Early intervention with corticosteroids and cyclosporin A and 2-hour postdose blood concentration monitoring improves the prognosis of acute/subacute interstitial pneumonia in dermatomyositis.

OBJECTIVE: We retrospectively examined the effect of combination therapy with prednisolone and cyclosporin A (CSA) for dermatomyositis (DM) presenting with acute/subacute interstitial pneumonia (A/SIP), the daily CSA dose, and the time from diagnosis of A/SIP to initiation of CSA treatment. METHODS: Subjects were 16 DM patients with A/SIP. Seven patients were treated initially with 1 mg/kg/day prednisolone. When IP was progressive, CSA was added (Group A). Nine patients were treated initially with 1 mg/kg/day prednisolone and 4 mg/kg/day CSA, and 2-h postdose blood concentration (C2) monitoring was used to maintain the serum CSA level at 1000 ng/ml (Group B). RESULTS: Four of 7 patients in Group A (57%) and 1 of 9 patients in Group B (11%) died of respiratory failure related to IP (p = 0.06). Combination therapy with prednisolone and CSA at >or= 200 mg/day initiated within 15 days of diagnosis was effective for treatment of DM-A/SIP. The trough level (C0) and daily CSA dose were higher in Group B (201.3 ng/ml and 200.0 mg/day, respectively) than in Group A (140.0 ng/ml and 166.4 mg/day). CSA was continued in all patients without severe side effects. No patient died of infection. CONCLUSION: Combination therapy of corticosteroids and CSA should be initiated during the early stage of IP. The daily CSA dose should also be controlled with measurement of serum CSA concentration to achieve maximal immunosuppressive effect. C2 monitoring is a useful tool for this control.

[A patient with interstitial pneumonia associated with dermatomyositis who relapsed after reducing cyclosporin-A treatment].

A 67-year-old female noticed dyspnea on exertion associated with the development of erythema in the eyelids and the bilateral fingers, and was admitted to our hospital on July 21, 2004. Proximal muscle weakness in the limbs, heliotrope rash, and Gottron's sign were observed, but the CK level was normal (194 U/l). All autoantibodies except for rheumatoid factor were negative. Hypoxemia and interstitial pneumonia on chest CT images were observed. Based on these findings, a diagnosis of advanced interstitial pneumonia associated with dermatomyositis was made. Combination immunosuppressive therapy was initiated with corticosteroid pulse therapy and cyclosporin-A (Cy-A), resulting in marked improvement. The Cy-A trough concentration was markedly high (456.4 ng/ml). When cytomegalovirus infection developed, the dose of Cy-A was reduced. Although the blood trough concentration of Cy-A was maintained at an adequately high level, the patient died of recurrence of rapidly progressive interstitial pneumonia. Careful observation is required when the dose of Cy-A is reduced for a patient with interstitial pneumonia associated with dermatomyositis. Furthermore, it is suggested that the trough concentration level of Cy-A is not always a useful parameter.

Serum protein profile of rheumatoid arthritis treated with anti-TNF therapy (infliximab).

We analyzed the changes in the serum protein profile by infliximab using two-dimensional gel electrophoresis and mass spectrometry. More than 50 gel spots were seen to increase or decrease in correlation with clinical improvements of RA. The spots corresponding to CRP, C3, and Apo J showed reduced staining intensity, while the spots corresponding to Apo A-I, RBP, and transthyretin were enhanced. The protein profile of RA patients treated with infliximab was mostly similar to that of normal healthy controls except for several protein spots. This suggested that infliximab normalized the serum protein profile of RA patients, leading to modification in the serum lipid profile and antioxidant status in RA.