Primary focal segmental glomerulosclerosis

2018 ◽  
Author(s):  
Alain Meyrier ◽  
Patrick Niaudet
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Viral Infections ◽  
Therapy Response ◽  
High Dose ◽  
African Origin ◽  
Donor Kidney ◽  
Progressive Loss ◽  
Segmental Glomerulosclerosis ◽  
Collapsing Fsgs ◽  
Focal Segmental Sclerosis

Primary focal segmental glomerulosclerosis (FSGS) causes nephrotic syndrome and by definition is not caused by any of the known causes of podocyte toxicity or focal segmental sclerosis such as viral infections or toxins. A number of genetic causes of FSGS are commonly diagnosed in early childhood. Other causes of segmental scarring need to be distinguished. Genotypes in APOL1 of African origin are associated with higher incidence of FSGS and poorer responses to treatment. Cellular and collapsing FSGS are variants of FSGS in which there is overt acute podocytopathy and they have a relatively poor prognosis. A glomerular tip lesion is thought to have a slightly better prognosis than other types. Some cases of primary FSGS respond to high-dose corticosteroids, sometimes only after prolonged therapy. Response to steroids is a good prognostic sign, and without a response, progressive loss of renal function is likely. A circulating factor is implicated by the observation that proteinuria can recur in a donor kidney within hours of transplant. Plasma exchange appears to remove this factor but it is not conclusively identified.

scholarly journals Collapsing Focal Segmental Glomerulosclerosis Following Treatment with High-Dose Pamidronate

2001 ◽  
Vol 12 (6) ◽  
pp. 1164-1172 ◽  
Author(s):  
GLEN S. MARKOWITZ ◽  
GERALD B. APPEL ◽  
PAUL L. FINE ◽  
ANDREW Z. FENVES ◽  
NICHOLAS R. LOON ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Insufficiency ◽  
Focal Segmental Glomerulosclerosis ◽  
Metastatic Breast ◽  
Recommended Dose ◽  
Temporal Association ◽  
High Dose ◽  
Metastatic Breast Carcinoma ◽  
Segmental Glomerulosclerosis ◽  
Collapsing Fsgs

Abstract. Collapsing focal segmental glomerulosclerosis (FSGS) is a distinct clinicopathologic entity seen most commonly in young African American patients who present with renal insufficiency and nephrotic syndrome. The only epidemiologic factor previously linked to collapsing FSGS is HIV infection. Here clinicopathologic findings are reported for a distinctive population of seven patients, who were older, Caucasian, and HIV negative and developed collapsing FSGS during active treatment of malignancy (multiple myeloma in six patients and metastatic breast carcinoma in one). Although oncologic treatment regimens included vincristine for four patients, doxorubicin for five patients, cisplatin for two patients, and total-body irradiation for one patient, the only agent common to all patients was pamidronate (Aredia). All patients had normal renal function before the administration of pamidronate. Patients began therapy with pamidronate at or below the recommended dose of 90 mg, intravenously, monthly, which was increased to 180 mg monthly in two patients and 360 mg monthly in three patients. Patients received pamidronate for 15 to 48 mo before presentation with renal insufficiency (mean serum creatinine, 3.6 mg/dl) and full nephrotic syndrome (mean 24-h urinary protein excretion, 12.4 g/d). Pamidronate, which is a member of the class of bisphosphonates, is widely used in the treatment of hypercalcemia of malignancy and osteolytic metastases. At the recommended dose of 90 mg, intravenously, monthly, renal toxicity is infrequent; however, higher doses have produced nephrotoxicity in animal models. The temporal association between pamidronate therapy and the development of renal insufficiency, the use of escalating doses that exceed recommended levels, and the distinctive pattern of glomerular and tubular injury strongly suggest a mechanism of drug-associated podocyte and tubular toxicity. These data provide the first association of collapsing FSGS with toxicity to a therapeutic agent.

P1757RITUXIMAB FOR RECURRENCE OF PRIMARY FOCAL SEGMENTAL GLOMERULOSCLEROSIS AFTER KIDNEY TRANSPLANTATION: RESULTS OF A NATIONWIDE STUDY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Camille Lanaret ◽  
Dany Anglicheau ◽  
Audard Vincent ◽  
Celine Lambert ◽  
Lionel Couzi ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Standard Of Care ◽  
High Dose ◽  
Infectious Risk ◽  
Segmental Glomerulosclerosis ◽  
Group 2 ◽  
Retrospective Multicenter Study ◽  
Group 1 ◽  
Rejection Rates

