Atteinte du sinus caverneux au cours d’une granulomatose avec polyangéite (maladie de Wegener) sans ANCA

Introduction

Granulomatosis with polyangeitis (Wegener's granulomatosis) (GPA) is a granulomatous vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA). Affected organs usually include upper and lower respiratory tracts, and kidneys. Limited forms of GPA may affect the central nervous system (vasculitis, hypertrophic pachymeningitis, encephalitis), a location in which diagnosis is often uneasy.

Case report

We report a 74-year-old woman who presented with a limited form of GPA affecting the cavernous sinus. Diagnosis was considered in view of a retrospectively suggestive clinical presentation, compatible cerebral MRI and temporal artery biopsy, despite the absence of ANCA. It was supported by a favourable outcome with cyclophosphamide administration.

Conclusion

GPA presenting as a cavernous sinus syndrome is rare. Three co-existing pathogenic mechanisms may be involved in GPA affecting the central nervous system: contiguous invasion from nasal or orbitary granulomatous sites, vasculitis, or primary intra-cerebral granulomatous inflammation. Lack of biopsy evidence of affected tissues and ANCA negativity should not delay diagnosis and appropriate therapeutic management in central nervous system GPA.     

Agranulocytose et vascularite chez un patient cocaïnomane : lévamisole ou l’autre poison caché

Introduction

Adulterants are compounds added to street drugs to increase profits for the seller. Levamisole, a veterinary antihelminthic agent, has become the most common adulterant of cocaine. The prevalence of levamisole in samples of cocaine is increasing. Levamisole can lead to neutropenia and to a dramatic vasculopathy and even vasculitis of small and medium-size blood vessels.

Case report

We here reported the first French case of levamisole related toxicity, due to cocaine use in a 50-year-old man, revealed by fever and agranulocytosis, high titters of antineutrophil cytoplasmic antibodies (ANCA), anticoagulant and positive Coombs tests. Outcome was slowly favorable with exposition withdrawal.

Conclusion

Clinicians should be aware that agranulocytosis or vasculitis or vasculopathy could be related to levamisole toxicity in individuals who use cocaine.     

Vascularite cutanée à anticorps anti-cytoplasme des polynucléaires neutrophiles induite par le benzylthio-uracile : à propos d’un cas et revue de la littérature

Introduction

Vasculitis associated to antineutrophil cytoplasmic antibodies is a rare complication of therapy with antithyroid medication. They were mainly reported in patients treated with propylthiouracil and rarely with benzylthiouracil.

Case report

We report a 22-year-old woman treated with benzylthiouracil for Graves’ disease, who developed a vasculitic skin involvement. The presence of antineutrophil cytoplasmic antibodies with anti-myeloperoxidase specificity was documented. The discontinuation of benzylthiouracil was followed by a complete disappearance of skin lesions and of antineutrophil cytoplasmic antibodies.

Conclusion

To our knowledge, only ten cases of antineutrophil cytoplasmic antibodies vasculitis induced by benzylthiouracil have been previously reported in the literature. Our patient was characterized by the occurrence of isolated cutaneous vasculitis, without renal involvement. Early discontinuation of benzylthiouracil may have prevented the occurrence of severe visceral complication.     

Maladie de Niemann-Pick de type B : description clinique de trois cas familiaux

Introduction

The Niemann Pick disease type B is a rare deficiency in sphingomyelinase activity, autosomal recessively inherited.

Case reports

We report three patients (two men, one woman) of the same family, who showed pulmonary and hepatosplenic lesions, usually present in the disease but also adrenal gland lesions confirmed by tomodensitometry.

Conclusion

The current treatment of Niemann Pick disease is purely symptomatic awaiting the use of enzymatic replacement therapy which has been successfully experimented in animal model.     

Efficacité clinique et biologique du tocilizumab au cours de la maladie de Horton : à propos de trois observations et revue de la littérature

Introduction

Treatment of giant cell arteritis is based on prolonged corticosteroid therapy but adverse side effects are common especially in the elderly.

