episodes of mild hemoptysis. CT angiography revealed a right inferior lobar sequestration supplied by three systemic arteries. The patient underwent thoracotomy with right lower lobectomy and section-ligature of the three abnormal vessels., Conclusion: This case highlights the radiological features as well as the intraoperative appearances of this rare anomaly., (Copyright © 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

The association of immune thrombocytopenic with malaria is a rare event. We describ the case of a young soldier who, after returning from Central Africa, presented a fever associated with petechial purpura and gingivorrhagia, hemogram showed deep thrombocytopenia and macrocytic normochrome anemia, thick peripheral blood smears confirmed the diagnosis of Plasmodium falciparum malaria, the patient was treated with quinine, but deep thrombocytopenia and hemorrhagic manifestations persisted, the patient then underwent corticosteroid therapy, with favorable evolution and progressive normalization of platelets.

Each year, hundreds of cases of malaria are reported in Morocco and occur after anopheles in patients who have stayed in endemic areas, but transmission following an accident of exposure to blood is rare or exceptional, only about 20 cases are published. We report a case of malaria in a nursing staff following an accidental sting with a catheter needle.

Introduction: Polyarteritis nodosa (PAN) is a systemic vasculitis with polymorphic manifestations. Ocular involvement is rare; we report two such cases., Cases: The first case was a 56-year-old man with PAN treated with corticosteroids. A year and a half later, during a disease outbreak associated with decreased visual acuity, ocular examination objectified a localized pigment epithelial detachment in the right eye. The outcome was favorable with corticoid bolus followed by azathioprine. The second case was a 41-year-old man presenting with PAN and decreased visual acuity. Ophthalmologic examination showed inferotemporal branch vein occlusion in the right eye with serous retinal detachments in the left eye. The outcome was favorable with intravenous cyclophosphamide followed by azathioprine., Discussion: The incidence of ocular involvement in PAN varies from 10 to 20%. It is most often associated with vasculitis of retinal and choroidal arteries. Fluorescein angiography is the gold standard of diagnosis. Multifocal acute ischemia of the choriocapillaris is very pathognomonic. Immunosuppressant drugs are effective and should be reserved for severe forms., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Acute pancreatitis and haemophagocytic syndrome (HS) are rarely seen in systemic lupus erythematosus (SLE)., Patients and Methods: We report the case of a young female patient without any noteworthy prior history, who was hospitalised for abdominal pain associated with acute pancreatitis possibly related to SLE with associated haemophagocytic syndrome., Discussion: Screening for cutaneous symptoms of lupus in patients with pancreatitis can help avoid diagnostic errors., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Possible adverse complications related to rituximab (RTX) are low, some of which are extremely rare. The authors describe one situation visibly waning exceptional treatment with RTX for SLE refractory to conventional therapies., Comment: The authors report a patient of 34 years followed for months for an illness in its bullous lupus, with cutaneous, articular, hematologic and immunologic. Given a corticosteroid resistance, several therapeutic background based hydroxychloroquine, cyclophosphamide and methotrexate, were initiated without any improvement. Immunomodulatory therapy type RTX was introduced to this form refractory at a rate of 375mg/m(2)/week. The waning of the second infusion, the patient presented a sudden intense abdominal pain syndrome, revealing an acute catarrhal appendicitis. At distance from the appendectomy, the consequences of which were favorable, treatment with RTX was resumed. In the aftermath of the third infusion, the patient presented in table tract marked by profuse watery diarrhea whose explorations reveal a morphological endoscopic appearance of erythematous, ulcerative colitis, reversible upon discontinuation of treatment. Histological data revealed important infiltrates composed mainly of CD8T lymphocytes., Conclusion: Gastrointestinal immunological consequences to the requirements of the targeted therapies deserved very careful and rigorous monitoring. However, at the slightest sign of digestive, a detailed morphological exploration is essential, to avoid in particular surgical emergency, evolution without treatment could engage in short-term vital prognosis., (2011 Elsevier Masson SAS. All rights reserved.)

