RESUMO
The macrophage activation syndrome (MAS) i a rare pediatric condition characterized by enhanced activation of the macrophage and T-cell system, with increased liberation of T-cell cytokines. Clinically it resembles a multiorgan failure syndrome of infectious etiology. There is still some debate as to the nomenclature of this syndrome, hemato-oncologists usually classify this syndrome as a histiocytic disorder, while rheumatologists define it as MAS. We review a patient in which MAS was diagnosed during the early stages of the disease. The patient had juvenile idiopathic arthritis (systemic presentation).
Assuntos
Masculino , Adolescente , Humanos , Artrite Juvenil/complicações , Artrite Juvenil/metabolismo , Ativação de Macrófagos , SíndromeRESUMO
Background: The diagnostic profile of patients with rheumatic diseases admitted to a general hospital is variable. Aim: To report the epidemiological profile of patients with rheumatic diseases admitted to a tertiary care hospital. Material and methods: All admissions to a Medicine ward of a general hospital and seen by the Rheumatology team were prospectively registered during one year in 1999. Patients were classified as primarily admitted for a rheumatic disease or admitted for other cause that required a consultation with the Rheumatology team. Results: One hundred forty five admissions due to rheumatic diseases were registered. Of these, 82 were due to primary rheumatic diseases. Systemic lupus erythematosus, rheumatoid arthritis and vasculitis were the main diagnoses and the mean hospital stay was 18.5 days. Sixty three patients required a consultation with the Rheumatology team specially due to osteoarthritis and crystal induced diseases. Conclusions: Admissions due to rheumatic diseases are prolonged, correspond to 0.46 per cent of all admissions and the main responsible disease is systemic lupus erythematosus
Assuntos
Humanos , Feminino , Masculino , Adolescente , Adulto , Pessoa de Meia-Idade , Artrite Reumatoide/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/epidemiologia , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/terapia , /estatística & dados numéricos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Tempo de Internação/estatística & dados numéricosRESUMO
Nocardia asteroides infection are unusually observed in sistemic Lupus erithematous (SLE) patients. They are generally associated to steroidal and immunosuppressive therapy. We report a 24 years old female with SLE diagnosed in 1994 who developed a severe preeclampsia in her first pregnancy requiring emergency caesarean section. Post partum acute renal failure and type IV lupus nephropathy were treated with hemodialysis, metilprednisolone, cyclophosphamide and prednisone. Three months later, while she was receving the fourth cyclophosphamide dose, she presented with a pleuro pneumonia and occipital abscess, both caused by Nocardia asteroides. She was treated with cotrimoxazole + cefixime and pleural decortication was required. Five months later, she developed Meningitis caused by Nocardia asteroides and hydrocephalus. She was treated with ceftriaxone, vancomycin, cotrimoxazole and ventricular shunting procedure. Two months later, a retroperitoneal abscess was diagosed and surgically drained but the patient died, due to a methicillin-resistant Staphylococcus aureus septicemia
Assuntos
Humanos , Feminino , Adulto , Nocardia asteroides/patogenicidade , Nocardiose/complicações , Lúpus Eritematoso Sistêmico/complicações , Nocardiose/tratamento farmacológico , Combinação Trimetoprima e Sulfametoxazol/administração & dosagem , Hospitalização/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricosRESUMO
The clinical picture of primary systemic vasculitis, that cause inflammation and necrosis of vessel walls, depend on the type, size and location of involved vessels. Frequently, their clinical presentation does not reflect the specific type of vasculitis and its prognosis. Moreover, the correct diagnosis, early and adequate treatment have an important prognostic value. Recently, new diagnostic methods for systemic vasculitis have been devised. This article critically reviews and provides information for the rational use of these new imaging techniques and laboratory procedures for the diagnosis and follow up of systemic vasculitis