A 66 year old Hispanic Female with hypertension, diabetes, and autoimmune hemolytic anemia (AIHA) was admitted with thrombocytopenia and acute kidney injury (AKI). Initial workup was negative for immune thrombocytopenia purpura (ITP) and thrombotic thrombocytopenia purpura (TTP). Progressive cytopenia triggered a search for systemic lupus erythematosus (SLE), myelodysplastic syndrome, and reactivation of AIHA (see table 1). Workup revealed both SLE and antiphospholipid syndrome (APS), while escalating renal failure prompted a kidney biopsy significant for stage III lupus nephritis. Her status improved with aggressive immunosuppressive therapy: prednisone, mycophenolate and rituximab. On HD 39, she was discharged to an acute rehabilitation facility on prednisone and mycophenolate. Follow up was facilitated with nephrology, hematology, and rheumatology.
Discussion:
A new diagnosis of SLE was made based on both the 1997 American College of Rheumatology (ACR) and 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria. Unique to our patient was the late onset and concomitant diagnoses of both Evans Syndrome (ES) and APS.
SLE is most commonly diagnosed in women in their 2nd-4th decades of life, while late onset SLE (LoSLE) (ages 50-65) is seen ~12-18% of total SLE diagnoses.1,2 LoSLE presents in a more benign manner, but with a poorer prognosis as it occurs in the context of pre-existing comorbid conditions.2 Compared to early SLE, LoSLE patients have a greater incidence of cytopenias, yet meet fewer criterions for SLE diagnosis (less frequent cutaneous manifestations, nephritis, and photosensitivity). The average time frame from symptom onset to diagnosis is 19-50 months in LoSLE versus 5-24 months in younger populations.1,2
Furthermore, ES is an autoimmune condition defined as the presence of hemolytic anemia and thrombocytopenia contemporaneously or sequentially. Its presentation with SLE is rare, and is seen in only 0.8%-3.7% of patients with AIHA or ITP.3 As in our patient, SLE with ES presents more frequently with nephritis.4 There are no treatment guidelines given its rarity, but glucocorticoids are considered first line. 3,4,5,6
Conclusions:
Our patient’s SLE had several unique traits that presented concurrently: Advanced age > 65, ethnicity, ES, APS, and lupus nephritis. The rarity of these factors, combined with a low incidence of LoSLE may contribute to a delayed diagnosis. It is also not uncommon for hospital patients to have varying degrees of cytopenias and kidney damage. However, due to poorer prognosis of LoSLE, it is prudent to maintain an awareness of this entity. Hospitalists need to be aware of LoSLE and not to exclude SLE in their differentials of cytopenias or renal failure, as delayed diagnosis can lead to adverse clinical outcomes. 