Abstract
A 29-year-old female with adult-onset Still’s disease (AOSD) presented with progressive shortness of breath both on rest and on exertion, increased abdominal girth, and swelling in both legs. She was on oral prednisone and was recently started on canakinumab (interleukin-1 antagonist) for joint pain and rash of AOSD. Echocardiogram showed severely dilated right ventricle, dilated pulmonary artery, moderately reduced right ventricular systolic function, but with normal left ventricular systolic function. Computed tomography with contrast ruled out pulmonary embolism. Blood tests ruled out other rheumatologic diseases. The patient was diagnosed with right-sided heart failure likely secondary to AOSD. Right heart catheterization was needed but could not be performed because of severely dilated pulmonary artery. The patient was transferred to a higher center for further management and possible cardiopulmonary transplant.
Highlights
Adult-onset Still’s disease (AOSD) is a rare inflammatory autoimmune disease that is classically described by “Still’s triad” of fever, maculopapular rash, and arthritis, but it has other atypical features like leukocytosis, elevated liver enzymes, and high serum ferritin levels.[1]
Pulmonary arterial hypertension (PAH) has been reported with connective tissue disorders like systemic lupus erythematosus and systemic sclerosis, its association with AOSD is very rare
Apart from these criteria, elevated ferritin levels can aid in the diagnosis of AOSD.[3]
Summary
Adult-onset Still’s disease (AOSD) is a rare inflammatory autoimmune disease that is classically described by “Still’s triad” of fever, maculopapular rash, and arthritis, but it has other atypical features like leukocytosis, elevated liver enzymes, and high serum ferritin levels.[1]. The minor criteria satisfying the diagnosis of AOSD were cervical lymphadenopathy on presentation, abnormal liver function tests (raised alkaline phosphatase to 138 U/L, aspartate aminotransferase of 75 U/L, and alanine aminotransferase of 60 U/L), a negative antinuclear antibody, and a negative rheumatoid factor. She met 5 criteria of AOSD with 2 major and 3 minor criteria. Workup for other rheumatologic conditions including systemic lupus erythematosus, systemic sclerosis, and CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome was negative She was initially started on prednisone followed by conventional disease-modifying antirheumatoid drugs with little benefit.
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