ANA-Negative Hydralazine-Induced Pericardial Effusion

Abstract

This case describes a patient with pericardial effusion as a phenomenon of the drug-induced lupus erythematosus (DILE) syndrome due to hydralazine. The relevance of this case report lies in the fact that although hydralazine has been a known causative agent of DILE, its presentation may involve a negative anti-nuclear antibody (ANA) study. Pericardial effusion is a documented adverse effect as a result of hydralazine use. It is typically common to screen for DILE with the serum ANA test prior to proceeding to more costly and specific tests (i.e., anti-histone antibody). As per our literature review, this is the second case of hydralazine causing DILE with a negative ANA. As in our case, although the screening serum ANA is the initial next best step for suspicion of DILE by hydralazine, it is important to consider the diagnosis without ANA positivity.

Antinuclear antibody-negative lupus? An ominous presentation of hydralazine-induced lupus syndrome

“…HILS should be suspected in any patient on hydralazine presenting with unexplained effusion; moreover, in our patient, additional laboratory evidence revealed the presence of active lupus, including low C3, elevated ESR and CRP, pancytopenia, negative anti-dsDNA, and positive anti-histone antibody ( 5 ). We believe the clinical picture and laboratory findings of our patient were consistent with the presentation of HILS with negative ANA ( 3 , 4 ).…”

“…Infrequently, serositis leading to pericardial and pleural effusions can develop. Several case reports have described HILS presenting with pericarditis and/or cardiac tamponade ( 1 – 4 ). Following the African American Heart Failure trials that demonstrated the mortality benefit of BiDil (combination of hydralazine and isosorbide dinitrate), hydralazine is more frequently prescribed than ever before ( 7 , 9 ), and the incidences of HILS are likely to rise.…”

“…In up to 95% cases, HILS presents with positive ANA, positive anti-histone antibody, and negative anti-dsDNA antibody ( 5 , 7 ). Based on the current literature search, this manuscript is the third of its kind to report ANA-negative HILS ( 3 , 4 ). Our patient and the two other previous reported cases presented similar pericardial effusions.…”

“…HILS can present vague symptoms, including arthralgia, myalgia, fever, anorexia, and fatigue. Cutaneous and serosal involvements are infrequent; rarely, pericardial or pleural effusions can be the sole manifestation of the disease ( 1 – 4 ). Positive antinuclear antibody (ANA) serology is the standard for SLE and DILS diagnosis.…”

“…Positive antinuclear antibody (ANA) serology is the standard for SLE and DILS diagnosis. In the literature, only two cases so far have described an unusual presentation of HILS with negative ANA ( 3 , 4 ). In both these cases, patients presented with pericardial effusion.…”