Synthetic ACTH for Treatment of Glomerular Diseases: A Case Series

Rationale: Synthetic adrenocorticotropic hormone (Tetracosactide) has been used in the treatment of refractory glomerular diseases. Literature surrounding the use of this medication is limited to small case series and there is conflicting data on the rate of adverse events associated with this medication. Presenting concerns of the patient: Glomerulonephritis not in remission after at least 6 months of treatment with conservative care. Stable doses of concurrent immunosuppression were permitted. Diagnoses: Membranous nephropathy, IgA nephropathy, minimal change disease, and focal and segmental glomerulosclerosis. Intervention: Intramuscular synthetic adrenocorticotropic hormone (Tetracosactide, Synacthen Depot) with doses of either 1 mg weekly or 1 mg twice weekly. Outcomes: Five of 12 patients had at least a partial remission with Tetracosactide. Median time to response was 6 months for responders. Five of the 12 patients had adverse events documented, 2 of which led to treatment discontinuation. No patients with focal and segmental glomerulosclerosis responded to treatment. Lessons Learned: Higher rate of adverse events than previously reported with synthetic adrenocorticotropic hormone and uncertain treatment efficacy.

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Emerging therapeutic strategies for minimal change disease and focal and segmental glomerulosclerosis

Expert Opinion on Investigational Drugs ◽

10.1080/13543784.2018.1540587 ◽

2018 ◽

Vol 27 (11) ◽

pp. 839-879 ◽

Cited By ~ 4

Author(s):

Rossella Siligato ◽

Valeria Cernaro ◽

Chiara Nardi ◽

Francesca De Gregorio ◽

Guido Gembillo ◽

...

Keyword(s):

Minimal Change Disease ◽

Therapeutic Strategies ◽

Minimal Change ◽

Focal And Segmental Glomerulosclerosis ◽

Segmental Glomerulosclerosis

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Efficacy and safety of rituximab in adult frequent-relapsing or steroid-dependent minimal change disease or focal segmental glomerulosclerosis: a systematic review and meta-analysis

Clinical Kidney Journal ◽

10.1093/ckj/sfaa191 ◽

2020 ◽

Author(s):

Cheng Xue ◽

Bo Yang ◽

Jing Xu ◽

Chenchen Zhou ◽

Liming Zhang ◽

...

Keyword(s):

Systematic Review ◽

Adverse Events ◽

Focal Segmental Glomerulosclerosis ◽

Minimal Change Disease ◽

Meta Analysis ◽

Minimal Change ◽

Efficacy And Safety ◽

Segmental Glomerulosclerosis ◽

Steroid Dependent

Abstract Background The efficacy and safety of rituximab (RTX) in adult frequent-relapsing (FR) or steroid-dependent (SD) nephrotic syndrome (NS), including minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS), are still inconclusive. Methods We performed a systematic review and meta-analysis registered in PROSPERO (CRD42019148102) by pooling data of cohort studies or case series on adult patients with difficult-to-treat NS. Steroid-resistant NS was excluded. The primary outcomes were the complete remission (CR) rate and the relapse rate. Partial remission (PR) rate, no response (NR) rate and adverse events were the secondary outcomes. A random-effects model was performed for all the outcomes. Results We included 21 studies involving 382 adult MCD/FSGS subjects with a median follow-up duration from 12 to 43 months. RTX treatment induced a pooled 84.2% CR rate [95% confidence interval (CI): 67.7–96.3%], while MCD patients had a high 91.6% CR rate and FSGS patients a moderate 43% CR rate. However, 27.4% (95% CI 20.7–34.5%) of the patients relapsed during the follow-up. The pooled PR and NR rates were 5.8% (95% CI 1.2–12.5%) and 5.2% (95% CI 0.0–15.0%), respectively. RTX was associated with trivial adverse events and good tolerance. Conclusions In summary, by pooling results of current pilot studies, RTX may be an effective and relatively safe alternative for most adult FR or SD MCD/FSGS to displace calcineurin inhibitors or prednisone in the hierarchy of treatment. More clinical trials comparing RTX with other immunosuppressants and concerning the long-term adverse events are needed.

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Review of Glomerular Diseases: Focal Segmental Glomerulosclerosis (FSGS) and Minimal Change Disease (MCD)

Sciential - McMaster Undergraduate Science Journal ◽

10.15173/sciential.v1i2.2091 ◽

2019 ◽

pp. 9-17

Author(s):

Gursharan Kaur Sohi

Keyword(s):

