Nephrotic Syndrome in Scleroderma

Feijoo, María Luisa Velloso; Montero, Sergio Rodríguez; Moreno, Paz González; González, Lucía Mayordomo; Gieb, Eduardo Rejón; Alonso, Jorge Fernández; Fuente, José Luís Marenco de la

doi:10.1016/s2173-5743(07)70263-0

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“…In the present case, our patient had been diagnosed with diffuse SSc 8 years before developing nephrotic syndrome (focal segmental glomerulosclerosis). It is uncommon for patients with SSc to develop nephrotic syndrome, but certain drugs, such as D-penicillamine, can induce it, or rarely, it can be a manifestation of secondary amyloidosis [ 11 ]. To the best of our knowledge, our patient had not been prescribed any medication that leads to nephrotic syndrome; however, since the association between SSc and nephrotic syndrome is rare, it is essential to note that the results of this case do not necessarily prove that nephrotic syndrome is a consequence of SSc.…”

Section: Discussionmentioning

confidence: 99%

Nephrotic syndrome due to focal segmental glomerulosclerosis complicating scleroderma: a case report

Mehdipour Dalivand,

Hadjiabbasi,

Ramezanzadeh

et al. 2024

J Med Case Reports

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Background Systemic scleroderma (SSc) is an insidious autoimmune connective tissue disorder with multiorgan involvement. Renal involvement is one of the important causes of morbidity and mortality in scleroderma; however, nephrotic syndrome is reported rarely in association with SSc. We present a patient with SSc who developed focal segmental glomerulosclerosis (FSGS) as a complication of scleroderma. Case presentation A 59 year old Caucasian female patient, with a known history of diffuse systemic sclerosis from 8 years, presented to our clinic with symptoms of anasarca and weight gain. Her physical examination was unremarkable except for periorbital and extremity edema. Her biochemistry assessment revealed decreased serum albumin levels and elevated serum creatinine levels. A renal biopsy was performed, which showed histopathological patterns of FSGS type of nephrotic syndrome. After administration of high doses of steroid and rituximab in the course of her treatment for 6 months, her symptoms and proteinuria were improved without the occurrence of scleroderma renal crises. Conclusion SSc is a complex multisystemic autoimmune disorder. SRC is the most prominent renal involvement in SSc, but other renal pathologies may also occur. Each patient should be precisely investigated since managing these renal conditions can differ significantly. Nephrotic syndrome is a rare complication of SSc, which could be managed with prompt diagnosis and steroid administration.

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Section: Discussionmentioning

confidence: 99%