Fibrose rétropéritonéale idiopathique : évolution à long terme du pronostic rénal dans une série rétrospective multicentrique de 30 cas

Purpose

Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory disorder, affecting the aorta and the surrounding vessels and tissues. The prognosis is mainly driven by the risks of chronic kidney disease and relapse. Our aim was to assess the prevalence of chronic kidney disease at follow-up.

Methods

We retrospectively reviewed the medical records of patients diagnosed for IRF in Seine-Saint-Denis (France) between 1987 and 2011. We collected informations about presentation, radiologic findings and follow-up. Diagnosis of IRF was confirmed when all the following criteria were met: infiltration of the infrarenal aorta or iliac vessels, absence of aneurysmal dilation, lack of clinical suspicion of malignancy.

Results

Thirty patients were identified, with a male/female ratio of 4.9. Mean age was 55 ± 13 years old. The mean creatinine clearance was 66 mL/min/1.73 m² and the mean CRP was 45 ± 36 mg/L. In 24 (80%) patients, the location of IRF was periaortic and periiliac. Eleven patients (37%) underwent a diagnostic biopsy, and 14 (47%) required an ureteral procedure. A mean follow-up of 63 months was available for 29 patients: 69% relapsed, 7 developed chronic renal disease (24%), and one died of urinary sepsis. Older age (P = 0.023), diabetes (P = 0.007), and initial renal insufficiency (P = 0.05) were associated with a risk of chronic renal insufficiency.

Conclusion

The high frequency of relapses and chronic renal disease emphasizes the need of close follow-up in patients diagnosed with IRF.     

Amylose systémique découverte par une atteinte trachéobronchique

Amyloidosis is a spectrum of disease characterized by the abnormal deposition of fibril amyloid-related proteins in the extracellular space. The most common types of amyloidosis are AL and AA amyloidosis. Amyloidosis is also classified according to the extent of the deposition as systemic or localized. Respiratory amyloidosis is classified as laryngeal, tracheobronchial or parenchymal amyloidosis. Tracheobronchial amyloidosis is classically known as a rare manifestation of the localized disease. A few cases only of systemic amyloidosis presenting as a tracheobronchial disease have been reported. We here report a 26-year-old man who presented with hemoptysis related to a systemic tracheal primary amyloidosis.     

Narcolepsie révélant un lupus érythémateux systémique

Many neurologic and psychiatric manifestations have been associated with systemic lupus erythematosus. Narcolepsy, currently hypothesized as related to an autoimmune process, has been rarely associated with systemic lupus erythematosus. We report a 36-year-old woman who presented with narcolepsy and who subsequently developed systemic lupus erythematosus. Excessive daytime sleepiness resolved after the administration of four intravenous bolus of cyclophosphamide and methylprednisolone followed by maintenance therapy with hydroxychloroquine, aspirine and prednisone. Narcolepsy should be included in the neuropsychiatric manifestations of systemic lupus erythematosus and it may have a parallel clinical course to the activity of the lupus.     

La pyomyosite : une complication infectieuse du lupus érythémateux systémique

Pyomyositis is a primary pyogenic muscular infection. It has been originally described in the tropics. Presenting signs and symptoms are not specific. It is frequently misdiagnosed in the early stages. We report the case of a 47-year-old woman with systemic lupus erythematosus treated with corticosteroids who presented a thigh pyomyositis evidenced by echography and MRI. Outcome was uneventful after antibiotics and surgical drainage. Pyomyositis is increasingly described among immunocompromised people, including lupus patients treated with corticosteroids.     

Le risque cardiovasculaire au cours du lupus systémique

Multiple factors contribute to the increased cardiovascular risk observed in patients with systemic lupus erythematosus (SLE). Among these are the so-called classical cardiovascular risk factors, the disease itself through its activity, treatments, and complications, and the thrombotic risk due to antiphospholipid antibodies (aPL). Observational studies suggest that most classical cardiovascular risk factors are observed more frequently in SLE patients than in the general population, and that these are insufficient to explain the increased cardiovascular risk observed in most studies. Given this high risk, adequate management of cardiovascular risk factors should be recommended in SLE patients. Paradoxically, the benefit due to the anti-inflammatory properties of treatments such as corticosteroids may exceed, in certain cases, their pro-atherogenic effect. Importantly, the tools that were developed for the estimation of cardiovascular risk at the individual level among the general population cannot be used reliably in SLE patients, as these tools appear to underestimate the true cardiovascular risk. The adequate indications and targets of cardiovascular treatments are therefore not fully known in SLE. A better understanding of the determinants of the cardiovascular risk in SLE will allow the identification and more tailored management of these high-risk patients.     

Pseudo-obstruction intestinale au cours du lupus érythémateux systémique

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.     

Une atteinte du système nerveux central au cours de la sclérodermie systémique

Systemic sclerosis is a connective disease usually known to spare the central nervous system. This has been much debated by recent imaging studies. We report a 56-year-old woman followed-up for systemic sclerosis since 2005. Four years later, she presented with cerebellar and pyramidal syndrome. Magnetic resonance imaging showed signs of cerebral vasculitis. The patient was treated by corticosteroids and cyclophosphamide pulses followed by azathioprine for maintenance therapy. Clinical and radiological outcomes were favourable. In patients with systemic sclerosis and neurological symptoms, abnormalities in the cerebral magnetic resonance imaging may, in the absence of another obvious etiology, indicate a central nervous system involvement associated with this systemic disorder.     

Le lupus systémique à début pédiatrique : une pathologie polygénique ou monogénique ?

Systemic lupus erythematosus (SLE) results from the complex interaction between genetic and environmental factors. It is usually thought that SLE results from the combined effect of variants in a large number of genes, and several genome whole association studies (GWAS) have identified a great number of single-nucleotide polymorphisms (SNP) associated with SLE. However, the loci identified so far can account for only about 15% of the heritability of SLE. Recently, some Mendelian variants of lupus have been identified, especially in childhood-onset SLE. Children present with more severe illness, a lower sex-ratio female:male and a higher genetic contribution compared to adults with SLE. pSLE phenotype heterogeneity could be related to genetic heterogeneity, and pSLE in part might consist in a collection of rare, genetically distinct monogenic disorders.