PMID- 8003065
OWN - NLM
STAT- MEDLINE
DCOM- 19940708
LR  - 20190718
IS  - 0004-3591 (Print)
IS  - 0004-3591 (Linking)
VI  - 37
IP  - 6
DP  - 1994 Jun
TI  - Influence of disease manifestation and antineutrophil cytoplasmic antibody titer 
      on the response to pulse cyclophosphamide therapy in patients with Wegener's 
      granulomatosis.
PG  - 919-24
AB  - OBJECTIVE: To assess the effectiveness of pulse cyclophosphamide (CYC) in the 
      treatment of Wegener's granulomatosis (WG) and to identify the patients who are 
      responsive to the treatment. METHODS: The prospective study included 43 patients 
      with biopsy-proven WG. Clinical, radiographic, laboratory, and immunologic data 
      were evaluated for predictive values regarding the outcome of pulse CYC therapy. 
      RESULTS: Only 42% of the patients showed complete or partial remission that 
      lasted at least 6 months after cessation of pulse CYC therapy. These responders 
      had a higher frequency of disease activity limited to the upper and lower 
      respiratory tract (39%, versus 8% in the nonresponder group; P < 0.05) and had 
      lower titers of classic antineutrophil cytoplasmic antibody (cANCA) prior to 
      treatment (< 1:64 42%, versus 6% in the nonresponder group; P < 0.05). In the 58% 
      of patients who did not respond to pulse CYC treatment, there was both systemic 
      disease involving more than 4 organ systems (mainly, the heart, nervous system, 
      eye, and skin) and constitutional symptoms. Serious side effects induced by pulse 
      CYC occurred in only 1 patient. CONCLUSION: Based on these findings, pulse CYC 
      therapy appears to be effective in WG patients with moderate disease activity and 
      low titers of cANCA, but of little benefit in patients with severe WG. Pulse CYC 
      should therefore not be used as first-line therapy in patients with severe and 
      rapidly progressing forms of WG associated with high titers of cANCA.
FAU - Reinhold-Keller, E
AU  - Reinhold-Keller E
AD  - Medizinische Universitat Lubeck, Abteilung Klinische Rheumatologie, Bad 
      Bramstedt, Germany.
FAU - Kekow, J
AU  - Kekow J
FAU - Schnabel, A
AU  - Schnabel A
FAU - Schmitt, W H
AU  - Schmitt WH
FAU - Heller, M
AU  - Heller M
FAU - Beigel, A
AU  - Beigel A
FAU - Duncker, G
AU  - Duncker G
FAU - Gross, W L
AU  - Gross WL
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Arthritis Rheum
JT  - Arthritis and rheumatism
JID - 0370605
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
RN  - 0 (Autoantibodies)
RN  - 0 (Delayed-Action Preparations)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
CIN - Arthritis Rheum. 2002 Jan;46(1):278-80. PMID: 11817605
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Antibodies, Antineutrophil Cytoplasmic
MH  - Autoantibodies/*blood
MH  - Cyclophosphamide/*administration & dosage
MH  - Delayed-Action Preparations
MH  - Female
MH  - Granulomatosis with Polyangiitis/complications/*drug therapy/immunology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Prospective Studies
MH  - Remission Induction
MH  - Severity of Illness Index
EDAT- 1994/06/01 00:00
MHDA- 1994/06/01 00:01
CRDT- 1994/06/01 00:00
PHST- 1994/06/01 00:00 [pubmed]
PHST- 1994/06/01 00:01 [medline]
PHST- 1994/06/01 00:00 [entrez]
AID - 10.1002/art.1780370622 [doi]
PST - ppublish
SO  - Arthritis Rheum. 1994 Jun;37(6):919-24. doi: 10.1002/art.1780370622.