Collapsing Glomerulopathy in Systemic Lupus Erythematosus

Haas, Mark

doi:10.2215/cjn.03590412

Cited by 13 publications

(17 citation statements)

References 26 publications

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“…The correlation of non-necrotizing crescents with the presence of APOL1 risk alleles is likely a result of the morphologic similarity between a florid collapsing lesion and cellular crescent formation. 13 Given this overlap and the higher incidence of crescents in biopsies from patients with two APOL1 risk alleles, it is plausible that some of these proliferative cases also had a component of undiagnosed CG. If so, then the results of this study would actually under-represent the strength of association between APOL1 risk alleles and CG in SLE.…”

Section: Discussionmentioning

confidence: 99%

Apolipoprotein L1 Risk Variants Associate with Systemic Lupus Erythematosus-Associated Collapsing Glomerulopathy

Larsen

Beggs

Saeed

et al. 2013

Journal of the American Society of Nephrology

187

136

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Collapsing glomerulopathy is a devastating renal disease that primarily affects African Americans and associates with numerous etiologies, such as HIV and autoimmune disease. The presence of APOL1 risk alleles associates with HIV-associated collapsing glomerulopathy, but it is unknown whether these risk alleles also associate with systemic lupus erythematosus (SLE) -associated collapsing glomerulopathy. Here, re-examination of 546 renal biopsies from African-American patients with SLE identified 26 cases of collapsing glomerulopathy, which we genotyped for APOL1 risk alleles using DNA extracted from archived biopsy tissue. APOL1 strongly associated with SLE-associated collapsing glomerulopathy (P,0.001). In a recessive model, two APOL1 risk alleles conferred 5.4-fold (95% CI=2.4 to 12.1) higher odds of developing SLE-associated collapsing glomerulopathy (P,0.001). In conclusion, APOL1 genotyping of African-American patients with SLE might help identify patients at risk for collapsing glomerulopathy, an entity with a poor prognosis that is often resistant to treatment.

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Section: Discussionmentioning

confidence: 99%

Apolipoprotein L1 Risk Variants Associate with Systemic Lupus Erythematosus-Associated Collapsing Glomerulopathy

Larsen

Beggs

Saeed

et al. 2013

Journal of the American Society of Nephrology

187

136

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“…(most notably HIV but also parvovirus B19 and hepatitis C), drugs, gene mutations, and vascular occlusion, in addition to idiopathic forms. 5 Histopathological examination is characterized by the global or segmental collapse of the glomerular capillary tuft, with wrinkling and retraction of the capillary walls overlaid by epithelial cell proliferation in the Bowman space. Collapsing glomerulopathy occurring in the setting of SLE has a strong association with persons of African descent; a typical clinical presentation with heavy proteinuria and often renal insufficiency and frequent progression to ESRD.…”

Section: Final Discussionmentioning

confidence: 99%

Lupus Nephritis or Infections—Whom to Blame?

Nada¹,

Kohli²,

Bardia³

et al. 2021

Journal of Postgraduate Medicine, Education and Research

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A 20-year-old woman was admitted with pedal edema, facial puffiness of 5-6 months duration, along with additional complaints of alopecia, oral ulcers, and photosensitivity. There was no arthralgia/ arthritis or Raynaud's phenomenon. On clinical examination and laboratory investigations, she was found to be anemic (Hb-6.8 mg/ dL) with pancytopenia (TLC-2,800/μL, platelet count-119,000/ μL). She was having nephrotic range proteinuria (24-hour urinary protein-3.2 g/TV) and mild renal dysfunction (S. creatinine-1.3 mg/dL). Her serum complement (C3/C4) was low. Based on the above findings, a diagnosis of systemic lupus erythematosus (SLE) was made with hematological and renal involvement. This was further supported by positive ANA and dsDNA titers. Her pANCA was also positive. Hence, a renal biopsy was performed for histopathological examination which confirmed the presence of crescentic glomerulonephritis due to lupus nephritis (LN) (class IV) along with thrombotic microangiopathy (TMA). She was given MP pulses and oral steroids (1 mg/kg/day). Cell counts improved after steroid treatment. Thereafter, a monthly cyclophosphamide pulse was given according to NIH protocol with oral steroids. Two weeks later, she complained of depressive symptoms. Her renal function deteriorated with rising serum creatinine (4.8 mg/dL) and blood urea (116). Her platelet count dropped to 78,000/mL and serum LDH was high (2,200 IU/L). A possibility of TTP/CNS lupus was considered. She was managed with anti-depressants, seven sessions of plasma exchange, and another dose of endoxan in view of worsening of disease activity. Neuropsychiatric symptoms improved and serum creatinine came down to 3.3 mg/dL. However, after a few days, she became febrile with pain and swelling of the left thigh, decreased oral intake, and irritability. A possibility of left thigh cellulitis was considered and managed with piperacillin and tazobactam in emergency OPD. She subsequently became afebrile with a reduction in redness and swelling. Despite this, she remained irritable and had decreased oral intake. Thus, she was readmitted for the third time.

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“…While each of these pathological findings has been reported to occur independently in association with SLE, this is the first instance in which they are reported to occur simultaneously in the setting of both SLE and HLH. Most investigations consider CG to represent the most severe morphological expression of lupus podocytopathy, in which there is rapid and widespread podocyte loss 16 17…”

Section: Discussionmentioning

confidence: 99%

Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles

et al. 2019

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Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II. Genotyping for APOL1 risk alleles revealed the presence of double (G1/G2) risk alleles. Our patient achieved a complete renal recovery and resolution of HLH within 1 month of treatment with steroids and mycophenolate mofetil, highlighting the importance of prompt, aggressive therapy.

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Collapsing Glomerulopathy in Systemic Lupus Erythematosus

Cited by 13 publications

References 26 publications

Apolipoprotein L1 Risk Variants Associate with Systemic Lupus Erythematosus-Associated Collapsing Glomerulopathy

Apolipoprotein L1 Risk Variants Associate with Systemic Lupus Erythematosus-Associated Collapsing Glomerulopathy

Lupus Nephritis or Infections—Whom to Blame?

Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles

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Collapsing Glomerulopathy in Systemic Lupus Erythematosus

Cited by 13 publications

References 26 publications

Apolipoprotein L1 Risk Variants Associate with Systemic Lupus Erythematosus-Associated Collapsing Glomerulopathy

Apolipoprotein L1 Risk Variants Associate with Systemic Lupus Erythematosus-Associated Collapsing Glomerulopathy

Lupus Nephritis or Infections&mdash;Whom to Blame?

Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles

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Lupus Nephritis or Infections—Whom to Blame?