Objective
To determine serum ferritin levels in patients with idiopathic inflammatory myopathies(IIM), including dermatomyositis(DM), clinical amyopathic dermatomyositis(CADM)and polymyositis(PM), and to evaluate their association with disease activity, especially with interstitial lung disease(ILD).
Methods
Clinical data and laboratory results were collected from 120 patients with IIM. A double-site enzyme immunoassay was conducted to measure serum ferritin levels in 120 patients with IIM and 63 healthy human controls. Statistical analysis was carried out to assess the relationship of serum ferritin levels with inflammatory biomarkers, autoantibodies, serum muscle enzymes, and severity of ILD in patients with IIM.
Results
Serum ferritin levels were elevated in 36 out of the 120 patients, but normal in the other 84 patients. Compared with patients with normal serum ferritin levels, those with elevated serum ferritin levels showed increased levels of C-reactive protein(14.1 ± 6.5 vs. 3.6 ± 1.7 mg/L, P < 0.01), aspartate aminotransferase(111.8 ± 44.6 vs. 46.0 ± 9.0 U/L, P< 0.01), lactate dehydrogenase(388.6 ± 81.5 vs. 260.7 ± 29.1 U/L, P< 0.01), as well as erythrocyte sedimentation rate(34.8 ± 8.2 vs. 15.4 ± 2.7 mm/h, P< 0.01). However, no significant difference was observed in the level of creatine kinase between patients with elevated and normal serum ferritin levels. Moreover, serum ferritin levels were significantly higher in both IIM patients complicated with acute/subacute interstitial pneumonia(A/SIP, 650.5 ± 268.5 ng/ml)and those with chronic interstitial pneumonia(CIP, 489.9 ± 157.3 ng/ml)than in those without ILD(155.7 ± 90.8 ng/ml)and those with imageological changes but no clinical symptoms of ILD(193.3 ± 62.1 ng/ml)(all P < 0.01). Moreover, compared with patients with normal serum ferritin levels, those with elevated serum ferritin levels showed a significant increase in the incidence of CIP(52.8% vs. 25.9%, P < 0.01), A/SIP(22.2% vs. 3.5%, P < 0.01), as well as appearance of ground-glass opacity(67.6% vs. 43.4%, P < 0.05)and honeycomb-like changes(14.7% vs. 2.6%, P < 0.05)on high-resolution CT scans.
Conclusion
Serum ferritin levels are highly correlated with erythrocyte sedimentation rate, C-reactive protein, aspartate aminotransferase, lactate dehydrogenase, ILD and high-resolution CT findings, and may be of great clinical significance for the evaluation of disease activity, especially the severity of ILD.
Key words:
Myositis; Dermatomyositis; Ferritins; Lung diseases, interstitial; Idiopathic interstitial pneumonias
Objective: Integrin β3 is implicated in numerous biological processes such as its relevance to blood triglyceride, yet whether β3 deficiency affects this metabolic process remains unknown. Approach and Results: We showed that the Chinese patients with β3-deficient Glanzmann thrombasthenia had a 2-fold higher serum triglyceride level together with a lower serum LPL (lipoprotein lipase) level than those with an αIIb deficiency or healthy subjects. The β3 knockout mice recapitulated these phenotypic features. The elevated plasma triglyceride level was due to impaired LPL-mediated triglyceride clearance caused by a disrupted LPL secretion. Further analysis revealed that β3 directly bound LPL via a juxtamembrane TIH (threonine isoleucine histidine) 720 –722 motif in its cytoplasmic domain and functioned as an adaptor protein by interacting with LPL and PKD (protein kinase D) to form the PKD/β3/LPL complex that is required for β3-mediated LPL secretion. Furthermore, the impaired triglyceride clearance in β3 knockout mice could be corrected by adeno-associated virus serotype 9 (AAV9)-mediated delivery of wild-type but not TIH 720– 722 -mutated β3 genes. Conclusions: This study reveals a hypertriglyceridemia in both β3-deficient Chinese patients and mice and provides novel insights into the molecular mechanisms of the significant roles of β3 in LPL secretion and triglyceride metabolism, drawing attention to the metabolic consequences in patients with β3-deficient Glanzmann thrombasthenia.
Objective
To explore the association of serum cytokine levels with disease activity in patients with dermatomyositis (DM) and clinically amyopathic dermatomyositis (CADM) , especially their association with skin lesions and interstitial lung disease (ILD) .
Methods
Enzyme-linked immunosorbent assay (ELISA) and cytometric beads array (CBA) were performed to detect the serum levels of interleukin (IL) -2, IL-4, IL-6, IL-10, IL-17A, IL-18, tumor necrosis factor (TNF) and interferon (IFN) -γ in 40 patients with DM or CADM, as well as in 16 health checkup examinees (healthy control group) . Then, the association of serum cytokine levels with skin lesions, inflammatory biomarkers and severity of ILD was analyzed.