Introduction: The cardiac involvement in hypereosinophilia remains a major cause of morbidity and mortality. Recent advances have identified new molecular mechanisms responsible for the expansion of the eosinophilic lineage, allowing a better classification of the different forms of Hypereosinophilic syndrome (HES) and especially targeted therapy. Since the discovery of the involvement of deregulated tyrosine kinases in the pathophysiology of these diseases, and particularly the identification of the fusion gene FIP1L1-PDGFRA, new molecules inhibiting specifically this signaling pathway (imatinib) were individualized, leading to dramatic therapeutic benefits in proliferative forms of HES considered before that of very poor prognosis., Case Report: We report here the dramatic effectiveness of imatinib used as second line therapy for dilated cardiomyopathy revealing a hypereosinophilic syndrome in a patient in whom the search for FIP1-L1-PDGFRA fusion gene was negative., Conclusion: If hypereosinophilia has varied clinical and morphological outcome, its clinical consequences, particularly on heart function, are sometimes dreadful, and are not correlated either with blood eosinophil levels or with a specific etiology. We report here a case of HES lacking the FIP1-L1-PDGFRA fusion gene showing that despite the absence of this molecular defect, imatinib mesylate may have therapeutic interest in those cases of HES resistant to first line therapies., (2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Autoimmune hemolytic anemia with cold autoantibodies or cold agglutinin disease is a rare chronic disorder in which no treatment has, until now, evidence of its effectiveness., Clinical Case: We report a patient who successfully responded to rituximab for a cold agglutinin disease refractory to conventional therapy with very good tolerance and a complete remission., Conclusion: There are only few observations that have been reported in the literature regarding the efficacity of rituximab in the treatment of cold agglutinin disease. This promising therapy could, in the future, constitute a real alternative., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

The acronym RACAND means the association of Raynaud's phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynaud's phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells.

Septic pylephlebitis or purulent thrombosis of the portal venous system generally results from a progressive extension of suppurated thrombophlebitis, secondary to an intrabdominal infection. Germs most often found are Escherichia coli and Streptococcus, isolation of Enterobacter cloacae is unusual. We report a particular observation of septic pylephlebitis associated with E. cloacae bacteremia, without biliary, digestive or pancreatic lesion on the CT-scan. The antibiotic sensitivity pattern of the isolated germ and the negative epidemiologic investigation pled in favour of community acquired infection. The infection resolved with antibiotics and anticoagulation, followed by total repermeation of the portal system., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)

McArdle's disease (MAD) is a rare hereditary myopathy secondary to a deficit in myophosphorylase, an essential enzyme for the use of muscular glycogen reserves. Exercise intolerance to a variable degree is the fundamental manifestation. Muscular enzymes are usually normal or slightly elevated, except during episodes of rhabdomyolysis. Generally, the electromyogram has poor sensitivity for the diagnosis of exercise myopathies. The muscular biopsy can be misleadingly normal. The role of MRI in the diagnosis of MAD is not well clarified in the literature. We report the case of a 16-year-old patient, hospitalized in July 2008 for exercise intolerance. On admission, he was asymptomatic and the physical examination was non contributive. Serum creatine kinase levels and renal function measures were normal. Cycloergometer exercise testing unmasked the disease. EMG and muscular biopsies were normal. During the second hospitalization, this time for rhabdomyolysis, T2 weighted MRI of the thighs showed high intensity signals from the gracilis muscles. The control MRI, made after 2 weeks of rest, was normal. Right gracilis muscle biopsy demonstrated excess glycogen with myophosphorylase deficiency, establishing the diagnosis of MAD. MAD is a rare metabolic myopathy to consider in patients with a history of exercise intolerance. The muscle biopsy can be misleadingly normal and should be, to our opinion, be guided by MRI findings.

Introduction: The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease., Exegesis: We report a case of Wilson's disease revealed by acute intravascular repeating hemolytic anemia associated with liver failure. The initially negative etiologic investigation was directed by occurred of liver failure. The genetic study allowed to discover an other similar case. The evolution was favourable under treatment with zinc sulfate and penicillamine., Discussion: Diagnosis of Wilson's disease must be considered in case of acute hemolytic anemia associated with liver failure in young adults.