Abstract
Tubular occlusion from red blood cell casts secondary to immunoglobulin A nephropathy (IgAN) is a rare, serious complication of tonsillitis that can cause acute renal failure, also referred to as acute kidney injury. IgAN is the most common primary glomerulonephritis with up to 20% of cases resulting in renal failure worldwide. Tonsillectomy is an effective treatment option for patients suffering from IgAN secondary to recurrent acute tonsillitis. Tonsillectomy alone or in combination with additional medical modalities improves renal function and can have a positive effect on long-term renal survival.
1
Introduction
Tonsillitis is a common illness usually having few minor complications. Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis with up to 20% of cases resulting in renal failure. IgAN can occur as a rare, serious complication of tonsillitis and may result in renal failure. Some patients with acute recurrent or chronic tonsillitis develop exacerbations of IgAN manifesting as transient macroscopic hematuria and decreased renal function. These patients have an increased number of IgA producing cells in the tonsils . During infection (eg, tonsillitis), the tonsils increase production of underglycosylated IgA1 molecules which then travel through the blood stream and become deposited into the renal glomeruli and compromise renal function . Tonsillectomy is suggested for treatment of tonsillitis induced IgAN and improvement of overall survival.
2
Case report
A 34-year-old female presented with a three day history of fever, chills, sore throat, and 1 day of bilateral flank pain, vomiting, hematuria and oliguria (10 mL/h). Past medical history revealed multiple episodes of hematuria and oliguria preceded by tonsillitis/pharyngitis beginning in her teenage years and continuing into adulthood. These episodes would generally resolve without sequelae; however, during her first pregnancy, she developed persistent asymptomatic hematuria. Upon hospital admission, laboratory evaluation revealed a positive rapid strep test, white blood cell (WBC) 18.1 × 10 3 /mm 3 , blood urea nitrogen 27 mg/dL, and creatinine 2.7 mg/dL. Urinanalysis revealed a large amount of blood, nitrates, protein, granular casts, and WBC 10 to 15 × 10 3 /mm 3 per high-power field. She received intravenous antibiotics and fluids. Urine sediment revealed helmet cells suggestive of a glomerular origin of the hematuria. Nephrology and urology were consulted. Additional testing revealed negative results for glomerular basement membrane (BM) disease, antibody, circulating anti-neutrophil cytoplasmic antibody, Lyme IgG and IgM, and a normal angiotensin converting enzyme level. A renal ultrasound revealed nephrolithiasis and mild hydronephrosis. A kidney, ureter, and bladder x-ray and abdominal computed tomographic scan demonstrated small kidney stones. The patient elected observation and remained mildly proteinuric and hematuric.
Several days after admission, her condition failed to improve and acute kidney injury developed as evident by increasing serum creatinine and anuria. A renal biopsy was performed to help delineate a diagnosis. Histopathologic evaluation revealed glomeruli with a minimal diffuse segmental mesangial hypercellularity without crescents or necrosis. Many tubules contained large erythrocyte casts. The blood vessels were unremarkable ( Fig. 1 ). Immunofluorescent microscopy revealed 1+IgA, 2+C3 in a diffuse generalized mesangial pattern, trace C4d, and κ and λ chains in a diffuse, segmental mesangial pattern. Electron microscopy could not be performed due to limited specimen. Intravenous hydration and lasix were started in addition to calcium carbonate for hyperphosphatemia. Her renal function gradually improved, and she was discharged home after 1 week. Follow-up laboratory evaluation demonstrated normal antistreptolysin O (ASO) titer, normal C3 and C4 levels, and antibodies negative for S RNA/protein (RNP), smith (anti-Sm), anti-Promyelocytic Leukemia (PML), anti-Ro (SSA), anti-La (SSB), anti Scl-70, and anti-double stranded DNA (ds-DNA).
After suffering from multiple additional episodes of acute tonsillitis/pharyngitis followed by hematuria and oliguria over the following two years she presented to otolaryngology clinic for tonsillectomy. She underwent this procedure without complication and has not experienced recurrence of her hematuria or oliguria in over 2 years.
2
Case report
A 34-year-old female presented with a three day history of fever, chills, sore throat, and 1 day of bilateral flank pain, vomiting, hematuria and oliguria (10 mL/h). Past medical history revealed multiple episodes of hematuria and oliguria preceded by tonsillitis/pharyngitis beginning in her teenage years and continuing into adulthood. These episodes would generally resolve without sequelae; however, during her first pregnancy, she developed persistent asymptomatic hematuria. Upon hospital admission, laboratory evaluation revealed a positive rapid strep test, white blood cell (WBC) 18.1 × 10 3 /mm 3 , blood urea nitrogen 27 mg/dL, and creatinine 2.7 mg/dL. Urinanalysis revealed a large amount of blood, nitrates, protein, granular casts, and WBC 10 to 15 × 10 3 /mm 3 per high-power field. She received intravenous antibiotics and fluids. Urine sediment revealed helmet cells suggestive of a glomerular origin of the hematuria. Nephrology and urology were consulted. Additional testing revealed negative results for glomerular basement membrane (BM) disease, antibody, circulating anti-neutrophil cytoplasmic antibody, Lyme IgG and IgM, and a normal angiotensin converting enzyme level. A renal ultrasound revealed nephrolithiasis and mild hydronephrosis. A kidney, ureter, and bladder x-ray and abdominal computed tomographic scan demonstrated small kidney stones. The patient elected observation and remained mildly proteinuric and hematuric.
Several days after admission, her condition failed to improve and acute kidney injury developed as evident by increasing serum creatinine and anuria. A renal biopsy was performed to help delineate a diagnosis. Histopathologic evaluation revealed glomeruli with a minimal diffuse segmental mesangial hypercellularity without crescents or necrosis. Many tubules contained large erythrocyte casts. The blood vessels were unremarkable ( Fig. 1 ). Immunofluorescent microscopy revealed 1+IgA, 2+C3 in a diffuse generalized mesangial pattern, trace C4d, and κ and λ chains in a diffuse, segmental mesangial pattern. Electron microscopy could not be performed due to limited specimen. Intravenous hydration and lasix were started in addition to calcium carbonate for hyperphosphatemia. Her renal function gradually improved, and she was discharged home after 1 week. Follow-up laboratory evaluation demonstrated normal antistreptolysin O (ASO) titer, normal C3 and C4 levels, and antibodies negative for S RNA/protein (RNP), smith (anti-Sm), anti-Promyelocytic Leukemia (PML), anti-Ro (SSA), anti-La (SSB), anti Scl-70, and anti-double stranded DNA (ds-DNA).
