[Hemophagocytic syndrome complicating adult's seropositive rheumatoid arthritis].
ABSTRACT Macrophage activation syndrome (MAS) is a severe complication of chronic rheumatic diseases, particularly juvenile rheumatoid arthritis. However, MAS is rarely described in adult rheumatoid polyarthritis.
We report a case of MAS complicating a seropositive rheumatoid polyarthritis after 20 years of evolution. Pancytopenia with fever, renal failure and hepatic dysfunction revealed the disease that was confirmed by multiple macrophages and monocytes invading the bone marrow specimen.
Outcome has been spectacular under corticosteroids.
- SourceAvailable from: Takahisa Gono[show abstract] [hide abstract]
ABSTRACT: We report a patient with rheumatoid arthritis (RA) who showed bicytopenia with hyperferritinemia and hepatic dysfunction ascribable to hemophagocytic syndrome (HPS) 2 weeks after commencement of bucillamine. Pathology of the bone marrow showing infiltration of macrophages confirmed the diagnosis of HPS. On the basis of renal dysfunction with an increase in fibrin degradation products, disseminated intravascular coagulation was considered to be concurrent with HPS. Oral prednisolone and cyclosporine A were started right after cessation of bucillamine, and yielded complete normalization of hepatic and renal function and hematology. As there was neither disease activity of RA nor associated infection throughout the clinical course, bucillamine was suspected of being the cause of HPS in our patient. HPS is a very rare complication in RA, but should be actively considered when abnormalities in laboratory data, especially pancytopenia and hepatic dysfunction, quickly worsen.Internal Medicine 02/2007; 46(21):1809-13. · 0.97 Impact Factor