Focal Segmental Glomerulosclerosis ◽

Large Scale ◽

Minimal Change Disease ◽

Treatment Protocol ◽

Minimal Change ◽

Future Research ◽

Line Treatment ◽

Glomerular Diseases ◽

Hand Search ◽

Segmental Glomerulosclerosis

Purpose: Idiopathic focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are chronic glomerulopathies which may compromise patients’ quality of life, and for which there is no cure. This literature review aimed to summarize our current understanding of the pathophysiology, clinical characteristics, and best available treatment for the two conditions in order to outline a consolidated treatment protocol and identify future research considerations. Methods: PubMed was systematically searched by a single reviewer in order to identify primary studies pertaining to the diagnosis, treatment and classification of FSGS and MCD. Additionally, a hand search of UpToDate was conducted to glean further information about the best available evidence as summarized for clinician use. Relevant information was extracted and synthesized. Results: Primary FSGS and MCD result from distinct pathogenic mechanisms, hypothesized to involve kidney injury via immune dysregulation. Patients require a kidney biopsy for diagnostic purposes. First-line treatment involves glucocorticoids (i.e. prednisone), although patients’ responsiveness may be inconsistent; second-line treatment is immunotherapy. Conclusion: This review summarized clinically-important information about FSGS and MCD, and emphasized the need for further research in the field of clinical nephrology. Large scale trials such as the Cure Glomerulonephropathy should be conducted to gather information about the affected population.

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The Primary Glomerular Diseases

10.2310/nephro.12022 ◽

2017 ◽

Author(s):

Richard J. Glassock ◽

Sanjeev Sethi ◽

Fernando C. Fervenza

Keyword(s):

Renal Biopsy ◽

Membranous Nephropathy ◽

Minimal Change Disease ◽

Crescentic Glomerulonephritis ◽

Minimal Change ◽

C3 Glomerulonephritis ◽

Focal And Segmental Glomerulosclerosis ◽

Glomerular Diseases ◽

Dense Deposit ◽

Primary Glomerular Diseases

Glomerular disorders in which the manifestations of disease are primarily confined to the kidneys, without multisystem involvement, are not only common but very heterogeneous in terms of pathogenesis and clinical features. Typically, these primary glomerular diseases are characterized according to the findings on renal biopsy, as studied by light, immunofluorescence, and electron microscopy. The principal primary glomerular diseases are minimal change disease, focal and segmental glomerulosclerosis, membranous nephropathy, C3 glomerulonephritis and dense deposit disease, IgA nephropathy, and renal-limited crescentic glomerulonephritis. These clinicopathologic entities are discussed according to epidemiology, clinical features, pathology, pathogenesis (and genetics if appropriate), prognosis, and treatment, emphasizing recent findings. Key words: C3 glomerulonephritis, dense deposit disease, focal and segmental glomerulosclerosis, glomerulonephritis, IgA nephropathy, membranous nephropathy, minimal change disease, renal biopsy, renal-limited crescentic glomerulonephritis

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Efficacy of abatacept treatment for focal segmental glomerulosclerosis and minimal change disease: A systematic review of case reports, case series, and observational studies

Clinical Nephrology ◽

10.5414/cn110134 ◽

2020 ◽

Vol 94 (3) ◽

pp. 117-126

Author(s):

Panupong Hansrivijit ◽

Max M. Puthenpura ◽

Nasrollah Ghahramani

Keyword(s):

Systematic Review ◽

Focal Segmental Glomerulosclerosis ◽

Observational Studies ◽

Minimal Change Disease ◽

Case Reports ◽

Case Series ◽

Minimal Change ◽

Segmental Glomerulosclerosis

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Urinary Exosomal miR-193a Can Be a Potential Biomarker for the Diagnosis of Primary Focal Segmental Glomerulosclerosis in Children

BioMed Research International ◽

10.1155/2017/7298160 ◽

2017 ◽

Vol 2017 ◽

pp. 1-6 ◽

Cited By ~ 7

Author(s):

Zhibin Huang ◽

Yong Zhang ◽

Jianhua Zhou ◽

Yu Zhang

Keyword(s):

Focal Segmental Glomerulosclerosis ◽

Minimal Change Disease ◽

Roc Analysis ◽

Minimal Change ◽

Glomerular Diseases ◽

Segmental Glomerulosclerosis ◽

Diagnostic Potential ◽

Potential Biomarker ◽

Noninvasive Biomarker ◽

Exosomal Microrna

Background. Glomerular upregulation of miR-193a has been detected in primary focal segmental glomerulosclerosis (FSGS) but not in other glomerular diseases. We aimed to isolate exosomes from urine of children with primary FSGS and to assess the diagnostic potential of urinary exosomal miR-193a for primary FSGS. Methods. The first morning urine samples were collected from children with primary FSGS (n=8) and minimal change disease (MCD, n=5). Isolated urinary exosomes were confirmed by electron microscopy and Western blotting. Urinary exosomal microRNA was extracted, and the expression levels of exosomal miR-193a were quantified by real-time PCR. The diagnosis value of urinary exosomal miR-193a levels for primary FSGS was evaluated by ROC analysis. Results. The isolated vesicles were qualitatively compatible with exosomes. The levels of urinary exosomal miR-193a were significantly higher in children with primary FSGS than those in children with MCD. Moreover, the area under the ROC for the diagnosis of primary FSGS using urinary exosomal miR-193a was 0.85. Conclusions. A significant increase in the levels of urinary exosomal miR-193a in primary FSGS patients compared to those in MCD ones was observed. This study suggests that urinary exosomal miR-193a may be a new noninvasive biomarker for the diagnosis of primary FSGS.

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