RESUMEN
The combination of Goodpasture's disease and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is not exceptional. However, to the best of our knowledge, their association with multiple myeloma (MM) with kidney involvemen thas not been described. We report probably the first case of double-seropositive vasculitis for antiglomerular basement membrane (GBM) antibodies and ANCA associated with MM with renal involvement. A 60- year-old-female presented with severe acute kidney injury. Three months earlier, she had a history of bone pain. Blood workup found a creatinine of 1100 µmol/L and a C-reactive protein of 60 mg/L. Anti-GBM antibodies returned positive at 400 U/mL and pANCA positive at 380 U/mL. Plasma protein immunofixation found a monoclonal immunoglobulin G (IgG) KAPPA peak; the myelogram found a 10% plasmocytosis. On the day 4 of hospitalization, the patient presented with alveolar hemorrhage. The renal biopsy showed diffuse crescentic glomerulo-nephritis with linear glomerular deposits of IgG, with kappa light chain cast nephropathy. The association between vasculitis and malignant blood disease is very rare; the pathophysiology of this association remains unclear. It would seem interesting to look for possible ANCA or anti- GBM activity carried by the monoclonal immunoglobulin.
Asunto(s)
Lesión Renal Aguda/inmunología , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/inmunología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Autoanticuerpos/sangre , Inmunoglobulina G/sangre , Cadenas kappa de Inmunoglobulina/sangre , Riñón/inmunología , Mieloma Múltiple/inmunología , Lesión Renal Aguda/sangre , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/terapia , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/sangre , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/terapia , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/sangre , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/terapia , Biomarcadores/sangre , Femenino , Humanos , Riñón/patología , Persona de Mediana Edad , Mieloma Múltiple/sangre , Mieloma Múltiple/diagnóstico , Mieloma Múltiple/terapia , PronósticoRESUMEN
Calcific uremic arteriolopathy, also called calciphylaxis, is a rare and severe disorder that presents with skin ischemia and necrosis, sometimes it presents with systemic necrosis, the process is secondary to the obliteration of the arterioles first by sub-intimal calcium deposits and then by thrombosis. These lesions can often lead to death due to infectious complications and comorbidities such as diabetes, obesity, arteritis, diffuse vascular calcifications, heart disease and undernutrition. The diagnosis is suggested by the characteristic ischemic skin lesions and their distribution, often bilateral and painful, associeted with calcific uremic arteriolopathy risk factors (phosphocalcic abnormalities, anti-vitamin K). The presence of radiological vascular calcifications is highly suggesting the diagnosis, but remains not very specific. The indication of skin biopsy is rare and reserved for difficult diagnoses. The goals of treatment are: reduce the extension of calcification and treatment of mineral and bone metabolism disorders of end-stage renal disease, dialysis adequacy, local treatment of skin lesions, tissue oxygenation, pain management, discontinuation and contraindication of medications that may contribute to the disorder. We propose to discuss it from a review of the literature and illustrate it with five clinical cases.
Asunto(s)
Calcifilaxia/diagnóstico , Diálisis Renal/efectos adversos , Calcificación Vascular/etiología , Anciano , Arteriolas/patología , Calcifilaxia/terapia , Diagnóstico Diferencial , Humanos , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Piel/patologíaRESUMEN
Hemodialysis (HD) safety has become a clinical priority; therefore, the use of checklists for making the dialysis session safe is now widely adopted. The aim of our study was to assess different shortcomings in the clinical practice of nurses working in different Moroccan dialysis centers and to discuss the interest of using such checklists. This cross-sectional study was performed in 13 chronic HD centers. Clinical practice of nurses was evaluated through checklists used in European outpatient dialysis units. We noted several deficiencies mainly related to the clinical evaluation of dialysis patients and to aspects related to hygiene and protection measures against contamination. Optimal safety of dialysis sessions requires the use of simple and reproducible means that improve clinical skills of the health staff.