Discussion: 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 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. 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 standard guidelines for therapy. G-CSF and methylprednisolone have been tried with success in one study, while our patient demonstrated a robust response to low dose IV solumedrol. Given strongly positive nonspecific anti-neutrophil antibodies in the setting of a negative ANA noted in our patient, it is likely that there are other currently unknown antibodies associated with SLE which may correlate strongly with autoimmune neutropenia.
1. 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.
2. A strongly positive anti-neutrophil antibodies titer found in the setting of a negative ANA suggests that additional biomarkers associated with SLE may exist.
3. Inherent limitations of the 1997 ACR and 2012 SLICC classification criteria due to disease heterogeneity reduce their sensitivity as diagnostic tools; ultimately the diagnosis of SLE relies on clinical judgment. Future work in criteria refinement and biomarker development would improve the time to diagnosis for patients with variable presentations of SLE.
To cite this abstract:Zhao, M; Seymann, GB; Kantor, MA; Gupta, V . WHEN ANA GOES MIA: A CASE OF ANA-NEGATIVE LUPUS PRESENTING AS SEVERE AUTOIMMUNE NEUTROPENIA. Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev. Abstract 806. Journal of Hospital Medicine. 2017; 12 (suppl 2). https://www.shmabstracts.com/abstract/when-ana-goes-mia-a-case-of-ana-negative-lupus-presenting-as-severe-autoimmune-neutropenia/. Accessed September 23, 2019.