Case reports

We report three patients with giant cell vasculitis treated by tocilizumab, an interleukin-6 receptor antibody, owing to resistance or intolerance to corticosteroid therapy. A favorable outcome was rapidly observed both on clinical and biological data allowing a corticoid therapy sparing.

Conclusion

Tocilizumab is a promising treatment of giant cell arteritis but controlled trials are needed to confirm its efficacy.     

Syndrome d’encéphalopathie postérieure réversible induit par un décongestionnant nasal contenant de la pseudo-éphédrine

Introduction

Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric oedema on neuroimaging. Many pathological conditions and treatments have been associated with this syndrome.

Case report

We report a 19-year-old woman, followed-up for hypocomplementemic urticarial vasculitis, who presented with a posterior reversible encephalopathy syndrome induced by the intake of an over-the-counter cold remedy containing pseudoephedrine. Clinical manifestations and radiological abnormalities resolved after anti-hypertensive therapy and withdrawal of sympathomimetic drug.

Conclusion

The diagnosis of posterior reversible encephalopathy syndrome should be considered in patients with compatible clinical and radiological presentation because of its potential reversibility with an appropriate management. Intake of drugs, including over-the-counter cough and cold drugs, should be looked for in the history as well as autoimmune disorders.     

Événements infectieux au cours des vascularites nécrosantes systémiques : étude rétrospective de 82 cas

Purpose

The aim of this study was to assess the infections occurring in a series of 82 patients followed for a systemic necrotizing vasculitis and to determine potential risk factors.

Methods

We studied retrospectively the medical files of 23 Churg and Strauss syndrome, 18 periarteritis nodosa, 14 microscopic polyangiitis, and 27 granulomatosis with polyangiitis, over a 15-year period. Infection delay corresponded to the period from treatment to first infection or between two infections.

Results

A total of 61 patients developed 147 infections. Causal agent was identified in 70 cases, 42 were bacterial, 20 viral and 8 fungal. Bronchopneumonia was the most frequent infection (43 %). Sixty-two percent of infections occurred within 2 years after vasculitis diagnosis. Seven infections were major, requiring intensive care, with one infection-death related. Pneumocystis prophylaxis concerned 75 % of patients on cyclophosphamide. Significant factors reducing infection delay were initial hypergammaglobulinemia, hypoalbuminemia, lymphopenia, as well as cyclophosphamide and methotrexate treatment. Large quantities of corticosteroids, cyclophosphamide or azathioprine increased infection delay. This result underlines the early occurrence of infectious complications during vasculitis course.

Conclusion

Infectious events occurring in systemic necrotizing vasculitis are frequent and occurs early in disease course, and could be prevented with simple prophylactic measures. Vasculitis relapse and infection share similarities and this require permanent clinical vigilance.     

Tamponnade et myocardite aiguë au cours d’un syndrome de Churg-Strauss

Introduction

The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome.

Case report

A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment.

Conclusion

Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment.     

Complications de la gastroentérite à éosinophiles : une observation et revue de la littérature

Introduction

Eosinophilic gastroenteritis is an unusual disease characterized by an eosinophilic infiltration of the gastrointestinal tract. The esophageal location of this disorder is uncommon and is usually revealed by dysphagia. Diagnosis is obtained by histology during endoscopy after exclusion of differential diagnosis. Treatment is based on systemic corticosteroids, which improve dramatically symptoms and endoscopic lesions.

Case report

We report an 88-year-old man who presented eosinophilic gastroenteritis with esophageal injury complicated by gastrointestinal haemorrhage and fistule.

Conclusion

Eosinophilic gastroenteritis may have a potentially unfavourable outcome. The treatment of complicated forms is not codified and often empirical.     

Paralysie du nerf récurrent au cours d’une maladie de Lyme : à propos de deux observations

Introduction

Neuroborreliosis can be a difficult diagnosis which requires epidemiologic, clinical and biologic arguments.

Case reports

We report two patients who presented with a recurrent laryngeal nerve palsy with positive Lyme serology and favorable outcome after antibiotic therapy. In one case, a lymphocytic meningitis with intrathecal production of specific antibodies was evidenced.

