3) A 41-year-old woman, ex-smoker, with a history of ulceration secondary to ingestion of non-steroidal gastric disease, diffuse goiter with multinodular goiter diagnosed two years before and with biopsy compatible with chronic amyloid kidney disease.
Surgical interventions: cystectomy, trabeculoplasty of both eyes for glaucoma and right oophorectomy for ovarian cyst torsion.
From the age of 6 months, episodes of cold-induced urticaria of 24-48 hours of evolution (peak of attack at 2-3 hours) with habonosal-type lesions, which left a common erythematous, cold-type
In recent episodes, abdominal discomfort with nausea and anorexia
The symptoms required rest and progressively regressed with the help of antipyretics and corticosteroids.
In recent years, symptoms were more intense and disabling, with an increase in the number of episodes overlapped until few symptom-free periods were found.
In addition, hearing loss has decreased in recent years (hearing hearing loss persistent bilateral with significant decrease in acute).
Family history
Hija with mutation CIAS/NALP3 (frigerated familial adenoma syndrome).
Madre, uncle and maternal grandfather with urticaria, arthralgia-arthritis, conjunctivitis and cold-associated flu-like syndrome, with grandfather and renal failure.
CdL included creatinine 2.2 mg/dl; urea 54 mg/dl; creatinine clearance: 31.4 ml/min; albumin/m2; hemoglobin: 21.8 mg/dl; IgM 384 mg/min.
Total proteins, total cholesterol, liver function tests, creatine phosphokinase, IgA, IgG, C3, normal thyroid hormones, negative antinuclear antibodies
Urine: 5-7 leukocytes/field; proteinuria: 180 mg/24 hours.
Echocardiogram showed no abnormalities.
Abdominal ultrasound: preserved kidneys, diffuse increase in echogenicity.
It was decided to perform a renal biopsy showing 26 glomeruli, of which 17 are sclerosed.
In some of the non-sclerosed glomeruli very focal nodular deposits of hyaline eosinophilic material, acellular and weakly peryodic acid (PAS+) are also observed in vascular wall material.
This material is stained with the Congo red technique, staining that disappears after treatment with permanganate, and shows brownish green birefringence with polarized light.
Immunohistochemical techniques showed amyloid A staining in the acellular material. Moderate tubular fibrosis and interstitial fibrosis (20
With these histological data, the diagnosis of secondary renal amyloidosis (AA) with glomerular, vascular and interstitial involvement is made.
1.
A total thyroidectomy was performed in another hospital and the histological study also showed the presence of abundant amyloid deposits between thyroid follicles.