Abstract Background and Aims The indication of rituximab (RTX) in the treatment of primary focal segmental glomerulosclerosis (FSGS) recurrence after kidney transplantation (KT) remains controversial. The objective of our study was to evaluate the benefit and tolerability of adding RTX to the standard of care (SOC) comprising plasmapheresis (PP), corticosteroids, and high-dose anticalcineurins for the treatment of FSGS recurrence after KT. Method This retrospective, multicenter study reports on 148 patients, transplanted between 31 December 2004 and 31 December 2018, aged 39.9 + 13.4 years, who developed FSGS recurrence at 7 [3–23] days. In all 109 patients received a SOC (Group 1). RTX was introduced in this group after more than 28 days of SOC for failure or for therapeutic intensification (n = 19, Group 1a), or for early discontinuation of PP (n = 12, Group 1b); 39 patients received RTX associated at the outset with SOC (Group 2). Results We observed 46.6% complete remission (CR) and 33.1% partial remission (PR). Ten-year graft survival was 65.6% [51.4–76.6] and 13.4% [3.4–30.0] in responders and non-responders respectively. There was no difference in CR + PR rate between G1 (82.5%) and G2 (71.8%), p = 0.08, confirmed by propensity score +4.3% (95% CI [−9.0%-17.5%], p = 0.53). Following addition of RTX (Group 1a), we observed a CR rate of 26.3% and a PR rate of 31.6%. Patients with and without RTX experienced similar rejection rates (18.6% and 28.2%, p = 0.17) and infection rates (71.4% and 79.5%, p = 0.40). In multivariate analysis, the infections were associted with hypogammaglobulinemia <5g/l (OR = 8.04, 95% CI [1.65,39.25], p = 0.01). Conclusion Rituximab could be used in cases of SOC failure or in remission patients for early weaning of plasmapheresis, without increasing infectious risk.

scholarly journals Collapsing focal segmental glomerulosclerosis probably triggered by dengue virus infection - two case reports

2020 ◽  
Author(s):  
Patrícia Cruz Queiroz ◽  
Ana Elisa Souza Jorge ◽  
Plínio Henrique Vaz Mourão ◽  
Maria Goretti Moreira Guimarães Penido
Keyword(s):  
Renal Function ◽  
Nephrotic Syndrome ◽  
Virus Infection ◽  
Dengue Virus ◽  
Focal Segmental Glomerulosclerosis ◽  
Case Reports ◽  
Kidney Injury ◽  
Dengue Virus Infection ◽  
Segmental Glomerulosclerosis ◽  
Collapsing Fsgs

Abstract The reported cases describe the association between collapsing focal segmental glomerulosclerosis (FSGS) and acute dengue virus infection. In both cases, patients were diagnosed with dengue virus infection and had a severe kidney disease, with nephrotic syndrome and acute kidney injury. Kidney biopsy was performed and showed collapsing FSGS. The first patient, a 27-year-old man, was diagnosed with dengue virus infection and developed nephrotic syndrome after two weeks of illness. He was treated with methylprednisolone for three days and intravenous furosemide. This patient evolved well, although his renal function did not fully recover. The second patient, a 32-year-old man, was diagnosed with a milder clinical presentation of dengue virus infection. He had a past medical history of nephrotic syndrome in childhood, which might have caused its relapse. This patient was treated with intravenous furosemide and also did not fully recover renal function. These cases highlight the possible implication of dengue virus infection in the etiology of collapsing variant of FSGS. Healthcare professionals should be prepared to identify similar cases.

scholarly journals Collapsing Focal Segmental Glomerulosclerosis in a Patient with Acute Malaria

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Najamus Sehar ◽  
Emad Gobran ◽  
Suzanne Elsayegh
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Case Reports ◽  
Left Kidney ◽  
Elevated Serum ◽  
Segmental Glomerulosclerosis ◽  
Acute Distress ◽  
Night Sweats ◽  
History Of ◽  
Secondary Form ◽  
Collapsing Fsgs

Introduction. Collapsing focal segmental glomerulosclerosis (FSGS) is most commonly seen in association with HIV infection. Rare data is available about the association between collapsing FSGS and malaria.Case Description. A 72-year-old African male patient presented to the hospital for generalized body aches, fatigue, fever, and night sweats for three days. He had history of recent travel to Ghana. Patient looked in acute distress and was shivering. Laboratory tests showed elevated serum creatinine (Cr) of 2.09 mg/dL (baseline was 1.5 mg/dL in 2012). Hospital course was significant for rapid elevation of Cr to 9.5 mg/dL and proteinuria of 7.9 grams. Autoimmune studies resulted negative. Blood smear resulted positive forPlasmodium falciparumand patient was treated with Artemether/Lumefantrine. Patient’s fever and pain improved, but kidney function continued to deteriorate and he became oliguric. On day seven, he was started on Hemodialysis. Tests for different causes of glomerular pathology were also negative. He underwent left kidney biopsy which resulted in findings consistent with severe collapsing glomerulopathy.Discussion. This case illustrates a biopsy proven collapsing FSGS likely secondary to malarial infection requiring renal replacement therapy. Literature review revealed only few case reports that suggested the possible association of malaria with secondary form of FSGS.

