RESUMO
We describe a 47-year-old woman with severe aplastic anemia with genital bleeding who developed acute severe hepatitis after the administration of danazol while she was receiving cyclosporin. She had been diagnosed with severe aplastic anemia 1 year previously and, while hospitalized, had received methyl prednisolone pulse therapy, which was not successful. She was then referred to our hospital. She was treated with antithymocyte globulin, cyclosporin, granulocyte colony-stimulating factor, and methyl prednisolone; a good response was achieved after 3 months of this therapy. Subsequently, oral administration of cyclosporin was continued, but she was readmitted to our hospital when pancytopenia gradually developed and the genital bleeding recurred. Danazol was administered for pancytopenia and endometriosis. Four days after the first administration of danazol, epigastric pain occurred, and the danazol was stopped. Eighteen days after the first danazol administration, very severe hepatic injury occurred abruptly. The patient died of hepatic failure. Postmortem examination revealed centrilobular massive necrosis of the liver. Danazol was implicated as the agent responsible for causing the hepatic failure. Drug interactions between danazol and cyclosporin may cause adverse effects.
Assuntos
Anemia Aplástica/complicações , Danazol/efeitos adversos , Endometriose/tratamento farmacológico , Antagonistas de Estrogênios/efeitos adversos , Falência Hepática Aguda/induzido quimicamente , Anemia Aplástica/tratamento farmacológico , Ciclosporina/uso terapêutico , Danazol/uso terapêutico , Endometriose/complicações , Antagonistas de Estrogênios/uso terapêutico , Evolução Fatal , Feminino , Humanos , Imunossupressores/uso terapêutico , Falência Hepática Aguda/complicações , Falência Hepática Aguda/patologia , Pessoa de Meia-IdadeRESUMO
On November, 1997, a 15-year-old boy visited our hospital because of headache, fever and arthralgia. He was treated with 5 mg/day of prednisolone thereafter. On October 21, 1998, he was admitted because of remittent fever and multiple arthralgia and diagnosis of juvenile rheumatoid arthritis (JRA) was made. He was also found to have hypertension of 210/110 mmHg, and soon developed ptosis of the eye, facial paresis and perceptive deafness of the right side. Cerebrospinal fluid showed protein of 98 mg/dl and mildly elevated IgG, IgA and IgM levels with normal cell count. Brain MRI examination revealed multiple cerebral lesions in the frontal, parietal and cerebellar areas on the right, whose cause was thought to be vasculitis. Renal angiography demonstrated a right renal artery stenosis, compatible with renovascular hypertension. He was treated with 60 mg of prednisolone per day, which brought about a satisfactory improvement of the above rheumatic and neurologic signs. On November 17, 1998, he received a follow-up study of MRI, which failed to show any cerebral lesions, supporting the effectiveness of prednisolone. An angiotensin converting enzyme inhibitor successfully normalized hypertension and renin activity in serum, although renal blood flow did not increase.
Assuntos
Artrite Juvenil/complicações , Hipertensão Renovascular/etiologia , Vasculite do Sistema Nervoso Central/etiologia , Adolescente , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Artrite Juvenil/diagnóstico , Artrite Juvenil/tratamento farmacológico , Humanos , Hipertensão Renovascular/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Prednisolona/administração & dosagem , Resultado do Tratamento , Vasculite do Sistema Nervoso Central/diagnóstico , Vasculite do Sistema Nervoso Central/tratamento farmacológicoRESUMO
A case of intestinal perforation associated with SLE is presented. A 54-year-old woman was diagnosed as having SLE twenty-five years ago when she had facial erythema, photosensitivity, oral aphtha, polyarthraliga, leukopenia, positive LE cell and positive antinuclear antibody. She had been treated with prednisolone and admitted to Kushiro City General Hospital because of one month history of fever and anorexia in February 1996. Laboratory findings did not reveal activity of SLE, and a diagnosis of urinary tract infection was made based on the findings of urinalysis. After severe diarrhea, disseminated intravascular coagulation (DIC) developed. A rectal perforation was revealed by endoscopic and radiological examination. An emergency laparotomy revealed necrosis of the rectum and sigmoidostomy was performed. The biopsied specimen of the rectum were diagnosed as gangrene of ischemic colitis histologically. Because of a penetration to the urinary bladder, an ureterocutaneostomy was performed. She died of sepsis and DIC on the 127th day of admission. Only 11 cases of intestinal perforation associated with SLE have been reported in Japan, and the association of vasculitis has been considered. In the present case, the prolonged use of prednisolone might cause the necrotizing ischemic colitis.
