Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis.
OBJECTIVE
Acute/subacute interstitial pneumonia (A/SIP) in patients with polymyositis/dermatomyositis (PM/DM) is frequently fatal within months despite high dose prednisolone (PSL) therapy. Our objective was to improve the survival rate of patients with A/SIP associated with PM/DM; and to characterize patients with PM/DM who are at high risk of developing A/SIP.


METHODS
We conducted a pilot trial of combined immunosuppressive therapy with high dose PSL, 10-30 mg/kg of intravenous pulse cyclophosphamide (IVCYC) every 3-4 weeks, and 2-4 mg/kg/day of cyclosporin A (CSA) for patients with A/SIP. A/SIP was diagnosed based on a history of rapidly worsening respiratory symptoms, progressive radiological findings or hypoxemia, and compatible findings in high resolution computed tomography images.


RESULTS
Before December 2000, 12 patients with DM among 83 PM/DM patients developed A/SIP, and 9 patients died despite treatment using high dose PSL with or without a choice of CSA, cyclophosphamide, or azathioprine. Thereafter, 10 patients with DM among 27 PM/DM patients developed A/SIP, and they were given combination therapy with PSL, CSA, and IVCYC. Five patients survived and are doing well for more than 2 years, although the remaining 5 patients died of respiratory failure within 3 months. DM patients with A/SIP showed the following characteristic features: mild myositis, palmar papule, fever, and negative or low titer of antinuclear antibody.


CONCLUSIONS
Immediate institution of intensified immunosuppressive therapy should be considered for patients with A/SIP complicating DM. However, even early recognition of A/SIP and immediate commencement of a regimen including CSA and IVCYC in addition to high dose PSL may not be sufficient for some of those patients.
Kameda
Hideto
H
Division of Rheumatology/Clinical Immunology, Department of Internal Medicine, Saitama Medical Center, Kawagoe, Japan. kamehide@saitama-med.ac.jp
Nagasawa
Hayato
H
Ogawa
Hiroe
H
Sekiguchi
Naoya
N
Takei
Hirofumi
H
Tokuhira
Michihide
M
Amano
Koichi
K
Takeuchi
Tsutomu
T
D000208:Acute Disease; D000328:Adult; D000368:Aged; D003520:Cyclophosphamide / Q000008:administration & dosage*; D016572:Cyclosporine / Q000008:administration & dosage*; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007262:Infusions, Intravenous; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D011239:Prednisolone / Q000008:administration & dosage*; D011336:Probability; D020551:Pulse Therapy, Drug; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D015996:Survival Rate; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D016572:Cyclosporine; D003520:Cyclophosphamide; D011239:Prednisolone