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2018 ; 13
(4
): e0193274
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Acute glomerulonephritis with large confluent IgA-dominant deposits associated
with liver cirrhosis
#MMPMID29634718
Hemminger J
; Arole V
; Ayoub I
; Brodsky SV
; Nadasdy T
; Satoskar AA
PLoS One
2018[]; 13
(4
): e0193274
PMID29634718
show ga
BACKGROUND: Small glomerular IgA deposits have been reported in patients with
liver cirrhosis, mainly as an incidental finding in autopsy studies. We recently
encountered nine cirrhotic patients who presented with acute proliferative
glomerulonephritis with unusually large, exuberant glomerular immune complex
deposits, in the absence of systemic lupus erythematosus (SLE) or monoclonal
gammopathy-related kidney disease. Deposits were typically IgA
dominant/codominant. Our aim was to further elucidate the etiology, diagnostic
pitfalls, and clinical outcomes. METHODS: We present clinical features and kidney
biopsy findings of nine cirrhotic patients with an unusual acute immune complex
glomerulonephritis. We also identified native kidney biopsies from all patients
with liver cirrhosis at our institution over a 13-year period (January 2004 to
December 2016) to evaluate presence of glomerular IgA deposits in them (n = 118).
RESULTS: Six of nine cirrhotic patients with the large immune deposits had a
recent/concurrent acute bacterial infection, prompting a diagnosis of
infection-associated glomerulonephritis and treatment with antibiotics. In the
remaining three patients, no infection was identified and corticosteroids were
initiated. Three of nine patients recovered kidney function (one recovered kidney
function after liver transplant); three patients developed chronic kidney disease
but remained off dialysis; two patients became dialysis-dependent and one patient
developed sepsis and expired shortly after biopsy. Within the total cohort of 118
patients with cirrhosis, 67 others also showed IgA deposits, albeit small; and 42
patients had no IgA deposits. CONCLUSIONS: These cases provide support to the
theory that liver dysfunction may compromise clearance of circulating immune
complexes, enabling deposition in the kidney. At least in a subset of cirrhotic
patients, a superimposed bacterial infection may serve as a "second-hit" and lead
to acute glomerulonephritis with exuberant immune complex deposits. Therefore, a
trial of antibiotics is recommended and caution is advised before
immunosuppressive treatment is offered. Unfortunately, most of these patients
have advanced liver failure; therefore both diagnosis and management remain a
challenge.
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