Parkland Morning Report 7/18 – Lupus Cerebritis

At today’s morning report at Parkland R2 Corbin Eule presented a case of a 68 year-old female with 2 weeks of confusion and lower extremity motor and sensory neuropathy, on a background of chronic fatigue, malaise and unintentional weight loss.

Our case discussion was led by pulmonary/ critical care faculty Dr Carlos Girod. The initial differential was broad and included malignancy with paraneoplastic syndrome, connective tissue disorders, and infiltrative disorders such as amyloidosis and sarcoidosis. The patient’s admission labs were notable for pancytopenia and normal renal function and urine sediment. An autoimmune panel was revealing with ANA >1:2560, positive anti-dsDNA antibodies, and low complement levels. Subsequent studies included a bone marrow biopsy showing LE cells and peripheral nerve biopsy consistent with vasculitis.

The patient was diagnosed with systemic lupus erythematosus (SLE) and received a course of “pulse” dose methylprednisolone followed by initiation of mycophenolate and an oral prednisone taper, with which she clinically improved.

SLE Pearls:

–     SLE may present with a wide variety of neuropsychiatric manifestations, including cognitive dysfunction, encephalopathy, polyneuropathy, psychosis, mood disorders, and coma1.

–     Neuropathy associated with SLE is thought to be due to vasculopathy of the small arteries supplying nerves; given the systemic nature of the disease, multiple nerves can be affected at once2.

–     LE cells were first described in 1948 by hematologists Dr Malcolm Hargraves and Dr Robert Morton, who found cells in the bone marrow of patients with SLE that appeared to have had their nuclei phagocytosed by mature polymorphonuclear cells3.

Clinical Presentation:

–     SLE is diagnosed by likelihood (definite, probable or possible SLE) using clinical and immunologic criteria such as that described by the American College of Rheumatology (ACR)4 or the Systemic Lupus International Collaborating Clinics (SICC)5.

–     The presence of neurologic manifestations in the absence of other known causes is one of the clinical criteria described by both societies, as is the presence of blood cell dyscrasias (hemolytic anemia, thrombocytopenia, leuko- or lympho-penia), and serum autoantibodies (ANA, anti-dsDNA, anti-Sm or antiphospholipid antibodies) that were seen in this case.

Treatment:

–     Treatment of SLE depends on the severity of the flare, with systemic corticosteroids and immunosuppressants forming the mainstay of treatment.

–     Determining dosing intensity and duration of corticosteroid therapy for treatment of SLE is challenging. Whereas historically “pulse” steroids (methylprednisolone 0.5 to 1g/day for 3 days) were commonly regarded as the gold standard for severe SLE flares, more recent evidence suggests that lower doses may be equally efficacious, with reduction in infectious side effects6.

References:

1. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis and rheumatism. 1999;42(4):599-608.

2. Florica B, Aghdassi E, Su J, Gladman DD, Urowitz MB, Fortin PR. Peripheral neuropathy in patients with systemic lupus erythematosus. Seminars in arthritis and rheumatism. 2011;41(2):203-211.

3. Hepburn AL. The LE cell. Rheumatology (Oxford). 2001;40(7):826-827.

4. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis and rheumatism. 1997;40(9):1725.

5. Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis and rheumatism. 2012;64(8):2677-2686.

6. Badsha H, Edwards CJ. Intravenous pulses of methylprednisolone for systemic lupus erythematosus. Seminars in arthritis and rheumatism. 2003;32(6):370-377.