RESUMEN
The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren's syndrome and Hashimoto's thyroiditis.
Asunto(s)
Enfermedad de Hashimoto/complicaciones , Cirrosis Hepática Biliar/complicaciones , Poliangitis Microscópica/complicaciones , Síndrome de Sjögren/complicaciones , Anticuerpos Antinucleares/sangre , Biomarcadores/sangre , Biopsia , Femenino , Enfermedad de Hashimoto/sangre , Enfermedad de Hashimoto/diagnóstico , Enfermedad de Hashimoto/tratamiento farmacológico , Enfermedad de Hashimoto/inmunología , Humanos , Inmunosupresores/uso terapéutico , Cirrosis Hepática Biliar/sangre , Cirrosis Hepática Biliar/diagnóstico , Cirrosis Hepática Biliar/tratamiento farmacológico , Cirrosis Hepática Biliar/inmunología , Poliangitis Microscópica/sangre , Poliangitis Microscópica/diagnóstico , Poliangitis Microscópica/tratamiento farmacológico , Poliangitis Microscópica/inmunología , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Insuficiencia Renal/etiología , Síndrome de Sjögren/sangre , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/tratamiento farmacológico , Síndrome de Sjögren/inmunología , Esteroides/uso terapéutico , Resultado del TratamientoRESUMEN
Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy.
Asunto(s)
Amiloidosis/complicaciones , Síndrome de Behçet/complicaciones , Síndrome Nefrótico/etiología , Adulto , Amiloidosis/tratamiento farmacológico , Síndrome de Behçet/tratamiento farmacológico , Resultado Fatal , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.
Asunto(s)
Sistema Nervioso Central/patología , Poliangitis Microscópica/patología , Sistema Nervioso Periférico/patología , Potenciales de Acción/fisiología , Antiinflamatorios/uso terapéutico , Biopsia , Ciclofosfamida/uso terapéutico , Electromiografía , Humanos , Inmunosupresores/uso terapéutico , Hemorragias Intracraneales/complicaciones , Hemorragias Intracraneales/patología , Masculino , Poliangitis Microscópica/complicaciones , Persona de Mediana Edad , Mononeuropatías/complicaciones , Mononeuropatías/patología , Músculo Esquelético/inervación , Músculo Esquelético/patología , Miositis/complicaciones , Miositis/patología , Nervio Peroneo/patología , Prednisona/uso terapéutico , Recuperación de la Función , Tomografía Computarizada por Rayos X , Vasculitis del Sistema Nervioso Central/complicaciones , Vasculitis del Sistema Nervioso Central/patologíaRESUMEN
INTRODUCTION: Langerhans cell histiocytosis of the thoracic spine is a rare condition in adults. It is a serious condition with an increased risk of spinal cord compression. EXEGESIS: A 55-year-old man complained of lumbar and back pain. He developed a spastic paraparesis. Magnetic resonance imaging of the spine showed a spinal cord compression due to bone and epidural tumoral lesions of the eleventh and twelfth thoracic vertebrae, located in the posterior elements of the spine. A surgical biopsy showed an infiltrate of eosinophilic cells, positive for CD1a in immunohistochemistry studies. Final diagnosis was therefore langerhans cell histiocytosis of the thoracic spine complicated by spinal cord compression. The patient received glucocorticoid therapy combined with vinblastine followed by local radiotherapy and etoposide. However, the neurological deficit persisted and the patient finally died. CONCLUSION: Langerhans cell histiocytosis is a rare cause of spinal cord compression.