Sarcoidosis is a systemic inflammatory disorder characterized by non-caseating granulomas. Multiple organ systems may be affected, but the clinical prevalence of cardiac involvement is only five percent. These cardiac manifestations, in order of decreasing frequency, include complete heart block, ventricular arrhythmias, congestive heart failure, sudden death, first-degree heart block or bundle branch block, supraventricular arrhythmias, mitral valve dysfunction, and pericarditis . To our knowledge there have only been 2 prior case reports of pericardial tamponade as the initial presentation of sarcoidosis .
A 30 year old Caucasian male presented with 36 hours of chest pain and shortness of breath. An EKG showed diffuse ST segment elevations and mild PR depressions, but no electrical alternans. A chest x-ray and CT were significant for cardiomegaly and mediastinal lymphadenopathy. An echocardiogram revealed a moderate pericardial effusion with no evidence of tamponade. The patient was determined to have pericarditis and he was discharged on high dose NSAIDs with instructions to return in 1 week for a repeat echocardiogram. The repeat study demonstrated an enlarging effusion with impending tamponade. A pericardial window was performed with drainage of 1200ml of fluid. The pericardial pathology showed granulomatous tissue. The patient later underwent a mediastinoscopy, which identified granulomatous lymph nodes most consistent with sarcoidosis. He was initiated on steroid treatment with no recurrence of his effusion to date.
The differential diagnosis of a pericardial effusion resulting in tamponade in a patient with no known past medical history includes infectious pathogens, malignancies, connective tissue diseases, or inflammatory disorders. In our case, however, the patient’s bacterial gram stain and culture showed no growth, the AFB smear and culture demonstrated no evidence of tuberculosis, a urine histoplasmosis antigen was negative, and the giemsa stain showed no evidence of other fungal organisms. Additionally, all cytology results were negative, and a germ cell tumor was considered unlikely considering that the patient’s beta-HCG and alpha fetoprotein were within normal limits. Finally, all connective tissue disease markers were also negative. Given that the patient’s mediastinal lymph node biopsy showed granulomatous tissue and he responded well to steroids, a presumed diagnosis of sarcoidosis was made. Cardiac tamponade related to sarcoidosis is uncommon. A review of the literature reveals ten prior case reports . Each individual in the described cases had biopsy results consistent with sarcoidosis. The gender distribution was largely female, with only one of the ten cases describing a male patient. The ages of these patients ranged between 27 to 59 years. Eight of the ten patients had a more insidious disease course prior to the development of a clinically significant pericardial effusion. These eight patients had either symptoms and chest x-ray findings consistent with sarcoidosis, or a histological confirmation of the disease between two months and five years prior to development of tamponade . Conversely, only two of the ten reports described cases in which there was no lag time between the diagnosis of sarcoidosis and the presentation of tamponade. To our knowledge, our case represents only the third report in the literature in which tamponade was the initial presentation of sarcoidosis.
Clinically recognizable cardiac involvement in sarcoidosis is rare. Furthermore, sarcoidosis related tamponade is extremely unusual. Our case provides insight into an exceedingly rare example of a male patient presenting with cardiac tamponade as his initial manifestation of sarcoidosis. An awareness of this potential complication may better enable physicians in managing patients with sarcoidosis.
Jorge Guerrero Espinosa, No Financial Disclosure Information; No Product/Research Disclosure Information