Thrombocytopenia is found in nearly half of patients with CAPS and would not account for bleeding until the count is less than 50,000/cubic millimeters. When present, concomitant DIC should be ruled out, because this condition would require transfusions of fresh frozen plasma and platelets. The benefit of transfusions of fresh frozen plasma in the bleeding patient with CAPS without any evidence of a factor deficiency or DIC has not been established, but should be considered in the case of large-volume blood transfusions because of the dilutional effect on clotting factors.
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Explore This IssueAugust 2015
The precise mechanism by which APS causes pulmonary alveolar hemorrhage (PAH) has not been determined. In the largest case series of PAH in primary APS, lung biopsy specimen showed capillaritis and BAL fluid analysis revealed neutrophils and macrophages in keeping with an inflammatory cause of pulmonary hemorrhage. Anticoagulation, the first line in the treatment of CAPS, is contraindicated in these cases.
CAPS is rare, but it carries a high mortality rate. Pulmonary hemorrhage in particular is associated with a poor prognosis.4 Our case highlights the importance of increased awareness of the varied manifestations of this disease and the importance of aggressive treatment.
Joy-Ann Tabanor, MD, is the corresponding author, and a second-year resident physician in the Department of Medicine at Englewood Hospital and Medical Center in Englewood, N.J. She aspires to become a clinical rheumatologist.
Hyun Bae, MD, is a third-year resident physician in the Department of Medicine at Englewood Hospital and Medical Center in Englewood, N.J.
Girish Sonpal, MD, is a rheumatologist with decades of experience who practices in Whitestone, N.Y.
Karlene Williams, MD, is the associate program director for the Internal Medicine Residency Program at Englewood Hospital and Medical Center in Englewood, N.J.
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