Favorable Renal Outcome of Japanese Children with Severe IgA Nephropathy With Nephrotic Syndrome

Background: Nephrotic syndrome is a rare but severe feature of IgA nephropathy. Casecharacteristics: Nine Japanese children with severe IgA nephropathy with nephroticsyndrome. Intervention: All received low-dose intravenous methylprednisolone (IVMP)within five weeks after the disease onset.Eight out of nine patients achieved resolution ofproteinuria without severe adverse events. Message: Early low-dose intravenousmethylprednisolone may be safe and effective for children with severe IgA nephropathywith nephrotic syndrome

A combination of intravenous immunoglobulin and pulse methylprednisolone extended survival in pulmonary alveolar proteinosis with chronic interstitial pneumonitis: a case report.

Pulmonary alveolar proteinosis (PAP) is characterized by intra-alveolar accumulation of lipoproteinaceous material. The severe chronic pulmonary disease and susceptibility to pulmonary infection is a prominent feature of the disease. We reported a case of postnatal-onset PAP and chronic interstitial pneumonitis in a girl with chronic respiratory distress since she was 5 months of age. A lung biopsy confirmed the diagnosis. The therapeutic bronchoalveolar lavages, a short trial of granulocyte colony-stimulation factor (G-CSF) and a combination of low dose methylprednisolone and hydroxychloroquine were used at different times without noting satisfactory improvement. Intravenous immunoglobulin (IVIG) and pulse methylprednisolone were given monthly with gradual recovery. She did not require oxygen supplement after 21 months of this combination. Our report suggested that IVIG and pulse methylprednisolone might have a potential role in the treatment of PAP with chronic interstitial pneumonitis.

Side Effects of Intravenous Methylprednisolone Pulse Therapy in Eye Diseases

PURPOSE: To determine the side effects of short-term high-dose methylprednisolone therapy (pulse methylprednisolone therapy), which was used to treat patients with optic neuritis, traumatic optic neuropathy, orbital pseudotumor, and orbital myositis. METHODS: All 27 patients treated with pulse methylprednisolone therapy from June 2005 to June 2006 were included in this study. Each patient was injected with 1 g per day of methylprednisolone intravenously, administered in 250 mg doses every 6 hours, for 3 days. We measured body mass index (BMI), fasting glucose, HbA1c, blood pressure, and intraocular pressure. In addition, we performed slit lamp examination and chest X-ray. RESULTS: The average age of the 27 patients was 35.8 years, including 16 males and 11 females. Thirteen patients had optic neuritis, 10 patients had traumatic optic neuropathy, 3 patients had orbital pseudotumors, and 1 patient had orbital myositis. Before treatment, the average of body mass index (BMI) was 23.09 (kg/m2), but at 3 months after treatment it was 23.73 (kg/m2). All patients had fasting glucose levels higher than the normal range during the treatment. However, all fasting glucose levels returned to within the normal range at 3 months after treatment, except in 1 patient. In addition, a major complaint among patients was an increase in body weight. CONCLUSIONS: We performed a close observation of controlled diet and exercise on patients who were treated with pulse methylprednisolone therapy because we knew it would have a hyperglycemic effect. An increase in body weight is the most common complaint of steroid therapy. Even after treatment is completed, it is important to monitor glucose level and weight.

A Case of Transverse Myelitis associated with Systemic Lupus Erythematosus

Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE) and its prognosis is very poor including death or severe neurologic sequelae. We report a 14-year-old girl with transverse myelitis who was not exactly diagnosed as SLE before the onset of neurologic symptoms. Transverse myelitis was diagnosed based on the clinical presentations, cerebrospinal fluid analysis and MRI findings. We employed aggressive treatment with pulse methylprednisolone for acute episodes followed by monthly cyclophosphamide pulse therapy. For the first several months, clinical improvement of the neurologic impairment was noted, but unfortunately her neurological course was on exacerbation.