We report the case of a 72-year-old man diagnosed by the anti-Jo1 ASS rheumatology service positive without myosytic involvement with impaired renal function.
The patient had a history of hypertension, interstitial neuropathy, moderate mitral regurgitation and left ventricular hypertrophy with birefringence of the septum.
In a previous admission to the cardiology department, subcutaneous fat biopsy was performed with suspicion of amyloidosis, which was negative.
At the present admission, he was referred for renal failure study with creatinine of 2.9 mg/dl, proteinuria of 1.25 mg/24 h, without other biochemical alterations.
The physical examination revealed telangiectasias in the eyelids and erythematous-glandic cutaneous lesions hyperkeratozoa and interlocking palatal signs on the metatarsals.
The rest of the physical examination was normal.
The study of autoimmunity (ANA; negative ANCA; normal C3 and C4), immunofixation in blood and urine and proteinogram, without evident alterations were completed.
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A chest X-ray showed cardiomegaly due to atrial septal defects and cystural thickening with right costophrenic angle.
Abdominal ultrasound showed a right kidney of 9.2 cm with echogenic cortical and a small simple cyst of 1 cm in the lower pole.
The left kidney was 9.3 cm, with similar characteristics.
In the upper pole there was a simple cyst of about 3 cm, and in the lower pole another of 1.6 cm, with a smoking calcified inside.
A renal biopsy was performed to establish the diagnosis, sclerotic glomeruli in 9 cases.
Amorphous deposits were also detected in glomerulus, vessel and interstitium, Congo red positive and amyloid AA (HQ) + predominantly perivascular glomerulus-interstitial, nodular distribution.
The pathological diagnosis was AA type renal amyloidosis with glomerular, interstitial and mainly vascular involvement.
