Matters of the Heart: Rare Cardiac Complications of Rheumatic Disease

Doss JR, England JH, and Fuchs HA. Coughing up blood: Behçet’s disease.Am J Med (2014) Online first, February 26, 2014, doi: 10.1016/j.amjmed.2014.02.006

Fathi AT, Dec, GW, Richter JM. et al. Case 7-2014 - A 27-Year-Old Man with Diarrhea, Fatigue, and Eosinophilia. NEJM (2014)370:861-872  doi: 10.1056/NEJMcpc1302331

Two diagnostic puzzlers in the major journals last week involved rare cardiac complications of rheumatic disorders. The first involves a patient with Behçet’s disease who presented with vascular complications (which are common in Behçet’s disease ) as well as intracardiac thrombus (which is rare).

Cardiac complications have been reported in 5-10% of Behçet’s patients. In a case series, 1-18% of Behçet’s patients had pulmonary artery aneurysms, and of those, 93% would have hemoptysis. Rupture of aneurysms leads to mortality as high as 50% within 10 months. Early recognition and treatment has markedly improved mortality, with 10-year survival of about 62%.

The presence of large pulmonary aneurysms with intracardiac thrombi poses a dilemma. Because of the aneurysms, the thrombi can’t be treated with anticoagulation. Retrospective studies recommended anticoagulation only if thrombi don’t resolve with immunosuppression. Furthermore, thrombi associated with Behçet’s are generally well-organized and firmly adherent to the vascular lumens.

This patient was treated with prednisone and chlorambucil. His clinical condition improved within days, improved further at 1 month, and the pulmonary aneurysms showed marked improvement at 6 months. The intracardiac thrombus resolved.

In the second case study, a 27-year-old man with diarrhea, fatigue and eosinophilia was diagnosed with idiopathic hypereosinophilic syndrome.

This patient had ulcerative colitis, which was under control. The colonic mucosa were abnormal, but not characteristic of ulcerative colitis. An extensive investigation explored a wide variety of possible explanations for his symptoms, including infection, solid tumors, myeloid and nonmyeloid hematological tumors, drugs, and other allergic and rheumatological conditions.

This case had many characteristics of chronic eosinophilic leukemia, but neoplasm was ruled out because the bone marrow biopsy showed no genetic abnormalities, establishing idiopathic hypereosinophilic syndrome as the diagnosis of exclusion.

Importantly, morphologic abnormalities in eosinophils, such as hypogranulation and nuclear hyperlobation or hypolobation, are frequently seen in reactive eosinophilias and should not be considered proof of clonality, the authors warn.

Five-year survival of ≤80% has been reported, with cardiac sequelae the main cause of death. Glucocorticoids are the treatment of choice; a poor response can be followed with hydroxyurea or vincristine. Biologicals are used in appropriate subsets of patients. Eosinophils express CD52, and the anti-CD52 antibody alemtuzumab has shown efficacy.

This patient developed eosinophilic myocarditis. Cardiac function deteriorated from left ventricular ejection fraction (LVEF) of 71% on the first hospital day to 26% four weeks after admission. A CT scan suggested subendocardial hypoperfusion and microvascular compromise. Warfarin was used to treat mural thrombi, and then vincristine after an inadequate response.

The patient finally got a hematopoietic stem-cell transplantation. After two years, he does not require immunosuppressive medications, has no graft vs host disease, and has been able to return to business school.