ABSTRACT
La calcifilaxis ha sido definida como una sensibilidad a la calcificación de los tejidos, descrita primariamente en pacientes con insuficiencia renal crónica, trasplante renal o disfunción de glándulas paratiroides. Existen pocos casos descritos de pacientes con artritis reumatoide, sin insuficiencia renal, ni disfunción paratiroidea. Nosotros presentamos otro caso en una mujer con artritis reumatoide inactiva (AU)
Calciphylaxis has been defined as tissue sensitivity to calcification, described mainly in patients with chronic renal insufficiency, renal transplant, or parathyroid dysfunction. There are few cases described in patients with rheumatoid arthritis that do not suffer from renal failure or hyperparathyroidism. Another case is presented of calciphylaxis in a woman with inactive rheumatoid arthritis (AU)
Subject(s)
Humans , Female , Middle Aged , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/drug therapy , Calcinosis/complications , Calcinosis/drug therapy , Necrosis/complications , Necrosis/drug therapy , Vasculitis/drug therapy , Methotrexate/therapeutic use , Prednisone/therapeutic use , Biopsy/methods , Pressure Ulcer/complications , Pressure Ulcer/diagnosis , Vasculitis/complications , Renal Insufficiency, Chronic/complications , Renal Insufficiency, Chronic/diagnosis , Pressure Ulcer/drug therapy , Calcium/therapeutic use , Vitamin D/therapeutic useABSTRACT
Erdheim-Chester disease, although rare, has a wide range of manifestations. It is characterized by the xanthomatous infiltration of tissues by spumous histiocytes, surrounded by fibrosis. The symptoms can vary from bone pain, diabetes insipidus, exophthalmos, xanthelasmas, cardiovascular involvement, bilateral adrenal enlargement, renal impairment, testis infiltration, interstitial lung disease to retroperitoneal fibrosis with perirenal and/or ureteral obstruction. We present eight cases, four of them with only breast involvement and the others with bone, cardiovascular, central nervous system, and renal involvement. All showed infiltrates of histiocytes and fibrosis on microscopic evaluation and positive CD68 and negative CD1a on immunohistochemical stains.
Subject(s)
Abdomen/diagnostic imaging , Erdheim-Chester Disease/diagnostic imaging , Erdheim-Chester Disease/pathology , Adult , Argentina , Female , Fibrosis , Humans , Male , Middle Aged , Tomography, X-Ray ComputedABSTRACT
Calciphylaxis has been defined as tissue sensitivity to calcification, described mainly in patients with chronic renal insufficiency, renal transplant, or parathyroid dysfunction. There are few cases described in patients with rheumatoid arthritis that do not suffer from renal failure or hyperparathyroidism. Another case is presented of calciphylaxis in a woman with inactive rheumatoid arthritis.
Subject(s)
Arthritis, Rheumatoid/complications , Calciphylaxis/etiology , Calciphylaxis/diagnosis , Female , Humans , Middle AgedABSTRACT
An 82-year-old woman came to consultation with sudden visual loss in her left eye. Fifteen days before, she complained of diplopia. She had doubtful symptoms of giant cell arteritis and showed a normal physical exam. Lab results showed erythrosedimentation rate (ESR) = 62 mm/1°h; uremia = 0.56 g/dl (normal <0.45); serum creatinine = 1.7 mg% (normal <1.4); low calcium and phosphorus; and normal urine calcium and serous PTH. Fundus exam and brain magnetic resonance imaging (MRI) showed normality of optic nerves, chiasma, retrochiasmatic area, ocular muscles, eyeballs, lacrimal glands, periorbital fat, cavernous sinuses, and occipital cortex. A temporal arteritis was suspected; therefore, a biopsy was carried on. It showed the presence of large calcium deposits in the artery's tunica media and internal elastic lamina, with narrowing of the lumen, with no inflammation and multinuclear giant cells. Histological diagnosis is calciphylaxis. Although calciphylaxis is a well-described entity that occurs in end-stage renal patients, many cases are due to non-uremic causes. To date, there are only six cases described in literature of calciphylaxis mimicking GCA.
