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.     

Atteintes ORL de la maladie de Fabry : à propos de 25 observations

Purpose

Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disorder due to deficient activity of the enzyme alpha-galactosidase A. Males and females exhibit severe organ involvement. The high incidence of otological symptoms was recently reported.

Patients and methods

Monocentric and retrospective study of twenty-five patients with FD (13 families; seven males and 18 females). The patients underwent audiological assessment before initiation and during enzyme replacement therapy. We also analysed neurologic heart and kidney status.

Results

Twenty patients (80%; 13 females and seven males) complained of otologic symptoms. Audiological evaluation showed a sensorineural hearing loss in 17 patients, bilateral in 16 out of them. Vestibular examination showed a functional impairment in two patients (one female, one male). Correlations were found between hearing loss and either kidney disease (73,3%), neurological complications (100%) and cardiomyopathy (80%). Fourteen patients (56%; seven females, seven males) received enzyme replacement therapy. Improvement or stabilization of the audiological evaluation was reported in seven patients, whereas worsening was observed in three patients.

Conclusion

This study confirms the high frequency of audiological involvements in females and males with FD. Our analysis suggests that the frequency of hearing loss is increased in the presence of renal or neurologic involvement or cardiomyopathy. There is no clinically significant efficacy of enzyme replacement therapy on hearing function. Although the pathophysiology remains unknown, a vascular mechanism responsible of the inner ear involvement seems to be privileged.     

Le degré de l’insuffisance rénale chronique est associé aux taux de cytokines pro-inflammatoires, à l’hyperhomocystéinémie et au stress oxydant

Aim

To evaluate pro-inflammatory cytokines, homocysteinemia and markers of oxidative status in the course of chronic renal failure.

Patients and methods

One hundred and two patients (male/female: 38/64; age: 45 ± 07 years) with chronic renal failure were divided into 4 groups according to the National Kidney Foundation classification. They included 28 primary stage renal failure patients, 28 moderate stage renal failure, 28 severe stage renal failure and 18 end stage renal failure. The inflammatory status was evaluated by the determination of pro-inflammatory cytokines (tumor necrosis factor-α, interleukin-1β, interleukin-6) and total homocysteine. Pro-oxidant status was assessed by assaying thiobarbituric acid reactive substances, hydroperoxides, and protein carbonyls. Antioxidant defence was performed by analysis of superoxide dismutase, catalase, glutathione peroxidase, glutathione reductase.

Results

Inflammatory markers were elevated in the end stage renal failure group compared to the other groups (P < 0.001). Indeed, an increase in thiobarbituric acid reactive substances, hydroperoxides and protein carbonyls was noted in the end stage renal failure group in comparison with the other groups (P < 0.001), while the levels of antioxidants enzymes activity were decreased in the study population (P < 0.001).

Conclusion

Impaired renal function is closely associated with the elevation of inflammatory markers leading to both increased markers of oxidative stress and decreased antioxidant defense.     

Réponse prolongée après traitement par tocilizumab d’une polychondrite atrophiante

Introduction

Relapsing polychondritis (RP) is a rare inflammatory disease characterized by diffuse cartilage involvement, especially those of the respiratory tract, leading to potentially life threatening complications. Corticosteroids remain the first-line empirical therapy. Immunosuppressive drugs such as azathioprine, cyclophosphamide and tumor necrosis factor blockers (anti-TNFα) are commonly used as second-line therapy with varying degrees of success.

Case report

We report a 40-year-old man with severe RP for whom conventional therapy and immunosuppressive treatments were ineffective. Prolonged clinical remission was obtained after introduction of the anti-interleukin-6 receptor antibody (tocilizumab), which was perfectly tolerated and allowed to taper steroids and methotrexate to a very low dosage.

Conclusion

Our patient is the fifth published one documenting the efficacy of tocilizumab in severe refractory RP, which strengthens the use of anti-IL-6 in that indication.     

Déficit en cuivre et neuropathie périphérique compliquant une gastrectomie

Introduction

Bariatric surgery may be indicated in patients with morbid obesity. Peripheral and central neurological complications can occur after bariatric surgery.

