An initial serum ferritin level of ≥450 ng/mL, P[A-a]O of ≥30 mmHg, and right middle lobe GGO score of ≥2 (GGO ≥5% of the lobe) were identified as poor prognostic factors for anti-MDA5 Ab-positive DM-IP patients.

Tumour necrosis factor-alpha (TNF-α) is an important mediator in the pathogenesis of rheumatoid arthritis (RA) and hypertension. TNF-α inhibitors improve clinical symptoms and inhibit joint destruction in RA, but their effect on blood pressure (BP) has not been fully investigated. We measured 24-h BP using an ambulatory BP monitor in 16 RA patients treated with a TNF-α inhibitor, infliximab, to investigate its influence on BP and its association with the regulatory factors of BP and renin-angiotensin-aldosterone and sympathetic nervous systems. Infliximab significantly reduced the 24-h systolic BP (SBP) from 127.4±21.8 to 120.1±23.4 mm Hg (P<0.0001). Particularly, morning BP (0600-0800 h) decreased from 129.7±19.7 to 116.9±13.4 mm Hg (P<0.0001), and daytime BP decreased from 131.8±15.1 to 122.5±13.7 mm Hg (P<0.0001). Infliximab significantly reduced the plasma level of norepinephrine and plasma renin activity (PRA) (from 347.5±180.7 to 283.0±181.8 pg ml(-1) and 2.6±2.7 to 2.1±2.9 ng ml(-1) h(-1), respectively) but did not significantly reduce the plasma levels of dopamine and epinephrine. The reduction in morning SBP correlated with the reduction in the norepinephrine level (P<0.05) but not with that in PRA and inflammatory parameters related to RA. This study shows the effect of infliximab on ambulatory BP, especially daytime BP, which may be partly accounted for by the reduction of sympathetic nerve activity after infliximab treatment.

We retrospectively examined the effect of combination therapy with prednisolone and cyclosporin-A (CSA) on the findings of pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCTs) scans in patients with dermatomyositis (DM) and acute/subacute interstitial pneumonia (A/SIP). We also examined whether CSA therapy improved PFT and chest HRCT findings. DM patients (n=14) with A/SIP were treated with 1 mg/kg/day prednisolone and 4 mg/kg/day CSA within 4.4 days (range, 1-12 days) from diagnosis. The trough level (C0) and 2-h post-dose blood concentration (C2) of CSA were measured. PFTs and HRCT scans were performed before and 1 year after treatment. The total ground-glass opacity area was calculated with the HRCT findings and used as the CT score. Combination prednisolone and CSA therapy improved the TLC%, VC%, FVC%, EFV1.0%, and CT score (P=0.027, 0.003, 0.002, 0.001, and 0.001, respectively). The C0 level was 178.8 ng/ml (range, 71-456 ng/ml), and the C2 level was 1,336.6 ng/ml (range, 814-2,873 ng/ml). Therapeutic changes in FVC%, FEV1.0%, and DLCO% were correlated with the C2 CSA level (P=0.047, 0.025, and 0.035, respectively). However, the PFT results and CT scan scores did not correlate with the daily dose or C0 level of CSA. Improvements in the CT score were correlated with time from IP diagnosis to CSA initiation (P=0.014). Early intervention with prednisolone and CSA combination therapy and tight control of the daily CSA dose by monitoring the C2 level improved PFT and chest HRCT findings in DM-A/SIP.

BackgroundAcute/subacute interstitial pneumonia in dermatomyositis (DM-A/SIP) is a disease associated with a poor prognosis that resists treatment with glucocorticosteroids (GC) and progresses rapidly in a period of weeks to months to death. We retrospectively studied outcomes, prognostic factors, and their relations with survival rate in patients with DM-A/SIP treated with early cyclosporine A (CSA)/GC combination therapy and 2-hour postdose blood concentration monitoring.MethodsThis study comprised 32 DM-A/SIP patients who were simultaneously treated with CSA and prednisolone. Clinical and laboratory findings were compared between those who died due to DM-A/SIP and those surviving 24 weeks after beginning of therapy. Prognostic factors were extracted, and their relations with the survival rate were evaluated.ResultsOf the 32 DM-A/SIP patients, 25 survived, 5 died of DM-A/SIP, and 2 died of infections. In those who died due to DM-A/SIP, ferritin level and the alveolar-arterial oxygen gradient were significantly increased compared with the survivors (P<0.001 and P = 0.002, respectively). Multivariate analyses showed that ferritin and alveolar-arterial oxygen gradient were independent prognostic factors of poor outcome. The survival rate 24 weeks after beginning of treatment was significantly lower in those with a ferritin level of ≥600 ng/ml and alveolar-arterial oxygen gradient of ≥45 Torr (P<0.001 and P<0.001, respectively). All patients with both prognostic factors died, and the outcome was significantly poorer in these patients than in those with one or neither of the prognostic factors (P<0.001).ConclusionsWe identified pre-treatment high serum ferritin level and high alveolar-arterial oxygen gradient as poor prognostic factors in DM-A/SIP patients undergoing early CSA/GC combination therapy and showed that the outcomes were poor in patients with both factors.

We extended the scaled particle theory of Cotter and Wacker for multicomponent systems of straight hard spherocylinders to multicomponent solutions of wormlike hard spherocylinders and calculated phase diagrams of ternary systems consisting of two homologous semiflexible polymer components with different lengths and a low molar mass good solvent. The basic parameters in this theory are the chain contour lengths (Li, Li), the hard core diameter (d), and the persistence length (q) of the polymer components. The theoretical ternary phase diagrams calculated by this scaled particle theory for wormlike hard spherocylinders were compared with experimental ternary phase diagrams obtained previously for systems of schizophyllan + water and poly(n-hexyl isocyanate) + toluene. When the hard core diameter d was chosen to have a value close to that estimated from the osmotic pressure or solvent chemical potential data for the corresponding binary solutions, good agreements between experimental and theoretical ternary phase diagrams were obtained for the isotropic-anisotropic binodal curves and the tie lines of all the systems compared. On the other hand, the present theory failed to predict anisotropic-anisotropic-isotropic three-phase coexistence as well as anisotropic-anisotropic two-phase coexistence in ternary solutions containing two samples with the Kuhn segment numbers JVi (=LJ2q) = 0.930 andJVa (=L¡J2q) = 0.0765, whereas these multiphase separations were found for aqueous solutions of schizophyllan with the same N¡ s. When N2 was decreased to smaller than 0.07 with M kept at 0.93, these phase-coexisting regions appeared in the theoretical phase diagram.