76-year-old man.
The patient was admitted to the emergency department due to pleural effusion with signs of tamponade.
The visceral pericardium was thickened, hard and irregular, with the appearance of “I remained confident”.
1,200 ml of liquid containing abundant histiocytes was drained.
The parietal pericardium biopsy showed granular and foamy cytoplasm histiocytes whose immunohistochemical analysis showed positivity for CD68 and negativity for CD1a and S100 protein, characteristic EEC.
1.
Twenty-eight months before, the clinical diagnosis of CSE had been made due to the general state of the disease, anemia, high ESR, hemoptysis, presence of a non-confirmed balloon-like renal artery disease involving stenosis
There were also typical signs of chondrocalcinosis in the knees.
Since then she was being treated with methotrexate (MTX) 10 mg/week and prednisone 10 mg/day.
The history of this patient up to the time of cardiac taping was previously published in this same journal22.
drainage, CHF partially improved, remaining in functional capacity II-III (NYHA).
Days later she developed atrial fibrillation that reverted with amiodarone.
In June 2010, a scanner showed a tissue covering the coronary arteries and pericardial thickening with little effusion.
In view of disease progression under MTX and publications reporting that when cardiac involvement was present, the therapy of choice for CSE with interferon was often inefficient in controlling the disease, sirolimus was prescribed in combination with a recent disease.
The patient accepted its use.
Tolerance was good except occasional episodes of diarrhea.
In the following months, sirolimus was reduced to 1 mg/day achieving therapeutic levels (5 to 8 ng/ml).
He also received anagrelide (for essential thrombocythaemia), valsaltan, furosemide, hydrochlorothiazide and citalopram.
1.
In September 2010, an echocardiogram showed absence of pericardial effusion, normal left ventricular systolic function with abnormal relaxation.
Following anemization, panendoscopy revealed a hyperplastic and pull-through powder with evidence of bleeding.
She was treated locally and with chemotherapy.
1.
In November 2010, hypogonadotrophic hypogonadism and hypothyroidism secondary to hypopituitarism were diagnosed.
Magnetic resonance imaging showed pituitary abnormalities compatible with histiocytosis.
There were no other lesions in the central nervous system.
Testosterone and thyroxine were prescribed, with significant improvement in general condition.
Between 2011 and 2013, there were no variations in cardiac function, except for one episode of acute pulmonary edema.
This was associated with sudden weight gain due to excessive salt intake in the diet and reduction in the prescribed furosemide dose.
Physical examination revealed dyspnea at rest, orthopnea, wheezing, large jugular engorgement, galloping, and diffuse bilateral crypts in both lung fields.
He was treated at home with abundant doses of furosemide and isosorbide nitrate, returning within a few days to his basal condition.
Never had angina despite pericoronary involvement.
1.
During the first 4 years of sirolimus therapy there were no relevant side effects except intermittent diarrhoea.
Mild hypertriglyceridemia, renal function, minimal proteinuria and alterations attributed to thrombocythemia and anagrelide were observed.
In early 2014, an ulcer appeared on the lateral face of the neck that expanded rapidly, diagnosed as a squamous cell carcinoma.
An echocardiogram showed no progression of her heart disease.
In October 2014 the lesion was resected under general anesthesia.
In the immediate postoperative period, there was profuse bleeding in the operated area with hypotension, requiring transfusion of several units of red blood cells and platelets.
She had to be reoperated to control the bleeding on two occasions, which was achieved after a new platelet transfusion.
The generous contribution of volume and blood products was complicated by acute pulmonary edema.
She was connected to mechanical ventilation and furosemide, noradrenaline and dobutamine were administered.
Despite this, she developed persistent hypotension and anuria.
Her family, considering her underlying pathology, did not accept the proposition of invasive hemodynamic monitoring or dialysis with ultra-high.
The patient died two days later in cardiogenic shock at 80 years, 82 months after the clinical diagnosis of CSE, 54 months after the histological diagnosis and 52 months after the onset of sirolimus.
