We carried out a computer-based search (MEDLINE; National Library of Medicine, Bethesda, MD, USA) of English language articles published from 1946 to April 2012. We used the key words “proteinuria”, “nephrotic syndrome”, “membranous glomerulonephritis”, “focal segmental glomerulosclerosis” or “minimal change disease” on one side and “Guillain–Barré syndrome”, “acute inflammatory demyelinating polyneuropathy”, “chronic inflammatory demyelinating polyneuropathy” or “neuropathy” on the other. In addition, references cited by the articles produced by the literature search and the present case were included; a total of 33 cases were analyzed.[1-3, 5-30] We provisionally classified the 33 cases by the temporal course of disease as follows: GBS, duration to nadir was within 4 weeks and no relapse;[31, 32] and CIDP, duration to nadir was 8 weeks or more and/or relapses. Two patients defined as subacute inflammatory demyelinating polyneuropathy whose durations to nadir were between 4 and 8 weeks without relapses were included in CIDP in the present review. We could not adapt the current research criteria including electrodiagnostic tests[4, 32, 34] in the present retrospective study. We classified 12 cases as GBS and 21 cases as CIDP (Tables 2-4). Statistical analysis was carried out using JMP 8 (SAS Institute Inc., Cary, NC, USA).
The reports of 33 cases originated from various countries including the USA (13 cases), Australia (four cases), Taiwan (three cases), Germany (two cases), Japan (two cases), the UK (two cases), and one case each from Barbados, India, Italy, Korea, Switzerland, Tunisia and Turkey. The mean age of onset was mid 40s and nearly 85% of cases were male. Five cases were female, and were either schoolchildren or aged over 55 years. The main neurological symptoms were motor impairments, and the sensory disturbances were usually mild. We graded the severity of the motor impairments as follows: mild, able to walk or mean scores of MMT were more than 3; and moderate to severe, unable to walk or scores of MMT were 3 or less. Durations of illness were shorter in the cases with moderate to severe motor impairments in all IDP, but the difference did not reach statistical significance (P = 0.0552, Wilcoxon rank-sum test). Antiganglioside antibodies were investigated in four cases including the present case, and no specific findings were obtained.[15, 16, 23] NCS findings were described in eight of 12 GBS cases and 18 of 21 CIDP cases. They all indicated the presence of demyelinating neuropathy. As for symptoms of nephrotic syndrome, edema was noticed in 19 cases, whereas 12 cases (36.4%) were asymptomatic. Urinary protein excretion of each case was 1.9–25 g/day. Proteinuria appeared simultaneously with neuropathy in two-thirds of the cases. In most of the CIDP cases, proteinuria appeared or worsened at the nadir of the neurological symptoms, and reduced in parallel with the neurological improvement. Proteinuria improved in 13 cases and significant proteinuria persisted in the others. There was a tendency for follow-up periods to be shorter in the latter cases (P = 0.0853, Wilcoxon rank-sum test). Thus, the proteinuria might have improved much later in a fraction of the cases. Among the 10 cases with neurological relapses, significant proteinuria also recurred in three cases. As for therapy, two or more immunomodulating therapies were carried out in half of the GBS cases and most of the CIDP cases. Initial treatments were begun with corticosteroids, IVIg or plasma exchange in most of the cases, and were sometimes ineffective or only transiently effective. Relapses of neuropathy occurred in half of the CIDP cases; however, there was only one case with more than two relapses. The overall outcomes of neuropathy were favorable in both the GBS and CIDP cases, except a patient with GBS who died of respiratory failure complicating bronchopneumonia.
Renal biopsies were carried out in 28 patients (Fig. 1). There were no relationships between the histological diagnoses of renal biopsies, and forms of neuropathy, neurological severities, number of relapses or outcomes of neuropathy and proteinuria.
Figure 1. Histological diagnoses of nephrotic syndrome in the cases of Guillain–Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP) with nephrotic syndrome. FSGS, focal segmental glomerulosclerosis; MCD, minimal change disease; MGN, membranous glomerulonephritis; others, other histological diagnoses in two cases (one case with postinfectious glomerulonephritis and one case with immunoglobulin deposition) and histological diagnoses were not available in five cases.
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