Chronic Granulomatous Disease Associated with Chronic Glomerulonephritis

Abstract

A boy with chronic granulomatous disease (CGD) developed glomerulonephritis at the age of 12 years. The glomerulonephritis progressed to terminal uraemia at age 15 when maintenance haemodialysis was started. The clinical course was complicated by pulmonary aspergillosis and Pseudomonas septicaemia from which he eventually died. The glomerulonephritis was of unknown origin, and a possible relationship between CGD and glomerulonephritis is discussed.

Glomerulonephritis associated with chronic granulomatous disease and systemic lupus erythematosus

“…The case described by van Rhenen et al [7] deteriorated rapidly and reached end-stage renal disease within 4 months but recovered, at least partly, after 7 months on dialysis treatment. A partial recovery was also reported in the other cases except in that of Frifelt et al [9] who died after 15 months on dialysis. The immunosuppressive treatment applied in that case was repeatedly followed by pulmonary infiltrations due to Aspergillus sepsis and was therefore discontinued.…”

“…Besides focal proliferative and sclerotic lesions, diffuse glomerular involvement was observed as in our case. Granulomas of the kidney were noted in only one case [9], Other forms of renal granulomatous disease seem to be excluded. The presence of diffuse immunoglobulin and complement deposits in kidney or skin biopsies [6,7,10] suggests an immune-complex nephritis.…”

“…loma formation, eventually leading to strictures and renal abscesses [6]. In contrast, glomerular lesions have been described in only six cases of CGD aged 3-18 years [6][7][8][9][10]. The initial features were usually mild, consisting of proteinuria and microscopic haematuria.…”

Diabetes, Renal and Cardiovascular Disease in p47 phox−/− Chronic Granulomatous Disease

“…Primary renal disorders also appear more common in p47 phox deficient than in X-linked CGD. Interstitial nephritis [11–13], ESRD due to Henoch-Schonlein nephritis and associated 18q syndrome [14], immune complex glomerulonephritis associated with staphylococcal liver abscess [11, 15], FSGS [16, 10], papillary necrosis [11, 16, 17], nephrocalcinosis [10], and renal amyloidosis [18–21] have all been reported with most occurring in those with p47 phox deficiency [14–16, 22]. Renal transplant has also been successful in p47 phox deficient CGD patients with ESRD [10].…”

Renal Transplantation in a Patient With Chronic Granulomatous Disease: Case Report

“…The etiology is often multifactorial, attributed to the infections, amyloidosis, and necessary use of nephrotoxic anti-infective agents. Additionally, nephrotic-range proteinuria may occur, and native renal kidney biopsies may demonstrate a variety of histologies, including focal segmental glomerulosclerosis, membranous glomerulopathy (unpublished observations), chronic glomerulonephritis [2], and Henoch-Schonlein nephritis [3]. Regardless of etiology, renal replacement therapy is required in many patients, and the occurrence of chronic life-threatening opportunistic infections suggests that immunosuppression after renal transplantation could be contraindicated.…”