Renal amyloidosis in a patient with X-linked agammaglobulinemia (Bruton's disease) and bronchiectasis.

OBJECTIVE

We present a patient with Bruton's disease and bronchiectasis who developed renal AA amyloidosis.

METHODS

A 38 year-old man was diagnosed with X-linked agammaglobulinemia (Bruton's disease) when he was 3 years old, and he has been treated with parenteral immunoglobulin since then. Eighteen years later, he was diagnosed with central pulmonary bronchiectasis by computerized tomography (CT). In 2008, he gradually developed anemia, edema of lower limbs, and loss of weight.

RESULTS

Laboratory studies revealed deterioration of renal function, normocytic normochromic anemia and nephrotic range proteinuria. Hepatitis B and C and HIV serology were negative. Ultrasound and CT of abdomen were normal. A renal biopsy revealed deposits with positive PAS and Congo red staining in glomeruli, interstitium, and vessel's walls. Immunohistochemistry showed positive staining of the A amyloid. Direct immunofluorescence was positive with thioflavin and showed focal and glomerular mesangial IgG deposits, suggesting renal AA amyloidosis. For 2 years the patient conducted pharmacological treatment and follow-up for the Nephrology department with poor prognosis and progression of renal function impairment. In January 2011 he began dialysis treatment with improvement, and he is currently on the waiting list for renal transplantation.

CONCLUSIONS

We present a patient with Bruton's disease and bronchiectasis who developed renal AA amyloidosis a finding rarely reported.