Observation

We report a 47-year-old woman who presented with a tetraparesis related to copper deficiency after sleeve gastrectomy for morbid obesity.

Conclusion

Neurological complications related to copper deficiency can occur after bariatric surgery.     

Manifestations cardiaques au cours la granulomatose de Wegener : à propos de quatre observations et revue de la littérature

Introduction

Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegener's granulomatosis.

Case reports

Data from four patients were studied retrospectively. Three patients had associated airway localization and three had kidney involvement. All patients had positive test for anti-PR3 antineutrophil antibodies. Two patients presented with dilated cardiomyopathy (one with terminal cardiac failure), another patient with complete atrioventricular block and pericarditis, and the remaining one with myopericarditis. One patient was asymptomatic. For three of these patients, the cardiac manifestations were contemporary of the diagnosis of Wegener's granulomatosis and had a severe disease course.

Conclusion

Cardiac events in Wegener's granulomatosis are probably underestimated, given the various type of heart damage and the clinical presentation. Cardiac involvement seems to be associated with a poor prognosis. Thus, we recommend systematic and regular cardiac assessment in the follow-up of patients with Wegener's granulomatosis.     

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.     

Polyradiculonévrite aiguë révélant un lupus érythémateux systémique : une présentation inhabituelle d’évolution fatale

Introduction

Neurological manifestations of systemic lupus erythematosus are common and numerous. They mainly involve the central nervous system, peripheral involvement being rare. Acute polyradiculoneuropathy is very uncommon.

Case report

We report a 44-year-old man, who presented with acute polyradiculoneuropathy revealing systemic lupus erythematosus. Outcome was fatal despite treatment with corticosteroids and immunoglobulin.

Conclusion

Acute polyradiculoneuropathy is a very rare manifestation of systemic lupus erythematosus and can compromise functional and life prognosis. Early diagnosis and management are crucial.     

Gastroentérite à éosinophiles

Purpose

Eosinophilic gastroenteritis is a rare and heterogeneous disorder characterized by eosinophilic infiltration of one or more layers of the gastrointestinal tract. Although it can involve any part of the gastrointestinal tract, the stomach and the proximal small bowel are the most common sites of involvement. Clinical features depend on which layer and site are involved. We report eight cases of eosinophilic gastroenteritis.

Methods

We conducted a retrospective review of consecutive adult cases diagnosed with eosinophilic gastroenteritis from 1990 to 2010. The diagnosis was established by histologic examination of endoscopic biopsy or operative specimen or by the presence of eosinophilic ascites.

Results

Eight patients (three men, five women) were diagnosed with eosinophilic gastroenteritis during the study period. Three out of the eight patients had a history of allergy. All patients had gastrointestinal symptoms. The most common symptoms were abdominal pain, vomiting, weight loss and ascites. Seven patients (87.5%) had hypereosinophilia. Seven patients had involvement of the subserosa and one of the mucosa. Four patients were treated with oral prednisolone. The symptoms in all the patients subsided within one month. The remaining four patients improved spontaneously. Four of our patients were followed-up for at least 2 months (11 to 68 months). A single patient presented a relapse.

Conclusion

Eosinophilic gastroenteritis should be suspected in patients having gastrointestinal discomfort along with peripheral eosinophilia. Definitive diagnosis requires histological demonstrations of eosinophilic infiltration of the gastrointestinal wall or high eosinophilic count in ascites fluid.     

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.     

Diabetes complications and adverse health outcomes after coronary revascularization

Aims

To examine effects of diabetes complications on health outcomes following coronary artery bypass graft (CABG) and percutaneous coronary intervention (PCI), comparing outcomes for patients with diabetes complications to those without diabetes complications.

Methods

Retrospective analysis of discharge data for 61,566 patients with diabetes age 45 or older who had CABG or PCI in 2007 in United States community hospitals, using data from the Nationwide Inpatient Sample. Analysis included propensity score-adjusted logistic regression.

