Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome.
OBJECTIVE
To study the long term effectiveness of intravenous immunoglobulin and plasmapheresis associated with prednisone and cyclophosphamide in Churg-Strauss syndrome.


METHODS
and methods: We studied 18 subjects with new onset Churg-Strauss syndrome. All received the "standard" treatment based on prednisone (1 mg/kg/day for 1 month and then slowly tapered) and cyclophosphamide (2 mg/kg/day for 6 months in severe cases). In nine patients, synchronised cycles with plasmapheresis and intravenous immunoglobulin (2 g/kg) were repeated monthly for 6 months and every other month for a further three cycles. Clinical (disease activity monitored by Birmingham vasculitis activity score (BVAS) and damage index (modified Rankin score)) and functional (C reactive protein, blood eosinophil count, and electromyogram-electoneurogram) parameters were collected during treatment and the 3 year follow up period.


RESULTS
After 12 months, all patients in the treatment group and four (44%) in the control group were in remission. At the end of the 3 year follow up period, we documented significant differences in BVAS (p<0.01), global damage (p<0.02), modified Rankin score (p<0.04), and the daily maintenance prednisone dose (p<0.002) between the two groups. We found a tendency towards lower frequency of relapse and incidence of osteoporosis in the treatment group.


CONCLUSIONS
Complete clinical and functional recovery with a long term stable remission and a low incidence of side effects can be achieved by intravenous immunoglobulin associated with plasmapheresis in patients with Churg-Strauss syndrome.
Danieli
M G
MG
Istituto Clinica Medica Generale, Ematologia ed Immunologia Clinica, Polo Didattico Scientifico, Via Tronto 20, 60020, Torrette di Ancona, Italy. eleonora@univpm.it.
Cappelli
M
M
Malcangi
G
G
Logullo
F
F
Salvi
A
A
Danieli
G
G
D000328:Adult; D000368:Aged; D015267:Churg-Strauss Syndrome / Q000628:therapy*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004341:Drug Evaluation; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000009:adverse effects / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis / Q000009:adverse effects / Q000379:methods; D011241:Prednisone / Q000627:therapeutic use; D011446:Prospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone