RESUMEN
A 66-year-old male with scleroderma developed rapidly progressive glomerulonephritis (RPGN). Renal pathology revealed crescentic glomerulonephritis with interstitial inflammation and fibrosis. Immunofluorescent micrography showed linear deposition of IgG along the glomerular capillary wall. Both anti-glomerular basement membrane antibody (anti-GBM Ab), and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) were detected by an enzyme-linked immunosorbent assay (ELISA). These findings were compatible with ANCA-related vasculitis and anti-GBM Ab nephritis. Laboratory findings showed rapid elevation of the serum creatinine level (5.9 mg/dL), and a high titer of MPO-ANCA (530 EU) and anti-GBM Ab (21 EU). He was started on methylprednisolone pulse therapy and temporary hemodialysis. Since the immunosuppressive therapy lowered both antibody titers steadily and improved renal function, hemodialysis was discontinued 4 weeks after the therapy. It has been reported that some scleroderma patients developed rapid progressive glomerulonephritis due to ANCA-associated vasculitis in addition to the typical scleroderma renal crisis. There have been few reports of a scleroderma patient associated with RPGN, in whom both MPO-ANCA and anti GBM antibodies were detected.
Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/análisis , Autoanticuerpos/análisis , Membrana Basal Glomerular/inmunología , Glomerulonefritis/complicaciones , Peroxidasa/inmunología , Esclerodermia Sistémica/complicaciones , Anciano , Biomarcadores/análisis , Progresión de la Enfermedad , Glomerulonefritis/diagnóstico , Glomerulonefritis/patología , Glomerulonefritis/terapia , Humanos , Inmunoglobulina G/análisis , Masculino , Metilprednisolona/administración & dosificación , Prednisolona/administración & dosificación , Quimioterapia por Pulso , Diálisis Renal , Esclerodermia Sistémica/diagnósticoRESUMEN
A 53-year-old male was admitted to our hospital for a high fever. He suffered a change in personality, memory loss and disorientation as well. The findings of cerebrospinal fluid showed monocytosis, but the titers of glucose, C1 and ADA were all normal. Although there was no bacterium in the CSF, the patient's electroencephalography finding was abnormal. We diagnosed his condition as viral meningoencephalitis and started treatment with antiviral agents. Blood chemistry showed serum sodium of 130 mEq/l and plasma osmolarity was reduced to 272 mOsm/kg, while urine osmolarity was high at 353 mOsm/kg. Two potential causes of hyponatremia in this patient were the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) or cerebral salt wasting syndrome (CSWS). Physical findings revealed a contracted extracellular fluid volume, strongly suggesting the presence of CSWS. The massive urine sodium loss overcoming sodium intake supported this diagnosis. After treatment with vigorous sodium and volume replacement for over 4 weeks, hyponatremia as well as meningoencephalitis were improved without any complication. To the best of our knowledge, this is the first report on CSWS in a patient with viral meningoencephalitis.
Asunto(s)
Hiponatremia/etiología , Síndrome de Secreción Inadecuada de ADH/diagnóstico , Meningoencefalitis/complicaciones , Virosis/complicaciones , Diagnóstico Diferencial , Humanos , Síndrome de Secreción Inadecuada de ADH/etiología , Masculino , Persona de Mediana EdadRESUMEN
On April 25, 2003, a 62-year-old Japanese man had been admitted to a hospital because of heavy proteinuria and elevated serum creatinine level, and purpura on the lower extremities. On May 15, 2003, he was referred to our hospital for evaluation and treatment. Serum immunoglobulin and complements were within normal ranges. Immune serology was negative for antinuclear antibody, antiglomerular basement membrane antibody, and antineutrophil cytoplasmic antibodies. Histological examination of a percutaneous renal biopsy specimen revealed that all of the glomeruli had severe crescent formation without deposits of immunoreactants. A diagnosis of antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis was made. The patient was treated with one cycle of steroid pulse therapy (1000 mg methylprednisolone daily, given on 3 consecutive days), and subsequently with prednisolone (60 mg/day). Despite this treatment, renal failure progressed rapidly and hemodialysis was started 1 month after the acute presentation. On May 30, 2003, he suddenly developed massive hematochezia. A technetium-targeted red-blood-cell scan suggested bleeding in the small intestine. On June 11, he presented with massive melena. A bleeding ulcer was found in the third part of the duodenum, and was treated successfully with endoscopy, using a heater probe. On June 19, he presented with massive hematochezia again. Mesenteric angiography revealed active bleeding from the iliac branch of the superior mesenteric artery. He was treated with continuous intraarterial vasopressin infusion by a catheter seated in the branch artery. The majority of patients with pauci-immune crescentic glomerulonephritis, one of the most common causes of rapidly progressive glomerulonephritis, have glomerular disease as part of a systemic vasculitis. Massive gastrointestinal bleeding, although rare, should be considered one of the serious complications in these patients.
Asunto(s)
Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/inmunología , Glomerulonefritis/complicaciones , Glomerulonefritis/inmunología , Angiografía , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Glomerulonefritis/patología , Humanos , Glomérulos Renales/patología , Glomérulos Renales/ultraestructura , Masculino , Arterias Mesentéricas , Microscopía Electrónica , Persona de Mediana Edad , RecurrenciaRESUMEN
OBJECTIVE: We examined whether thiazide diuretics could restore nocturnal blood pressure (BP) decline and reduce urinary protein excretion in patients with glomerulopathy treated with angiotensin II modulators (angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers). METHODS: Twenty-five Japanese outpatients (11 men, 14 women; mean age 43 +/- 12 years) with biopsy-proven immunoglobulin (Ig)A nephropathy, preserved renal function (serum creatinine concentration =1.2 mg/dl), stable non-nephrotic proteinuria (0.5-3 g daily), and treatment with angiotensin II modulators were studied. The patients received a diuretic, trichlormethiazide (2 mg daily) for 4 weeks after a baseline period lasting for 4 weeks. RESULTS: Diuretic therapy significantly reduced conventional, 24-h, daytime and night-time blood pressures. Nocturnal blood pressure fall was significantly enhanced by diuretic therapy and a significant interaction existed between diuretic therapy and nocturnal fall in mean arterial pressure, which indicated that the degree of nocturnal blood pressure fall was affected by diuretic therapy. The urinary protein excretion rate was significantly reduced from 1.10 +/- 0.62 to 0.63 +/- 0.39 g/day by diuretic therapy. Diuretic/baseline ratio of urinary protein excretion rate was not correlated with diuretic/baseline ratio of conventional, 24-h and daytime mean arterial pressures, but with diuretic/baseline ratio of night-time mean arterial pressure (r = 0.54, P = 0.006). CONCLUSIONS: Diuretics enhanced nocturnal BP decline and reduced urinary protein excretion in patients with IgA nephropathy treated with angiotensin II modulators. The combination of angiotensin II modulators and diuretics may have additional therapeutic advantages in relieving the renal and cardiovascular risks by reducing nocturnal high blood pressure.
Asunto(s)
Bloqueadores del Receptor Tipo 1 de Angiotensina II/administración & dosificación , Presión Sanguínea/efectos de los fármacos , Glomerulonefritis por IGA/tratamiento farmacológico , Hipertensión Renal/tratamiento farmacológico , Inhibidores de los Simportadores del Cloruro de Sodio/administración & dosificación , Triclormetiazida/administración & dosificación , Adulto , Anciano , Inhibidores de la Enzima Convertidora de Angiotensina/administración & dosificación , Ritmo Circadiano , Diuréticos , Quimioterapia Combinada , Femenino , Glomerulonefritis por IGA/fisiopatología , Humanos , Hipertensión Renal/fisiopatología , Masculino , Persona de Mediana Edad , Proteinuria/tratamiento farmacológico , Proteinuria/fisiopatología , Resultado del TratamientoRESUMEN
BACKGROUND: In patients with heavy proteinuria, corticosteroid therapy has been shown to have favorable effects on the progression of IgA nephropathy. However, the efficacy of corticosteroids on the progression of IgA nephropathy with moderate proteinuria is still controversial. METHODS: We assessed 45 adult (age, 18-50 years) patients with moderate proteinuria (0.5-2.0 g daily) and preserved renal function, (serum creatinine concentration, < or = 106 micromol/l) who were diagnosed as having primary IgA nephropathy between December 1993 and July 1998. Twenty-three of the patients were treated with corticosteroids (steroid group), and the remaining 22 patients had no steroid treatment (control group). All patients were followed up for more than 3 years. RESULTS: There were no differences in baseline characteristics between the two groups, except for proteinuria. In the steroid group, urinary protein excretion was significantly higher than that in the control group. During the follow-up period, urinary protein excretion was not changed in the control group. On the other hand, in the steroid group, mean urinary protein excretion decreased significantly. Seven patients in the control group and 2 patients in the steroid group reached the endpoint, which was defined as a 50% increase in serum creatinine concentration from baseline. Renal survival curves were significantly different between the two groups. A second biopsy was performed in 20 patients who received steroid therapy. Mesangial cell proliferation, mesangial matrix increase, and cellular crescents were significantly reduced in the second compared with the first biopsy specimens. CONCLUSIONS: Steroid therapy is effective in reducing the progression of IgA nephropathy with moderate proteinuria.