Conclusion

Recurrent laryngeal nerve palsy is an uncommon manifestation of neuroborreliosis. Lyme serology is an important tool when neurologic disorder occurs because of an atypical course of Lyme disease.     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

Un cas de syndrome de Hoigné lors d’une injection intraveineuse de ceftriaxone

Introduction

Hoigne's syndrome is characterized by the development of acute clinical manifestations which are mainly psycho-sensorial. Classically, these features immediately follow the injection of procaine penicillin G.

Case report

We report a 59-year-old man who presented with psycho-organic manifestations that occurred just after the intravenous injection of ceftriaxone; to our knowledge, this is the first case of Hoigne's syndrome reported after an injection of this antibiotic.

Conclusion

The pathophysiologic basis of this syndrome is still unknown. It is important to keep in mind its clinical characteristics, which may mimic immuno-allergic symptoms. It should be differentiated from anaphylactic manifestations because Hoigne's syndrome allows the continuation of the treatment.     

Rétinopathie dysorique à l’interféron au cours de l’hépatite chronique virale C. À propos d’un cas et revue de la littérature

Introduction

The ophthalmic complications following interferon therapy in chronic hepatitis C are rare. The most common adverse ophthalmic outcome is the dysoric retinopathy characterized by the presence at the fundus examination of cotton wool spots and retinal hemorrhages particularly around the optic disc.

Case report

A 63-year-old man presented to the hepatology department with a compensated cirrhosis C. His medical history was positive for hypertension controlled by medical treatment. A combined treatment with pegylated interferon α2a plus ribavirin was initiated. Three months later, the patient reported a sudden decreased vision in both eyes. Fundus examination revealed cotton wool spots with retinal hemorrhage. The diagnosis of dysoric retinopathy was established. The antiviral treatment was discontinued. One month later, the patient was asymptomatic and the ocular lesions have disappeared.

Conclusion

Dysoric retinopathy is a non-specific complication of interferon therapy in chronic hepatitis C. Despite its good prognosis, a careful fundus examination is required before and during the treatment especially for the patients with risk factors for this adverse event (advanced age, diabetes and high blood pressure).     

Caractéristiques cliniques,paracliniques et profil évolutif de l’atteinte aortique de la maladie de Horton : à propos de 26 cas d’aortite parmi 63 cas de maladie de Horton

Purpose

Aortic involvement that occurs in temporal arteritis is probably underestimated because it is usually asymptomatic. The characteristics of giant cell arteritis with aortic involvement are still poorly described and the relationship between aortitis and vascular outcome of the disease has not been clearly delineated. The objective of this retrospective study of 63 patients with giant cell arteritis, including 26 with aortic involvement, was to compare the features of patients with and without aortitis, and to assess the contribution of CT-scan and FDG-PET-scan in screening for vascular disease, monitoring, and therapeutic management of patients.

Methods

This retrospective study was conducted in the internal medicine department of the university hospital in Marseille, France, from January 1, 2005 to September 30, 2011. Patients had at least three out of the five American College of Rheumatology criteria for temporal arteritis and aortic involvement was investigated in all patients using CT-scan. Aortic wall thickness greater or equal to 3 mm was considered to be abnormal.

Results

Of 63 patients diagnosed with giant cell arteritis, 26 (41.3%) had aortic involvement diagnosed by aortic CT-scan. Age at diagnosis was significantly younger (66.8 vs 73.8 years; P = 0.002) in the group with aortitis. Inflammatory dorsal and low back pain, signs of vascular disease of the upper limbs (P = 0.009), and higher level of acute phase reactants were associated with aortitis. Aneurysmal lesions of the aorta were significantly more frequent in the group with aortitis. Twenty patients had both aortic CT-scan and FDG-PET-scan. For patients in whom aortic involvement was not demonstrated with CT-scan, FDG-PET-scan was always non-contributive. With corticosteroids, aortitis resolved within 6 months in all patients as evaluated by aortic CT-scan. However, aortitis persisted in 80% of cases at 6 months when evaluated with FDG-PET-scan, and in 66% of cases at 12 months, without influencing the treatment.

