Case Presentation: A 66 year old female with a history of RA, diabetes mellitus, and deep vein thrombosis, presented to ED with tachycardia and hypoxic respiratory failure requiring 5 liters of oxygen. Physical exam was notable for somnolence, decreased bilateral lower lobe breath sounds, swan neck deformity of the digits, and ulnar deviation. Chest radiograph revealed bilateral pleural effusions. Echocardiogram revealed an ejection fraction of 5-10% with global hypokinesis and a mild to moderate pericardial effusion, without evidence of tamponade. The electrocardiogram was without significant changes, troponin T was undetectably low on three occasions, and brain natriuretic peptide was elevated at 6176 pg/ml. The c-reactive protein and erythrocyte sedimentation rate were both notably elevated at 4.4 mg/L and >120 mm/hr, respectively. The patient was diagnosed with RA in 2009 and has since been non-compliant with her immunosuppressant therapy. During this admission she had high disease activity involving more than 8 bilateral small joints of hand: MCPs, PIPs and DIPs with swan neck deformity, and ulnar deviation. She was admitted to the ICU and started on solumedrol 40 mg i.v. BID due to suspicion for inflammatory myocarditis. Additionally, she was started on milrinone i.v. and diuretics for heart failure. A nuclear stress test was performed and was negative for ischemia. Thoracentesis revealed a transudative effusions, likely secondary to her heart failure. The evaluation of viral cardiomyopathy revealed negative antibodies to Epstein-Barr Virus, Cytomegalovirus, and Coxsackie. We obtained a repeat echocardiogram with strain analysis 4 days after her last echocardiogram and the initiation of corticosteroids. The echocardiogram showed a dramatically improved ejection fraction of 51% with only mild hypokinesis. The patient was transitioned to prednisone PO 40mg daily prior to discharge without further evidence of ongoing heart failure.
Discussion: There are very few cardiomyopathies which will reverse as rapidly as this patient’s disease did. In this case, her severe systemic inflammatory state from uncontrolled RA led to an acute inflammatory cardiomyopathy which responded impressively to corticosteroids. This case represents the importance of early recognition of RA-related myocardial dysfunction and effectiveness of early steroid treatment. The diagnosis of RA-related heart failure is challenging given its rarity and the overlap with other heart failure etiologies on clinical presentation. The most common RA-associated cardiac manifestation is pericarditis/pericardial effusion, however clinicians should be aware that RA can also cause an acute inflammatory cardiomyopathy.
Conclusions: Rheumatoid arthritis (RA) is an inflammatory disease involving primarily the synovial tissue of joints. Depending on the severity of inflammation, it can present in a myriad of ways. Non-articular manifestations occur in about 40% of patients with RA over a lifetime, but RA involving the heart and presenting as a heart failure is very rare. We present a case of a severe acute inflammatory cardiomyopathy secondary to uncontrolled RA, which rapidly and dramatically improved with corticosteroids.