Assuntos
Perfuração Intestinal/etiologia , Lúpus Eritematoso Sistêmico/complicações , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
A rare SLE patient with central nervous system involvement (CNS-SLE) who relapsed presenting new symptoms associated with the development of serum anti-Sm antibody and was then successfully treated with cyclophosphamide (CY) pulse therapy is presented here. A 47-years old housewife was admitted to Kushiro City General Hospital because of fever, limb erythema and drowsy consciousness in September 1995. On the basis of convulsion, proteinuria, leukopenia, thrombopenia, serum positive tests for both anti-nuclear antibody and anti-SSA antibody and low complement levels, as well as elevations of IgG index and IL-6 in the cerebrospinal fluid (CSF), she was diagnosed as having CNS-SLE. Serum tests for anti CL-beta 2 GPI antibody and lupus anticoaglant was negative. Serum test for HBs antigen was positive. She was treated successfully with methylprednisolone (mPSL) pulse therapy and plasma exchange (PE). Prednisolone was gradually tapered to the dosage of 17.5 mg per day and she was discharged in April 1996. She was re-admitted because of fever, an exacerbation of skin eruption and arthralgia in October 1996. Serum anti-Sm antibody was found to be positive. mPSL pulse therapy was not effective. On the basis of hallucination and elevations of IgG index and IL-6 in the CSF, a diagnosis of relapsed CNS-SLE was made. However the level of IFN-alpha in the CSF was normal. Although PE was not effective, CY pulse therapy was markedly effective.
Assuntos
Autoanticorpos/sangue , Autoantígenos/imunologia , Doenças do Sistema Nervoso Central/etiologia , Lúpus Eritematoso Sistêmico/complicações , Ribonucleoproteínas Nucleares Pequenas , Ciclofosfamida/administração & dosagem , Esquema de Medicação , Feminino , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/imunologia , Pessoa de Meia-Idade , Proteínas Centrais de snRNPRESUMO
A 71-year-old man had noticed a dry sensation in the mouth with swelling of bilateral parotid glands in 1988. He was given a diagnosis of Sjögren's syndrome (SS) on the basis of characteristic findings of sialography and a minor salivary gland biopsy. He was admitted to our department in Febuary 1995 because of general fatigue of 2 month's duration. Laboratory data showed both positive anti-SSA and anti-SSB antibodies, liver dysfunction, hypoalbuminemia, and thrombocytopenia. Abdominal CT and MRI demonstrated a 2-cm intrahepatic mass (S 6) with enhanced areas at the periphery. The liver biopsy yielded fragments from the intrahepatic mass and hepatic parenchyma. The former was composed of plasma cells, lymphocytes, and histiocytes, compatible with the diagnosis of inflammatory pseudotumor of the liver. The pathological diagnosis of the latter specimen was primary biliary cirrhosis, although antimitochondrial antibody was negative. The intrahepatic mass gradually decreased in size without treatment. Inflammatory pseudotumor is considered to be a benign inflammatory condition simulating a neoplasma and the possibility of an autoimmune reaction is suggested on the basis of etiology. This is the first report of an inflammatory pseudotumor associated with Sjögren's syndrome developing in the liver. The inflammatory pseudotumor should be considered as a possible diagnosis in cases where the tumor is embedded in the liver.