Conclusion

This case series shows no specific features of aorta and its main roots involvement in giant cell arteritis, justifying a systematic screening by CT-scan. The high frequency of this arterial involvement could help physicians in the diagnosis of giant cell arteritis. Aortitis seems to be associated with vascular complications as highlighted by the frequency of aortic aneurysm and a case of early aortic dissection. Finally, the role of PET-CT-scan for screening vascular disease and therapeutic monitoring remains to be clarified.     

L’entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique

Introduction

. Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis.

Case reports

. We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded.

Conclusion

. Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.     

Complications infectieuses induites par le mésusage de la buprénorphine haut dosage (Subutex) : analyse rétrospective de 42 observations

Purpose

Misuse of high-dose buprenorphine (HDB), mainly by injection, is responsible of frequent infectious adverse events.

Methods

This is a retrospective study of infectious complications occurring in patients using HDB by injection. Forty-two cases were identified (29 men and ten women) and the data were collected between March 1999 and December 2008.

Results

The infectious complications included cutaneous infections (27 cases), endocarditis (nine cases), osteoarticular infections (four spondylodiscitis and one sacroiliitis), and a vascular embolism with decrease in visual acuity.

Conclusion

The results of HDB maintenance treatment must be improved, both from the point of view of substitution and to limit its misuse by intravenous route injection. Health professionals have to play an important role in drug addict patients’ education and supervision, to prevent buprenorphine injection and related infectious complications.     

Cribado de Chagas en mujeres gestantes latinoamericanas. Experiencia en el Poniente Almeriense

Introduction

The transmission of Chagas disease is a public health problem in non-endemic countries.

Methods

Chagas screening was performed by two serological tests in pregnant women from endemic areas for 4 years.

Results

We studied 261 pregnant women from 13 Latin American countries, making a confirmatory diagnosis (two positive tests) in 4 cases. There was no case of vertical transmission.

Conclusion

Although Chagas disease has a low prevalence in the province of Almeria, the screening is necessary for the detection and treatment of infants with the disease.     

Complications vasculaires de l’abord artériel fémoral : impact du système de fermeture percutanée Perclose

Introduction

Vascular complications at the femoral access site is an important factor of morbidity. The aims of this study were to evaluate the efficacy and safety of the percutaneous closure device (Perclose) during interventional cardiology procedures.

Patients and methods

All patients with percutaneous closure of the femoral access site by the Perclose system in 2010 were included. We evaluated the indications of the procedures, the success rate of implantation and the bleeding complications according to antithrombotic therapy used.

Results

Three hundred and seventy five patients underwent a percutaneous closure by the Perclose system. Acute coronary syndromes with or without elevation of ST segment were the main indications of procedures (74.9%). The success rate of percutaneous closure of the femoral access site was 97.3%. The rate of minor, moderate, and severe bleeding of the entire cohort according to the GUSTO classification was respectively 4.2%, 0.5% and 1%. Bleeding complications were similar in both groups of patients (2.1% vs 7% P = 0.122). In case of unsuccessful deployment of femoral closure devices, the risk of bleeding complications range from 4.3% to 60% (P = 0.0000036).

Conclusion

The use of the Perclose system is associated with a low rate of severe bleeding at the femoral access site. However, the failures of percutaneous closure increase the risk of bleeding complications.     

Thrombophlébite cérébrale : une complication rare de l’infection aiguë à cytomégalovirus

Introduction

Acute cytomegalovirus (CMV) infection increases the risk of vascular thrombosis but reports of cerebral venous thrombosis are rare.

Case report

We report a 36-year-old woman who presented with a cerebral venous thrombosis and acute CMV infection heralded by a cytolytic hepatitis. Heterozygous factor V Leiden mutation was also identified. The patient was treated with anticoagulation for 1 year with favourable outcome.

Conclusion

Serologic tests for CMV infection should be performed in case of cerebral venous thrombosis with liver cytolysis or flu-like symptoms. CMV infection often triggers thrombosis in combination with other inherited or genetic predisposing risk factors that should always be searched.