February 18, 2025

aCd, f 400, periodic acid-Schiff (PAS) stain, e 200, hematoxylin and eosin (HE) stain Open in a separate window Fig

aCd, f 400, periodic acid-Schiff (PAS) stain, e 200, hematoxylin and eosin (HE) stain Open in a separate window Fig.?3 Immunofluorescence findings of the patients renal pathology: IgA(+), IgG(?), IgM(?), C1q(?), C3(+), C4(?), and fibrinogen(?). His creatinine level and proteinuria markedly improved. His microscopic hematuria disappeared after he underwent tonsillectomy. There have been only a few case reports describing patients with adult-onset HSPN necessitating IVCY. We present here a rare case of steroid-resistant Iopromide HSPN treated with IVCY and tonsillectomy, with reference to some recent findings. antinuclear antibody, myeloperoxidase-anti-neutrophil cytoplasmic antibody, proteinase-3 anti-neutrophil cytoplasmic antibody Open in a separate window Fig.?2 Light microscopy findings. a Cellular crescents, b segmental sclerosis, c endocapillary proliferation, d advanced hyperplasia of the mesangial cells and lobulation, e inflammatory cell infiltration to some renal tubules, and f Iopromide tubulointerstitial lesion. aCd, f 400, periodic acid-Schiff (PAS) stain, e 200, hematoxylin and eosin (HE) stain Open Rabbit polyclonal to Aquaporin10 in a separate window Fig.?3 Immunofluorescence findings of the patients renal pathology: IgA(+), IgG(?), IgM(?), C1q(?), C3(+), C4(?), and fibrinogen(?). aCb 400, IF Treatment with steroid-pulse therapy (methylprednisolone 1000?mg/day for 3?days) was started and repeated every week for 2?weeks. Antiplatelet therapy using clopidogrel sulfate (75?mg/day) was also started. However, there was no improvement in the proteinuria, hematuria, or renal dysfunction, even after the patient received steroid-pulse therapy twice. Plasma exchange (PE) (4500?mL/day for 3?days) was performed. Even after the PE, no improvement was found. Additional steroid-pulse therapy at the same dose as at the beginning was repeated 2 more times within 2?weeks, but the HSPN was apparently steroid resistant. Since no further improvement was achieved with these treatments, intravenous cyclophosphamide (IVCY, 1000?mg/day) was administered after the 4th steroid-pulse therapy and PE. IVCY was repeated every 2?weeks. No marked side effects were found. With repeated IVCY, the patients proteinuria and renal dysfunction gradually improved. After finishing the 4th round of IVCY, his serum creatinine level and proteinuria decreased to 1 1.6?mg/dL and 3.6?g/day, respectively, and Iopromide the dose of oral prednisolone was gradually tapered (Fig.?4). Then, azathioprine was started as maintenance therapy. Consequently, it was confirmed that the IVCY was very effective for his nephrotic syndrome and renal dysfunction. Open in a separate window Fig.?4 Patients clinical course. intravenous cyclophosphamide, azathioprine, oral cyclophosphamide, prednisolone, plasma exchange, serum creatinine, urinary protein The patient was discharged on the 107th hospital day. Seven weeks later, he was admitted again for a 5th round of IVCY. At that time, his creatinine level was 1.8?mg/dL and the amount of proteinuria decreased to 0.8?g/day. Eight months after the 1st admission, his proteinuria disappeared. The total amount of cyclophosphamide (oral and intravenous) used was about 8?g under our treatment. The patient had wanted to undergo tonsillectomy before he became ill with HSPN, because he reported that he had frequently experienced high fever with tonsillitis. On the 217th day, he underwent tonsillectomy of his own will. After that, his hematuria apparently decreased and finally disappeared at 10?months after tonsillectomy. Although his recent Cr level measured 1.6?mg/dL, he persistently has had no proteinuria and no hematuria. His HSPN remains in remission. Discussion Approximately half of the cases with HSP are preceded by an upper respiratory infection, especially streptococcal infection. Other infectious agents, vaccinations, and insect bites also have been implicated as possible triggers for HSP. HenochCSch?nlein purpura occurs mainly in fall, winter, and spring, and rarely in summer [3C5]. The present patient had no episode of infection or vaccination; he became sick after an insect bite. The insect bite might have been a trigger of HSP in his case. Although there were a few areas of glomerular sclerosis, Iopromide and crescents were almost all cellular, his Cr level did not improve to a normal level, even though his proteinuria and hematuria disappeared. Therefore, it might be suggested that his HSPN had been rather severe and that no persistent therapy for HSPN at the beginning in Thailand might possibly have produced a therapeutic response. Iopromide Most studies have found that renal involvement in HSP tends to be more severe in adults than in children, as manifested by a higher incidence of nephrotic syndrome, hypertension, and elevated Cr levels [1]. However, there are no definitive treatments, partly because only a small number of adults experience severe HSPN. The present patient was initially treated by a corticosteroid after kidney biopsy. It has been stated that oral glucocorticoids produce a genomic effect through their receptors; a steroid pulse produces a non-genomic effect, which induces apoptosis of the lymphocytes by acting on cell membranes directly [23]. Unfortunately, the patients HSPN was steroid resistant. There have been case reports on the efficacy of cyclosporine [7], intravenous immunoglobulin [8], PE [9, 10], and tonsillectomy [11, 12] in patients resistant to traditional immunosuppressants. Although tonsillectomy is recommended.