Asunto(s)
Corticoesteroides/uso terapéutico , Glomerulonefritis por IGA/tratamiento farmacológico , Proteinuria/tratamiento farmacológico , Adolescente , Adulto , Biopsia , Progresión de la Enfermedad , Femenino , Glomerulonefritis por IGA/patología , Humanos , Masculino , Persona de Mediana Edad , Proteinuria/patología , Resultado del TratamientoRESUMEN
BACKGROUND: Bucillamine, a disease-modifying antirheumatic drug widely prescribed in Japan, is reported to be a cause of proteinuria. However, to date, the clinical course of the nephropathy associated with the use of bucillamine has not been described in detail. METHODS: We analyzed renal biopsy findings from 10 patients with rheumatoid arthritis and concomitant bucillamine-induced nephropathy. Each patient was followed up until proteinuria had resolved. RESULTS: Proteinuria appeared 2-11 months after the initiation of the treatment with bucillamine. Nine patients, who stopped bucillamine treatment immediately (within 3 months) after the onset of proteinuria, were diagnosed as having stage I membranous nephropathy. Only one patient, who used bucillamine for 9.5 months after the onset of proteinuria, was diagnosed as having stage II membranous nephropathy. In all patients with stage I membranous nephropathy, the proteinuria disappeared within 7 months after they stopped bucillamine treatment. On the other hand, in the patient with stage II membranous nephropathy, the proteinuria persisted for 14 months after the use of bucillamine was stopped. In all the patients, the proteinuria resolved completely without deterioration of renal function. None of the patients has experienced recurrence of proteinuria. CONCLUSIONS: In patients with proteinuria induced by treatment with bucillamine, membranous nephropathy is the most common disorder. Immediate withdrawal of bucillamine results in prompt and complete resolution of proteinuria without deterioration of renal function.Bucillamine, a disease-modifying antirheumatic drug widely prescribed in Japan, is reported to be a cause of proteinuria. However, to date, the clinical course of the nephropathy associated with the use of bucillamine has not been described in detail.
Asunto(s)
Antirreumáticos/efectos adversos , Artritis Reumatoide/complicaciones , Cisteína/análogos & derivados , Cisteína/efectos adversos , Glomerulonefritis Membranosa/inducido químicamente , Adulto , Anciano , Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Cisteína/uso terapéutico , Progresión de la Enfermedad , Relación Dosis-Respuesta a Droga , Femenino , Glomerulonefritis Membranosa/complicaciones , Glomerulonefritis Membranosa/patología , Humanos , Riñón/patología , Pruebas de Función Renal , Masculino , Persona de Mediana Edad , Proteinuria/inducido químicamenteRESUMEN
In patients with systemic lupus erythematosus(SLE), interstitial cystitis(lupus cystitis) is an uncommon, but important manifestation. We report two Japanese patients with lupus cystitis. Case 1 was a 49-year-old woman diagnosed as having rheumatoid arthritis and membranous nephropathy. She was treated with prednisolone(5 mg daily). Case 2 was a 41-year-old woman also diagnosed as having rheumatoid arthritis previously and treated with a non-steroidal anti-inflammatory drug. Both cases presented abdominal pain, vomiting, dysuria and frequency of micturition. We diagnosed these cases as SLE on the basis of arthritis, renal disorder(proteinuria and hematuria), and positive antinuclear and anti-dsDNA antibodies. In addition, bilateral hydronephrosis was found in both cases. Thus, they were also diagnosed as probable lupus cystitis. The patients were treated with one cycle of methylprednisolone pulse therapy. Thereafter they were treated with 60 mg/day of prednisolone and their symptoms resolved promptly. Furthermore, no abnormal finding was found by abdominal ultrasonography and/or the intravenous pyelogram after therapy. Renal biopsies were performed and both cases showed lupus glomerulopathy (case 1: WHO class Vb, case II: WHO class IVb). Abdominal pain and/or dysuria, which is common in SLE patients, requires further examinations to evaluate the lupus cystitis.