Viral Nephropathies

2017 ◽  
Author(s):  
Kar Neng Lai ◽  
Andrew SH Lai ◽  
Sydney CW Tang
Keyword(s):  
Hepatitis C ◽  
Hepatitis B ◽  
Hiv Infection ◽  
Focal Segmental Glomerulosclerosis ◽  
Viral Infections ◽  
Parvovirus B19 ◽  
Laboratory Data ◽  
Molecular Testing ◽  
Segmental Glomerulosclerosis ◽  
Bk Polyomavirus

Viral infections are important causative agents in renal disease and are responsible for significant morbidity and mortality. The diagnostic criteria for virus-related nephropathy include detailed clinical and laboratory data and tissue molecular analysis. Possible pathogenetic mechanisms include kidney tropism of the virus, induction of abnormal immune complexes, direct cytopathogenic effects, and multiorgan failure. Hepatitis B virus is associated with membranous nephropathy and mesangiocapillary glomerulonephritis in endemic areas. Hepatitis C virus causes various forms of glomerulonephritis, including cryoglobulinemia-mediated glomerulonephritis. HIV infection is associated with a collapsing focal segmental glomerulosclerosis, a distinctive disease that mainly affects Africans and African Americans. In the course of HIV infection, other types of immune complex glomerulonephritis may occur. Recent reports indicate a role for parvovirus B19 in idiopathic collapsing focal segmental glomerulosclerosis. Acute kidney injury occurs in hantavirus and coronavirus-associated severe acute respiratory syndrome. Of particular interest are those viral infections with productive replication in the kidney, which often occur in immunocompromised hosts, such as renal allograft recipients. Epstein-Barr virus, cytomegalovirus, adenovirus, and BK polyomavirus are prominent members of this group causing specific diseases. Renal biopsy followed by appropriate serologic and molecular testing is essential for defining virus-related nephropathies and guiding prognostic and therapeutic evaluation. This review contains 4 figures, 3 tables, and 90 references. Key words: BK polyomavirus, cytomegalovirus, glomerulonephritis, hepatitis B, hepatitis C, HIV-associated nephropathy, viral infection

scholarly journals Interferon Induced Focal Segmental Glomerulosclerosis

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Yusuf Kayar ◽  
Nuket Bayram Kayar ◽  
Nadir Alpay ◽  
Jamshid Hamdard ◽  
Iskender Ekinci ◽  
...  
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Viral Infections ◽  
Behçet's Disease ◽  
Immune Deficiency Syndrome ◽  
Deficiency Syndrome ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Segmental Glomerulosclerosis

Behçet’s disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet’s disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon.

scholarly journals Infection-Related Focal Segmental Glomerulosclerosis in Children

2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Anne Katrin Dettmar ◽  
Jun Oh
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Viral Infections ◽  
Parvovirus B19 ◽  
Systemic Infection ◽  
Human Immune Deficiency Virus ◽  
Steroid Resistant ◽  
Pediatric Nephrologist ◽  
Segmental Glomerulosclerosis ◽  
B Virus ◽  
Viral Components

Focal segmental glomerulosclerosis (FSGS) is the most common cause of steroid resistant nephrotic syndrome in children. It describes a unique histological picture of glomerular damage resulting from several causes. In the majority of patients the causing agent is still unknown, but in some cases viral association is evident. In adults, the most established FSGS causing virus is the human immune-deficiency virus, which is related to a collapsing variant of FSGS. Nevertheless, other viruses are also suspected for causing a collapsing or noncollapsing variant, for example, hepatitis B virus, parvovirus B19, andCytomegalovirus. Although the systemic infection mechanism is different for these viruses, there are similarities in the pathomechanism for the induction of FSGS. As the podocyte is the key structure in the pathogenesis of FSGS, a direct infection of these cells or immediate damage through the virus or viral components has to be considered. Although viral infections are a very rare cause for FSGS in children, the treating pediatric nephrologist has to be aware of a possible underlying infection, as this has a relevant impact on therapy and prognosis.

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