Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy

Abstract

We contend that patients presenting with Congo-red-negative fibrillary deposits on renal biopsy should be evaluated carefully for monoclonal-gammopathy and cryoglobulins, but there is insufficient published data, as yet, to justify subclassification of FG and ITG as distinct clinical entities. Indeed, we argue that it remains to be determined if FG-ITG represents a unique condition or a forme fruste of cryoglobulin- or gammopathy-associated renal disease. Although the optimal treatment for FG-ITG has not been determined, renal transplantation appears an attractive option in patients with end-stage renal failure.

Enfermedad de depósito glomerular: a propósito de un caso de glomerulonefritis fibrilar

“…El tratamiento médico actual de la glomerulonefritis fibrilar e inmunotactoide en los casos en los que no se describe enfermedad sistémica subyacente o asociada, es insatisfactorio con corticoterapia e inmunosupresión (15,18). Además, se ha ensayado el trasplante renal, observándose en la mayoría de los casos recurrencia de la enfermedad, aunque el deterioro de la función renal evolucionó invariablemente más lentamente, por lo que se considera una opción atractiva en pacientes con enfermedad renal terminal (2).…”

Immuotactoid Glomerulopathy (Fibrillary Glomerulonephritis)

“…Pronovost et al (3) found that when ITG was defined on the basis of fibril size of Ͼ30 nM, 6.5% of 186 cases reviewed had ITG but that when they defined ITG on the basis of having parallel arrangement, the prevalence doubled (12%). Second, in one of the largest and most thorough reviews on the topic to date, Pronovost et al (3) found no difference in the clinical features (hypertension, hematuria, nephrotic syndrome, or renal insufficiency) at presentation when the diagnosis of ITG was subdivided on the basis of differences in fibril size (Ͼ30 versus Յ30 nM) or arrangement (random versus parallel bundles). They also recognized that any association with a lymphoproliferative disorder very much depended on the criteria used for study.…”

“…Patients with ITG range in age from 10 to 81 yr, but, on average, they have been 44 yr of age at presentation (3,4,6,7,9). More than 90% of the patients are white, and men compose from 47 to 61% of cases (3,6,11).…”

“…Hypertension and microscopic hematuria are common and present in Ͼ65 and Ͼ70% of patients, respectively. Renal insufficiency is seen at the time of diagnosis in Ͼ45% of patients (3,6,9).…”

“…In the past 25 yr, Ͼ200 cases of ITG have been reported (3)(4)(5). Various synonyms have been used to refer to the lesion that is described in these reports, including fibrillary glomerulonephritis (FGN), nonamyloidotic fibrillary glomerulopathy, amyloid-like glomerulopathy, and amyloid stain-negative microfibrillary glomerulopathy, but we believe that they all represent the same or a similar disease process (6 -8).…”