Discussion: Systemic lupus erythematosus (SLE) is a chronic, inflammatory, connective tissue disease that commonly affects a variety of organs due to an overactivation of the body’s immune system. Leukopenia, a decrease in granulocytes and/or lymphocytes, is a common hematological manifestation of SLE. However, severe autoimmune neutropenia occurs in only 5% of patients and may correlate with high anti-neutrophil antibody activity, as demonstrated in our patient. The mechanism of autoimmune neutropenia in SLE is unknown but may involve increased peripheral destruction, decreased bone marrow production, and increased margination. There are currently no guidelines for therapy, though G-CSF and methylprednisolone have been tried with success in one study. For our patient, we started him on low dose IV solumedrol and found that he responded drastically. As our patient had strongly positive nonspecific anti-neutrophil antibodies in the setting of a negative ANA, it is likely that there are other currently unknown antibodies associated with SLE which may correlate strongly with autoimmune neutropenia.
Conclusions: Autoimmune leukopenia is a common presentation in patients with SLE, while severe autoimmune neutropenia is an uncommon finding that may correlate with high anti-neutrophil antibody activity. A strongly positive anti-neutrophil antibodies titer was found in the setting of a negative ANA, suggesting that more biomarkers are associated with SLE than those that are currently known. Currently, the 1997 ACR and 2012 SLICC classification criteria have been useful in guiding clinical judgment. We are hopeful to see an increase in certainty of diagnosis for variable presentations of SLE with the advent of a new classification criteria and new biomarkers currently in development.
To cite this abstract:Zhao, M; Seymann, GB; Kantor, MA; Gupta, V . WHEN ANA GOES MIA: ANA-NEGATIVE PRESENTATION OF SLE IN MAN WITH SEVERE AUTOIMMUNE NEUTROPENIA. Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev. Abstract 807. Journal of Hospital Medicine. 2017; 12 (suppl 2). https://www.shmabstracts.com/abstract/when-ana-goes-mia-ana-negative-presentation-of-sle-in-man-with-severe-autoimmune-neutropenia/. Accessed September 23, 2019.