Progressive Systemic Sclerosis with the Nephrotic Syndrome and Acquired Factor X Deficiency

Abstract

A patient with progressive systemic sclerosis (PSS) involving skin, digit, esophagus, and lung developed the nephrotic syndrome and rapidly progressive renal insufficiency. Renal pathology revealed capillary collapse, cellular crescents, arteriolar hyaline deposition, and mesangial proliferation. On immunofluorescence IgM, C3, and fibrinogen were present in mesangium and capillary walls. Prebiopsy coagulation screening revealed a factor X deficiency which caused substantial prolongation of the partial thromboplastin time without an overt bleeding diathesis. The acquired factor X deficiency resolved after fresh frozen plasma and vitamin K administration, although some spontaneous improvement was noted. Nephrotic syndrome may occasionally be seen in the acute fulminant form of PSS and should not deter diagnosis of PSS.

Systemic sclerosis complicated with nephrotic syndrome: relevance with antiribosomal P antibody

“…Of note, immuno¯uorescence studies on the renal biopsy specimen of our patient disclosed granular deposition of C3 and IgM in the glomeruli, which was in accordance with observations by Palma et al [2] and Eiser et al [4]. They reported patients with SSc complicated with nephrotic syndrome who showed deposition of IgM and C3 in the glomeruli [2,4]. However, these ®ndings do not necessarily support the conclusion that the nephrotic syndrome in our patient is caused by SSc itself, since the association of both conditions was considered exceptional [2,3,4].…”

“…She had not been given any drugs that might result in this syndrome. Of note, immuno¯uorescence studies on the renal biopsy specimen of our patient disclosed granular deposition of C3 and IgM in the glomeruli, which was in accordance with observations by Palma et al [2] and Eiser et al [4]. They reported patients with SSc complicated with nephrotic syndrome who showed deposition of IgM and C3 in the glomeruli [2,4].…”

“…They reported patients with SSc complicated with nephrotic syndrome who showed deposition of IgM and C3 in the glomeruli [2,4]. However, these ®ndings do not necessarily support the conclusion that the nephrotic syndrome in our patient is caused by SSc itself, since the association of both conditions was considered exceptional [2,3,4].…”

“…Thus, chronic albuminuria of a mild degree is frequently seen in the absence of renal failure [1]. However, features of the nephrotic syndrome are exceptional and have been reported in only a few patients with SSc [2,3,4].…”

Acquired, non‐amyloid related factor X deficiency: review of the literature

“…Manifestations of acquired FX deficiency varied considerably and ranged from no observed bleeding to life threatening haemorrhage. Ten of the 34 patients (29%) had no evidence of bleeding at presentation [25,[31][32][33]38,45]. FX levels in these patients ranged from 1% [25] to 41% [38], with a mean FX level of 13.7%.…”