A Unique Presentation of AA Amyloidosis
- Glomerular Diseases: From Inflammation to Fibrosis - III
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1401 Glomerular Diseases: From Inflammation to Fibrosis
- Chatterjee, Totini S., Baylor College of Medicine, Houston, Texas, United States
- Tchakarov, Amanda, The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, Texas, United States
- Dinarvand, Peyman, Baylor College of Medicine, Houston, Texas, United States
- Walther, Carl P., Baylor College of Medicine, Houston, Texas, United States
While the overall prevalence of kidney amyloidosis in native kidney biopsies performed globally is 1.6%, in the United States, only 7% of these cases are due to AA amyloidosis. We describe a unique multisystem presentation of AA amyloidosis.
A 64 year-old Pakistani woman with hypertension and goiter presented to the hospital for five days of abdominal pain and watery diarrhea, in addition to several weeks of worsening fatigue. She had goiter and tachycardia on exam, and laboratory studies demonstrated hyperthyroidism and non-oliguric kidney failure with 8.8 g protein in a 24 hour urine collection. Thyroid ultrasound showed diffuse fatty infiltration; kidney and urinary tract ultrasound was unremarkable. Kidney histology showed glomerular mesangial expansion with eosinophilic matrix and substantial fibrosis. Congo red stain revealed amyloid deposition in glomeruli, vessels, and the tubulointerstitium, identified as amyloid A with immunohistochemistry. Thyroid histology showed adipocytes with entrapped benign thyroid follicles (diffuse lipomatosis) and amyloid deposits in vessel walls by Congo red stain.
AA amyloidosis is caused by extracellular deposition of serum amyloid A protein—a hepatic acute phase reactant—in tissues, and can occur in many chronic inflammatory conditions. Proteinuric kidney disease is a common presentation of AA amyloidosis; amyloid goiter is an uncommon presentation and associated hyperthyroidism is rare.Our patient’s presentation with amyloid goiter, hyperthyroidism, and kidney failure has not previously been reported to our knowledge. Treatment of AA amyloidosis largely depends on identification of an inciting chronic inflammatory disease; however, extensive workup did not reveal a cause in our patient. She was initiated on hemodialysis for kidney failure and treated with beta blockade and methimazole for hyperthyroidism.