Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Renal Dysfunction Is Associated with Increased Mortality in Adults with Macrophage Activation Syndrome.

Shakoory3,  Bita, Chatham3,  W. Winn, Alarcon1,  Graciela S., Cron2,  Randy Q.

Oakland, CA
Children's Hospital of Alabama, Birmingham, AL
Univ of Alabama-Birmingham, Birmingham, AL

Background:

Macrophage activation syndrome (MAS) remains a potentially fatal cause of multiorgan dysfunction (MOD) in adults, with an estimated mortality of 50%. This longitudinal study reports the short and long term outcomes in hospitalized adults with MAS due to various triggering factors from a single academic institution.

Methods:

Patients diagnosed with MAS from April '08 to May '10 at a regional tertiary institution were identified from rheumatology consultation logs. The diagnosis was made in patients with MOD with at least one of the following: A. Tissue hemophagocytosis; B. Consensus of >=2 physicians to treat; or C. Elevated ferritin >10,000 mg/L. Final decision for treatment was made independent of the study. The length of hospital stay (HS) and critical care (CC), the need for renal replacement therapy (RRT), vasopressor use (VP) and mechanical ventilation (MV), and death were recorded. Follow up data on survivors (chronic organ damage, readmission) were obtained from the time of discharge in 3–6 month intervals.

Results:

Seventeen adults (age 17–74y), 59% non-whites (53% AA, 6% Asian), predominantly women (76%), were included. A definite predisposing disorder could not be identified in one patient. The following potential etiologies were suggested in the remaining.

Infectious: Ehrlichiosis in 3, CMV, HSV, EBV in 6.

SLE in 7, adult-onset Still disease, IgA nephropathy, Wegener disease in 3.

Hematological malignancies in 3.

Pregnancy in 2.

The average HS was 31 days (5–112d, median 28d), 76% of patients had a CC stay (mean 11.2d, median 11d). MV and VP and RRT required in 65% (n=11). Nine of the 17 patients did not survive the hospitalization (53% mortality). Follow up in survivors (Mean 13m, Median 12m, range 3–26m, last discharge March '10) showed 4 readmissions in 2 survivors (1 for delivery, 1 with 3 readmissions) with chronic organ damage in 4: stage II liver fibrosis per biopsy in 2, worsening of chronic kidney disease and RRT after discharge in 3 (<6 month in 1, permanent RRT in 2).

The need for CC, MV, or RRT occurred more frequently in those who died than in survivors. There were no differences in age, gender, ethnicity, the presence of two or more triggering factors, or the length of HS or CC stay between survivors and non-survivors (Table).

Conclusions:

In adults, a diagnosis of MAS oftern results in prolonged and complicated hospital course with high mortality and should be investigated and treated in patients with early manifestations of MOD to prevent critical care interventions and associated morbidity and mortality.

 The Outcomes Correlates of MAS
 Deceased (N = 9)Mean (SD)Survived (N = 8)Student t Testp ValueTotal Mean (SD)
Age54 (15)38 (20)0.346 (19)
LoHA36 (31)24 (13)0.357 (24)
LoCC11 (8)6 (6)0.28 (8)
 N (%)N (%)Fischer Exact TestN (%)
Woman7 (78)6 (75)1.013 (76)
Non-White5 (55)3 (38)0.68 (47)
Two triggers7 (78)3 (38)0.110 (59)
CC9 (100)4 (50)<0.0513 (76)
MV8 (89)3 (37)<0.0511 (65)
RRT8 (89)1 (13)<0.0059 (53)
MAS: macrophage activation syndrome, LoHS: length of hospital stay, LoCC: length of critical care admission, MV: mechanical ventilation, RRT: renal replacement therapy, CC: critical care

To cite this abstract, please use the following information:
Shakoory, Bita, Chatham, W. Winn, Alarcon, Graciela S., Cron, Randy Q.; Renal Dysfunction Is Associated with Increased Mortality in Adults with Macrophage Activation Syndrome. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1668
DOI: 10.1002/art.29433

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