Complete remission of non-HIV collapsing glomerulopathy with deflazacort and lisinopril in an adult patient

Abstract

Collapsing glomerulopathy is a form of focal segmental glomerulosclerosis that is usually associated with HIV-1 infection, and is characterized by its poor prognosis and almost inevitable progression to end-stage renal disease. Its pathological features include collapsed glomeruli, podocyte hypertrophy and hyperplasia, and pseudocrescents. This case report shows the evolution of a 58-year-old patient with non-HIV idiopathic collapsing glomerulopathy who presented with severe nephrotic syndrome and renal insufficiency and was treated with lisinopril and deflazacort, a synthetic corticosteroid that has shown fewer cosmetic effects and glucose and bone metabolism complications than prednisone. The patient responded with full recovery of renal function and normal range of protein excreted in urine after less than two years of treatment. The patient has not suffered a recurrence of his nephrotic syndrome after three years of steroid withdrawal. There is no proven therapy for collapsing glomerulopathy, and this case highlights an alternative for treating this disease with few secondary effects.

Variante colapsante de la glomeruloesclerosis focal y segmentaria en niños

“…The collapsing variant of focal segmental glomerulosclerosis (FSGS), also called collapsing glomerulopathy, is a well-defined glomerular disease. This variant of FSGS was first described by Weiss in 1986, who found these lesions in HIV-negative patients (1)(2)(3). This glomerulopathy is one of five variants of FSGS (2) proposed by a group at Columbia University in 2000 (4).…”

“…Clinically, the collapsing variant of FSGS is characterized by nephrotic syndrome (NS) with massive proteinuria, hematuria, and hypertension (HTN) (2). Patients with this variant have a poor response to empirical therapy and exhibit rapid deterioration of renal function (3,8,9). Idiopathic cases exist; however, the collapsing variant of FSGS is often associated with secondary forms due to HIV and parvovirus infections (2,10); drugs, such as pamidronate (2); autoimmune diseases, such as lupus (11); malignant neoplasms (3), thrombotic microangiopathy, and mitochondrial diseases (3).…”

“…Patients with this variant have a poor response to empirical therapy and exhibit rapid deterioration of renal function (3,8,9). Idiopathic cases exist; however, the collapsing variant of FSGS is often associated with secondary forms due to HIV and parvovirus infections (2,10); drugs, such as pamidronate (2); autoimmune diseases, such as lupus (11); malignant neoplasms (3), thrombotic microangiopathy, and mitochondrial diseases (3). There have been several reports on this disease in family groups, and mutations in the CoQ2 and ACTN4 genes were found to be responsible (2,5).…”

Glomerulopatia colapsante

“…Sin embargo, existen incluso en Chile, casos de pacientes que habrían tenido remisión con lisinopril y esteroides sintéticos con un seguimiento a 3 años 7 . Inmunomoduladores como el rituximab están en experimentación en otras podocitopatías y podrían llegar a ser de utilidad en las GC 31 .…”

“…De hecho, su frecuencia ha aumentado en los centros de referencia de biopsias renales, reportándose en un estudio retrospectivo realizado en México, entre 1985 y 1995, 15% de GC en biopsias descritas como glomerulopatía focal y segmentaria 4 , similar al 10% reportado en Estados Unidos de Norteamérica 5 , lo cual probablemente se relaciona con un mayor índice de sospecha, mayor número de biopsias y lógicamente, la expansión de la pandemia del SIDA 6 . En Chile, se han reportado casos, pero no existen datos de su prevalencia 7 . En la Pontificia Universidad Católica de Chile, centro de referencia nacional en biopsias renales, la GC en su forma pura (VIH y no VIH) sigue siendo infrecuente en la casuística de biopsias, con 0 a 1 caso por año aproximadamente de un promedio de 400 biopsias renales/año, lo cual podría deberse al bajo índice de sospecha por parte de los clínicos y a una subutilización de la biopsia renal en los casos de síndrome nefrótico (Comunicación no publicada.…”