- Case 1 Case 2 Case 3 Case 4
Age/Gender 46/M 26/ F 35/F 33/F
SLE diagnosis (year) 2008 2007 1998 1999
Onset of CIDP post SLE Diagnosis (months) 8 months 8 months 7 years 13 years
CIDP symptoms Bilateral quadriceps weakness, normal sensation, normal reflexes Numbness of right upper limb with loss sensation, light touch and pinprick (C6-T1) and absent reflexes Bilateral intermittent upper limb numbness with progressive bilateral lower limb weakness. Neuropathic pain affecting all 4 limbs, with reduced power in both limbs with hyperreflexia
Durations of symptoms 2 weeks 3 weeks One year 2 weeks
EMG/MRI findings LP: elevated protein,
no oligoclonal bands
MRI Brain and Spine: normal
NCS: axonal and sensory polyneuropathy
LP: elevated protein
MRI Spine: normal
NCS: normal
LP: elevated protein, no oligoclonal bands
MRI Brain: normal
MRI Spine: transverse myelitis hyper intense lesion C3-T2
LP: normal,
no oligoclonal bands
MRI Spine: hyper intense lesion C5-C7 with cord oedema
Concomitant manifestations LN Class IV MSK
LN Class IV
Cerebral lupus
MSK
LN Class III
MSK
LN III/V
Antibodies
(at diagnosis)
C3(NR:86-185 mg/dL)
C4(NR:20-59 mg/dL)
C3 69.2 mg/dL
C4 21.7mg/dL
ANA 1:640
Anti dsDNA negative
ACL negative
C3 24.5 mg/dL
C4 < 10 mg/dL
ANA 1:320
Anti dsDNA positive
ACL negative
C3 49.6 mg/dL
C4 < 10 mg/dL
ANA 1:160
Anti dsDNA negative
ACL negative
C3 99.2 mg/dL
C4 14.1 mg/dL
ANA 1:160
Anti dsDNA positive
ACL negative
Treatment Corticosteroids,
Mycophenolate mofetil, gabapentin
Corticosteroids,
Plasmapheresis,
IV Immunglobulin
IV Cyclophosphamide for one year then maintained with mycophenolate mofetil and ciclosporin A
Corticosteroids,
Plasmapheresis,
IV Immunoglobulin, IV cyclophosphamide
then maintained with mycophenolate mofetil
Corticosteroids,
IV cyclophosphamide then maintained with myfortic and tacrolimus
Outcome Symptoms resolved gradually over one year Relapsed with new onset of numbness & reduced sensation in
T4 -T10 dermatomes whilst on treatment with IV
cyclophosphamide
for 18 months
Six months later symptoms recurred and repeat MRI demonstrated transverse myelitis C1-C7
Persistent numbness despite SLE being in complete remission but ADL independent
Relapsed 8 years later with generalized tonic clonic seizures and bilateral numbness and weakness of both limbs with hyperreflexia.
MRI Brain and Spine: hyperintense C3- T3 lesions
Treated as relapsed CNS lupus with IV cyclophosphamide
Symptoms persist but ADL independent
Symptoms improved gradually over 6 months
Follow up (years) 8 years 7 years 18 years 17 years

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