- | 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 |