Results

Of all patients, 21.2% of the weighted sample had diabetes complications. Older patients, Blacks and Hispanics, and those with greater illness severity were more likely to have diabetes complications. Unadjusted rates of in-hospital mortality, postoperative stroke, and renal failure were higher for patients with diabetes complications (rate ratios 2.2, 1.8, and 9.8, respectively; all p < 0.0001). In adjusted results, having diabetes complications was associated with higher odds of in-hospital mortality (odds ratio, OR 1.62, 95% confidence interval, CI 1.37–1.91) and renal failure (OR 3.03, CI 1.71–5.39). Compared to CABG, PCI was associated with extra risk of postoperative renal failure for those with diabetes complications.

Conclusion

Among patients with diabetes having revascularization, those with diabetes complications have higher risks of in-hospital death and renal failure irrespective of having CABG or PCI.     

Association d’une hypertension artérielle rénovasculaire et d’un hyperaldostéronisme primaire

Introduction

Renal artery stenosis is rarely associated with Conn adenoma.

Case report

We report a 27-year-old male patient who presented in 2001 with a severe high blood pressure associated with hypokaliemia. Radiologic investigations showed a left renal artery stenosis with agenesis of left kidney. A left nephrectomy was performed and blood pressure returned to normal with a single antihypertensive drug. Five years later, the patient again presented with severe high blood pressure. Laboratory studies revealed a low serum potassium level at 2.8 mmol/L associated with high urinary potassium excretion (84 mmol/24 h) and a very high aldosterone/renin ratio (> 462). Abdominal CT scan demonstrated a right adrenal mass. The patient underwent a right adrenalectomy (adenoma). Blood pressure returned to normal with a single antihypertensive. Serum potassium levels as well as aldosterone/renin ratio normalized.

Conclusion

We discussed whether the association between these two entities is merely fortuitous or conversely based on a causal relationship.     

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.     

Analyse des complications cardiovasculaires survenant lors des transferts inter-hospitaliers de patients ayant un syndrome coronarien sans élévation du segment ST non compliqué

Introduction

Managing patients with non-ST elevation myocardial infarction (NSTEMI) require their transfer to an interventional cardiology center. The inter-hospital transfer (IHT) of these patients is managed by the Emergency Medical Service (EMS) which often requests a medicalised transport of such patients due to the risk of complications.

Aim of the study

Evaluating the incidence of cardiovascular complications that require medical intervention, occurring during IHT of patients with uncomplicated NSTEMI.

Methods

We conducted a retrospective study for one year (August 2010–July 2011) in the SAMU-85 unit using the regulation software Centaure^®. All patients with NSTEMI who had been transferred from one of the peripheral hospitals in the region of Vendee were included.

Results

The study population group is composed of one hundred and fifty-nine patients. No cardiovascular complications requiring medical intervention occurred during the transport. Seventeen patients (10.7%) had cardiovascular complications: repolarization disorder (7), chest pain (3), dyspnea (3), arrhythmia (2), and bradycardia (2). These complications did not require action or medical intervention.

Conclusion

The medicalization of IHT of patients with NSTEMI who do not show complications before transfer, is probably questionable. Indeed, according to this study made on 159 patients, no cardiovascular event requiring medical intervention was reported during transport.     

Nécrose médullaire avec sepsis compliquant une thrombocytémie essentielle : à propos d’une observation et revue de la littérature

Introduction

Bone marrow necrosis is a very rare condition which is characterized by a necrosis of hematopoietic progenitors, adipocytes and reticulin network.

Case report

We report a 62-year-old woman admitted to an intensive care unit for an essential thrombocytemia associated with bone marrow necrosis complicated by septic shock and progressive multi-organ failure. To our knowledge, this is the second case reported in the literature. The clinical presentation of bone marrow necrosis includes non-specific symptoms such as fever, bone pain and sometimes a clinically significant medullar insufficiency syndrome. Biology can reveal cytopenias, elevated LDH and alkaline phosphatase serum levels. The diagnosis is confirmed by bone marrow trephine biopsy. Bone marrow necrosis is classified as extensive if more than 50% of the bone marrow biopsy show necrosis. Haematological malignancies (particularly leukaemia), and solid malignant tumours (particularly gastro-intestinal or lung cancers) represent up to 90% of aetiologies and must be actively researched. Also, sickle cell disease and catastrophic anti-phospholipid syndrome must also be investigated. Essential thrombocytemia remains an exceptional cause of bone marrow necrosis.