Results
The patients with DM/CADM showed significantly higher serum levels of IL-6 (37.8 ± 45.8 pg/ml) , IL-10 (16.1 ± 7.2 pg/ml) and IL-18 (492.0 ± 193.1 pg/ml) compared with the healthy controls (12.0 ± 2.7 pg/ml, 7.7 ± 1.4 pg/ml, 191.1 ± 39.2 pg/ml, respectively, all P < 0.001) , and there were no significant differences in the serum levels of the other 5 cytokines between the above 2 groups. The serum level of IL-6 was significantly higher in patients with elevated erythrocyte sedimentation rate (ESR) than in those with normal ESR (49.7 ± 46.8 pg/ml vs. 29.1 ± 45.4 pg/ml, P= 0.008) . The patients with raised C-reactive protein (CRP) levels showed significantly higher serum levels of IL-6 (68.7 ± 59.7 pg/ml) and IL-18 (635.1 ± 232.8 pg/ml) compared with those with normal CRP levels (IL-6: 30.6 ± 40.3 pg/ml, P= 0.013; IL-18: 440.2 ± 164.7 pg/ml, P= 0.020) . Moreover, the patients with elevated levels of lactate dehydrogenase (LDH) showed significantly higher serum levels of IL-10 (18.4 ± 6.9 pg/ml) , IL-17A (19.6 ± 6.7 pg/ml) and IL-18 (529.4 ± 197.2 pg/ml) compared with those with normal LDH levels (IL-10: 10.7 ± 4.8 pg/ml, P < 0.001; IL-17A: 11.4 ± 6.6 pg/ml, P= 0.001; IL-18: 404.9 ± 158.0 pg/ml, P= 0.037) . No significant difference in the cytokine levels was observed between the patients with elevated creatine kinase (CK) levels and those with normal CK levels. The patients with Gottron′s papules/sign showed significantly higher serum levels of IL-18 (513.7 ± 187.2 pg/ml) compared with those without Gottron′s papules/sign (297.1 ± 140.4 pg/ml, P < 0.05) . The serum levels of IL-10 and IL-18 were significantly higher in the patients with DM/CADM complicated by ILD (18.0 ± 6.7 pg/ml, 552.3 ± 192.8 pg/ml, respectively) than in those without ILD (11.6 ± 6.5 pg/ml, 351.4 ± 101.0 pg/ml, respectively, both P= 0.001) .
Conclusion
Serum levels of IL-6, IL-10 and IL-18 are highly associated with inflammatory biomarkers, skin lesions and ILD in patients with DM/CADM.
Key words:
Dermatomyositis; Cytokines; Lung Diseases, Interstitial; Interstitial lung disease; Clinically amyopathic dermatomyositis
It is critical for laboratories to conduct multianalyzer comparisons as a part of daily routine work to strengthen the quality management of test systems. Here, we explored the application of patient-based real-time quality controls (PBRTQCs) on comparative assays to monitor the consistency among clinical laboratories.
The complex correlation among 4 tests including CMV IgM, pp65 antigenemia, plasma, and cellular human cytomegalovirus (HCMV) Q-polymerase chain reaction (PCR) has not been thoroughly illustrated due to the deficiency of related basic research; a circumstance that greatly hindered the choice of the best method with which to detect HCMV productive replication for clinical use. In this article, 2 study populations, 1 immune competent and the other immune suppressed, were enrolled to analyze the intricate relationship among them using a cross-sectional and longitudinal study. It was found that the degree of agreement between plasma and cellular Q-PCR was always greatest among the tests. In the immunocompetent population, co-positive samples achieved 20.31% in the positive results of cellular Q-PCR and 59.09% in plasma Q-PCR, while the Pearson correlation coefficient (PCC) was 0.3056 (P=0.0001). In the human cytomegalovirus (HSCT) patients, most of the results showed plasma and cellular Q-PCR under the same detected reactivation-episodes with the latter being more sensitive than the former in 84.62% of episodes (22/26). As for the group of pp65 antigenemia and cellular Q-PCR, the agreement was weak in the healthy population (co-positive samples occupied 26.47% in the former with positive results and 14.06% in the latter, while PCC was 0.1024 [P=0.0245]), even though the situation was better in HSCT recipients. However, nearly half of the episodes showed that pp65 antigenemia testing was more sensitive than cellular Q-PCR (17.65%, 3/17) or both of them were not under the same detected CMV-reactivation episodes (23.53%, 4/17). The risk of replacing plasma Q-PCR by cellular Q-PCR should not overcome the benefit of doing so. However, pp65 antigenemia and cellular Q-PCR have complementary characteristics, and it appears that the value of combining these 2 tests would be greater than the value of any single method in clinical use.
Article A protein standard addition method for absolute quantification of cystatin C in human serum by LC-MS/MS was published on October 1, 2021 in the journal Clinical Chemistry and Laboratory Medicine (CCLM) (volume 59, issue 11).
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