A 73-year-old man with a history of hypertension treated with valsartan and hydrochlorothiazide and essential thrombocythaemia on therapy initially with hydroxyurea and then anagrelide.
By mid-December 2007 she consulted because for 8 months fatigue, asthenia, adynamia, night sweats, dyspnea on exertion and frequent dry cough were common non-compliance.
She had no fever, weight loss or pruritus.
The electrocardiogram and chest X-ray were normal, except for moderate aortic elongation and the echocardiogram showed mild aortic insufficiency, auricle and left ventricle with preserved size and function.
Hematocrit 29%, macrocytosis, rouleaux ++, ESR 49 mm/hour and platelets 717,000 mm3 stood out in the blood count.
General examinations were normal.
One week later, he presented with hemoptoic sputum and was hospitalized for suspected pulmonary embolism.
A chest CT angiography was performed, which ruled out thrombi but showed that the wall of the ascending aorta, over the aortic valve plane, was covered by a hypodense tissue extending through the inferior aorta month.
Left subclavian, renal and superior mesenteric arteries (SMA) also had a similar halo.
The renal arteries and mesentery were stenosed, the celiac trunk presented post-stenotic dilatation and the blood flow by the SMA was culprit.
There was a slight pericardial effusion, enlarged spleen of 15 cm and bilateral perinephric edema.
The renal scintigraphy showed decreased perfusion and left kidney function (RD 62%, IR 38%).
Neck vessels were normal on ultrasound.
In angioresonance, the periaortic tissue was reinforced with gadolinium concluding that it was an inflammatory-fibrous tissue.
Critical SMA stenosis; both left renal arteries and celiac trunk presented 70% stenosis and right renal artery and inferior mesenteric stenosis 50%.
The renal sinuses and perinephric areas were occupied by the tissue surrounding the aorta.
Stents were installed in SMA and both renal arteries, improving perfusion.
The radiologist proposed ECD.
The bone scintigraphy was compatible with this diagnosis, showing an increase in the uptake in both tibias.
Treatment was initiated with prednisone 60 mg/day and methotrexate 10 mg weekly, with symptomatic improvement, so the dose of corticosteroids was gradually reduced to 10 mg. Three months later (March 2008) he had a good response to antibiotic treatment.
Twelve months after diagnosis (May 2008) and the patient was practically asymptomatic, except for edema associated with the use of anagrelide, the patient remained anemic and had leukocytosis.
A scanner showed increased thickness of the periaortic tissue in both ascending and descending aorta and prolongation to the onset of idiopathic iliac arteries.
At 18 months (July 2009), due to knee pain, radiographs showed osteocalcin osteocalcosis in the same location as tibias, which were compatible with alterations in the cycthyrogram and cartidrogus.
A new scanner showed persistent previous findings and increased (70%) in stenosis of the inferior mesenteric artery.
At 28 months of diagnosis (April 2010), the patient began to present signs of congestive heart failure.
The scanner showed thickening of the perirenal tissue, thickening of the bile ducts and increased amount of fluid in the pericardium.
Echocardiography showed a large pericardial effusion with initial signs of tamponade and pericardium irregular surface suggestive of tumoral inversion.
The electrocardiogram showed low voltage.
The patient was hospitalized and a pleuroperipheral window was created.
During the operation, the tension pericardium was found draining 1,200 ml of a brown yellow liquid.
The parietal pericardium was normal and the visceral pericardium was diffusely infiltrated by hard tissue.
Pericardial fluid had histiocytes, as well as the resected parietal pericardial tissue, which had abundant CD-100 granular and foamy cytoplasm cells, and immunohistochemical analysis confirmed that these cells corresponded to CD-100 (AU)
The patient had a clear improvement of symptoms after the procedure.