Conclusion

Overall the prognosis of bone marrow necrosis is poor unless appropriate and intensive treatment, especially for sickle cell disease in which complete medullar regeneration has been observed.     

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.     

Hépatite cholestatique sévère révélant un Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome

Introduction

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a drug-induced hypersensitivity syndrome, characterized by rash, hyereosinophilia and multiorgan failure, including cytolytic hepatitis.

Case report

A 75-year-old man, treated with amoxicillin/clavulanic acid, presented with jaundice and disabling pruritus associated with severe cholestatic hepatitis, related to a DRESS syndrome. Because of the persistence of cholestasis and the severity of pruritus, a treatment with corticosteroids and plasma exchanges was initiated, allowing a rapid and complete remission.

Conclusion

Amoxicillin/clavulanic acid, although rarely described in the literature, is a rare cause of DRESS syndrome. Severe cholestatic hepatitis associated with disabling pruritus may be one of the systemic manifestations, with a good prognosis using corticosteroids and plasma exchanges.     

Mort subite au cours d’une dégénérescence cérébelleuse subaiguë paranéoplasique avec dysautonomie associée aux anticorps anti-Hu révélant un cancer bronchique à petites cellules

Introduction

Paraneoplastic syndromes are a rare cancer complication with a frequent subacute evolution.

Observation

A 62-year-old man was admitted presenting with a cerebellar syndrome and orthostatic hypotension with dysautonomia. Anti-Hu antibody research was positive. A subcarinal adenopathy biopsy found out a small cell lung carcinoma. Despite a treatment with immunoglobulin and chemotherapy, the patient died suddenly, after a raise of dysautonomia symptoms.

Conclusion

Sudden death observations represent exceptional complications of paraneoplastic syndrome. They might be secondary to arrhythmias, ictal asystol or laryngospasm. Systematic research of paroxystic heart arrhythmias with holter-ECG in paraneoplastic syndrome may prevent sudden deaths.     

Myopathie nécrosante auto-immune associée aux anticorps anti-hydroxy-méthyl-glutaryl-coenzyme A réductase

Introduction

Statins or 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase inhibitors (HMGCR) are among the most commonly prescribed treatment in France. They may be responsible for muscular intolerance with variable severity. They have been recently involved in the occurrence of an acquired inflammatory myopathy associated with anti-HMGCR antibodies. This new type of toxic myopathy remains poorly known by clinicians.

Observation

We report a 61-year-old woman treated with a statin for many years who developed a lower and upper limb disabling myopathy with a rapid unfavourable course despite treatment withdrawal. Clinical history and investigations, especially including an assay for anti-HMGCR antibodies led to the diagnosis of autoimmune necrotizing myopathy with anti-HMGCR antibodies. Subsequent initiation of an immunosuppressive treatment by corticosteroids and methotrexate was effective.

Conclusion

Statins may unmask or cause an autoimmune necrotizing myopathy associated with the presence of anti-HMGCR antibodies. Their identification is now routinely available. An immunosuppressive treatment is necessary and justified by the autoimmune nature of the disease.     

Infection disséminée au virus varicelle-zona avec gastrite hémorragique compliquant une leucémie lymphoïde chronique : une observation et revue de la littérature

Introduction

The reactivation of varicella-zoster virus occurs in immunocompromised patients, especially in cases of hematological malignancy. Disseminated reactivation could involve digestive tract with life-threatening condition.

Case report

A 76-year-old woman, with a history of chronic lymphocytic leukemia, presented with left hypochondrium pain, and a vesicular rash with hemorrhagic shock that revealed an hemorrhagic gastritis due to varicella-zoster virus. The literature review identified 28 additional cases of gastrointestinal mucosal damage during reactivation of varicella-zoster virus. Mortality is 40%. We report here the first case in the course of low-grade lymphoid malignancy.

Conclusion

Acute gastrointestinal symptoms in immunocompromised patients should evoke a varicella-zoster virus reactivation with gastrointestinal involvement. This clinical manifestation, although rare, should not be ignored because of its severity.