A potential pathogenic role of interleukin-6 in a child with ANCA-negative pauci-immune crescentic glomerulonephritis: case report and literature review

Abstract Background Crescentic glomerulonephritis is a disease characterized by severe glomerular injuries that is classified into five different pathological types. Patients with type V disease have pauci-immune crescentic glomerulonephritis (PICGN) that is negative for anti-neutrophil cytoplasmic autoantibodies (ANCAs). There are limited clinical data on the manifestations, treatment, and prognosis of type V crescentic glomerulonephritis, especially in children. Case presentation A 13-year-old girl who had an intermittent fever for more than 10 months was admitted to our hospital. She had no gross hematuria, oliguria, edema, or hypertension, but further tests indicated a decreased glomerular filtration rate, hematuria, proteinuria, and an elevated level of IL-6. The antinuclear antibody spectrum test was positive at 1:1000, and the ANCA and anti-glomerular basement membrane antibody tests were negative. A renal biopsy confirmed the diagnosis of ANCA-negative PICGN. We administered methylprednisolone pulse therapy with intravenous cyclophosphamide and oral mycophenolate mofetil. At the 3-month follow-up, her urine protein level was significantly lower, and her serum creatinine level was in the normal range. Conclusions Fever may be an extrarenal manifestation of ANCA-negative PICGN, and IL-6 may play a role in the pathogenesis of this disease. Early methylprednisolone pulse therapy with an immunosuppressant may reduce symptoms and improve prognosis.

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P0134PROTEINURIA IS NOT ALWAYS ASSOCIATED WITH PROGNOSIS IN IGA NEPHROPATHY

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0134 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Byoung-Soo Cho ◽

Hyaejin Yun ◽

Sungmin Jung ◽

Hyun-soon Lee

Keyword(s):

Renal Biopsy ◽

Iga Nephropathy ◽

Surrogate Marker ◽

Pulse Therapy ◽

Methylprednisolone Pulse Therapy ◽

Renal Pathology ◽

Crescent Formation ◽

Methylprednisolone Pulse ◽

Renal Biopsies

Abstract Background and Aims To date the most widely well studied risk factor for progression to ESRD in patients with IgA nephropathy is proteinuria. Recent report suggests proteinuria reduction as a surrogate end point in trial of IgA nephropathy(2019,CJASN). Sensitivity of most biomarkers such as blood and urine gd-IgA1 level, IgG/IgA autoantibody, sCD89, sCD71, NGAL, KIM-1, Cystatin-C etc were compared with the amount of proteinuria. Most nephrologists do not performing kidney biopsy in patients without proteinuria or proteinuria less than than 500mg/day even though IgA nephropathy is suspected. However we recently experienced severe IgA nephropathy (HSD Lee, grade IV) in patients with normal urinalysis, and more than half the patients showed stationary or aggravated renal pathology at the follow up renal biopsy although urinalysis findings were normalized after methylprednisolone pulse therapy. Method In our center we performed 892 renal biopsies during last 6 years, we experienced 253 IgA nephropathy, of which 152 cases were done follow up renal biopsies to see the pathologic changes who showed normalized urinalysis findings after methylprednisolone pulse therapy. Results Of the 253 patients 241 patients showed initial abnormal urinalysis like hematuria and or proteinuria. However eleven patients showed normal urinalysis at the time of renal biopsy, of which 5 cases were diagnosed as essential hypertension and 6 cases were normal urinalysis associated with lowered GFR. Of the 152 follow up renal biopsies we evaluated 99 cases who showed normalized urinalysis findings after therapy, of which 65 cases(65.7%) showed stationary or aggravated renal pathology. Conclusion In conclusion further long term studies are needed, proteinuria could not be a surrogate marker for prognosis of the IgA nephropathy, Regardless of proteinuria if associated with hypertension and or lowered GFR, renal biopsy should be done. Follow up renal biopsy might be needed to confirm the healing of IgA nephropathy regardless of urinary findings to see the disappearance of IgA deposition, decreasing mesangial and endocapillary hypercellularity, disappearance of crescent formation, decreasing sclerosis, etc.

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Identification of patients with COVID-19 who are optimal for methylprednisolone pulse therapy

Multidisciplinary Respiratory Medicine ◽

10.4081/mrm.2021.781 ◽

2021 ◽

Vol 16 ◽

Author(s):

Shosei Ro ◽

Naoki Nishimura ◽

Ryosuke Imai ◽

Yutaka Tomishima ◽

Clara So ◽

...

Keyword(s):

Cytomegalovirus Infection ◽

Elderly Person ◽

Pulse Therapy ◽

Methylprednisolone Pulse Therapy ◽

High Dose ◽

Pulmonary Aspergillosis ◽

Methylprednisolone Pulse ◽

High Dose Methylprednisolone ◽

Significant Difference

Background: Corticosteroids have been reported to reduce the mortality rates in patients with coronavirus disease 2019 (COVID-19). Additionally, the role of high-dose methylprednisolone pulse therapy in reducing mortality in critically ill patients has also been documented. The purpose of this study is to identify patients with COVID-19 who are suitable for methylprednisolone pulse therapy.Methods: This was a retrospective study that included patients with COVID-19 receiving methylprednisolone pulse therapy (>250 mg/day for 3 days) with subsequent tapering doses at our hospital between June 2020 and January 2021. We examined the differences in background clinical factors between the surviving group and the deceased group.Results: Of 156 patients that received steroid therapy, 17 received methylprednisolone pulse therapy. Ten patients recovered (surviving group) and seven patients died (deceased group). The median age of the surviving and deceased groups was 64.5 years (range, 57-85) and 79 years (73-90), respectively, with a significant difference (p=0.004). Five of the deceased patients (71%) had developed serious complications associated with the cause of death, including pneumothorax, pneumomediastinum, COVID-19-associated pulmonary aspergillosis, cytomegalovirus infection, and bacteremia. On the other hand, of the 10 survivors, only one elderly person had cytomegalovirus infection and the rest recovered without complications.Conclusion: Administration of methylprednisolone pulse therapy with subsequent tapering may be an effective treatment in patients with COVID-19 up to the age of early 70s; however, severe complications may be seen in elderly patients.

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