[A recovered case of SLE with central nervous system involvement who relapsed presenting new symptoms associated with development of serum anti-Sm antibody].
A rare SLE patient with central nervous system involvement (CNS-SLE) who relapsed presenting new symptoms associated with the development of serum anti-Sm antibody and was then successfully treated with cyclophosphamide (CY) pulse therapy is presented here. A 47-years old housewife was admitted to Kushiro City General Hospital because of fever, limb erythema and drowsy consciousness in September 1995. On the basis of convulsion, proteinuria, leukopenia, thrombopenia, serum positive tests for both anti-nuclear antibody and anti-SSA antibody and low complement levels, as well as elevations of IgG index and IL-6 in the cerebrospinal fluid (CSF), she was diagnosed as having CNS-SLE. Serum tests for anti CL-beta 2 GPI antibody and lupus anticoaglant was negative. Serum test for HBs antigen was positive. She was treated successfully with methylprednisolone (mPSL) pulse therapy and plasma exchange (PE). Prednisolone was gradually tapered to the dosage of 17.5 mg per day and she was discharged in April 1996. She was re-admitted because of fever, an exacerbation of skin eruption and arthralgia in October 1996. Serum anti-Sm antibody was found to be positive. mPSL pulse therapy was not effective. On the basis of hallucination and elevations of IgG index and IL-6 in the CSF, a diagnosis of relapsed CNS-SLE was made. However the level of IFN-alpha in the CSF was normal. Although PE was not effective, CY pulse therapy was markedly effective.
Hiura
K
K
Department of Internal Medicine, Gastroenterology and Rheumatology, Kushiro City General Hospital.
Abe
T
T
Hatanaka
Y
Y
Ikeda
Y
Y
Akaike
J
J
Hosokawa
A
A
Itoh
Y
Y
Takagi
H
H
Sakai
H
H
Suwa
T
T
Yonezawa
K
K
Tosaka
M
M
Chiba
S
S
Imai
K
K
D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D002493:Central Nervous System Diseases / Q000209:etiology*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D008875:Middle Aged; D017411:Ribonucleoproteins, Small Nuclear; D055517:snRNP Core Proteins
D001323:Autoantibodies; D001324:Autoantigens; D017411:Ribonucleoproteins, Small Nuclear; D055517:snRNP Core Proteins; D003520:Cyclophosphamide