Chronic Gvhd-Associated Serositis and Pericarditis

BACKGROUND:  Serositis and pericarditis are rare manifestations of chronic GVHD. No risk factors or predictive biomarkers associated with this syndrome have been recognized to date, and outcomes have not been described in large series.

METHODS:  In an effort to better characterize this condition, we searched our institutional database of allogeneic HCT occurring at OHSU between 1996-2012 for pts identified as having serositis with or without pericarditis. We reviewed clinicopathologic data to identify a cohort of patients who clearly had this complication in association with chronic GVHD. Laboratory studies from 1 month prior to diagnosis, at diagnosis, and 1 month post-diagnosis of serositis +/-pericarditis, as well as outcomes from invasive procedures, were included in our review.

RESULTS: 894 allogeneic HCT procedures took place during the study time frame.  23 cases were identified in the database search, although 8 were not included in the final analysis (1 acute GVHD, 2 anasarca without clear serositis, 1 pre-transplant pericarditis, 1 malignant effusion, 3 HCT or majority of care elsewhere).  15 met criteria for having cGVHD-associated serositis or pericarditis:  14 were male pts, and 10 received Cy/TBI conditioning.   All but 1 pt had a prior diagnosis of chronic GVHD.  The complication occurred in the setting of recent immunosuppression (IST) taper in 12 of the cases.  A significant increase in blood monocytes (median 500 vs. 1000 cells/uL p=0.002) and decrease in serum albumin (median 3.4 vs. 2.65, p=0.005) were identified at diagnosis compared with those values at one month pre-diagnosis. 13 of 15 pts were treated with steroids, with 7 of those demonstrating partial or complete response. 6 patients required invasive interventions (1 periocardiocentesis, 3 pericardial windows, and 2 pericardial stripping) due to severe symptoms.  3 pts died due to chronic GVHD complications. 

CONCLUSIONS: These data suggest that chronic GVHD-associated serositis with or without pericarditis occurs mainly in the setting of treated as opposed to de novo chronic GVHD, frequently in the setting of IST taper.  A notable predominance of male recipients receiving myeloablative TBI-based conditioning were identified in this cohort.  Biomarkers that appear to be associated with the syndrome include a decrease in albumin and an increase in absolute monocyte count. Outcomes data from larger series are required to better understand the role of invasive procedures and optimal treatment for this rare complication.