Elevated serum B‐cell activating factor levels in patients with dermatomyositis: Association with interstitial lung disease

Objective.Rapidly progressive interstitial lung disease (RP-ILD) is an intractable and fatal complication of juvenile dermatomyositis (JDM). This study evaluated serum levels of B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL) in JDM patients with complicating ILD, and their association with ILD phenotypes, clinical variables, and anti-melanoma differentiation-associated gene 5 (MDA5).Methods.We measured the levels of BAFF, APRIL, and anti-MDA5 in the sera of 23 JDM patients with ILD [8 in the RP-ILD group and 15 in the chronic ILD (C-ILD) group], 17 JDM patients without ILD (non-ILD group), and 10 age-matched controls, using the ELISA method. ILD was identified by high-resolution computed tomography.Results.Serum BAFF titers were significantly higher in the JDM patients with RP-ILD versus those with C-ILD (p = 0.011) and in healthy controls (p = 0.0004). The C-ILD group had significantly higher levels of BAFF versus controls (p ≤ 0.0001). Serum APRIL was markedly elevated in the RP-ILD group as compared with the C-ILD group (p = 0.003) and controls (p = 0.006). In patients with ILD, both BAFF and APRIL levels were correlated with serum Krebs von den Lungen-6 and interleukin 18. Subjects with high titer anti-MDA5 (> 200 U) had higher levels of BAFF and APRIL than those with low titer anti-MDA5 (< 100 U; p = 0.019 and p = 0.0029, respectively), which may have been due to a relationship between RP-ILD and high anti-MDA5 titer.Conclusion.Our findings of markedly elevated levels of BAFF and APRIL in patients with RP-ILD JDM suggest the potential importance of these cytokines in the diagnosis and treatment of RP-ILD accompanying JDM.

Download Full-text

Elevated Serum Krebs von den Lungen-6 in Early Disease Predicts Subsequent Deterioration of Pulmonary Function in Patients with Systemic Sclerosis and Interstitial Lung Disease

The Journal of Rheumatology ◽

10.3899/jrheum.160339 ◽

2016 ◽

Vol 43 (10) ◽

pp. 1825-1831 ◽

Cited By ~ 31

Author(s):

Masataka Kuwana ◽

Yuichiro Shirai ◽

Tsutomu Takeuchi

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Early Stage ◽

Characteristic Curve ◽

Elevated Serum ◽

Initial Assessment ◽

Baseline Serum ◽

Disease Modifying Drugs ◽

Highly Correlated

Objective.To identify predictors of poor prognosis in patients with systemic sclerosis (SSc) associated with interstitial lung disease (ILD).Methods.Fifty patients with early-stage SSc-ILD who had never received disease-modifying drugs and were either observed for ≥ 10 years or died from ILD-related causes were enrolled. The baseline variables of patients who developed endstage lung disease (ESLD) were compared with those of patients who remained ESLD-free, and the Cox proportional hazard model was used to identify initial factors that correlated with ESLD development.Results.Sixteen patients (32%) developed ESLD during 173.5 ± 64.7 months of followup. Elevated serum Krebs von den Lungen-6 (KL-6) at initial assessment was highly correlated with ESLD development (p = 0.0002). Receiver-operating characteristic curve analysis revealed that a KL-6 value of 1273 U/ml effectively discriminated patients who developed ESLD from those who did not. Patients with KL-6 > 1273 U/ml were less likely to remain ESLD-free compared with those with lower KL-6 levels (p < 0.0001). Multivariate analysis showed that KL-6 > 1273 U/ml was the most reliable predictor of ESLD development (OR 51.2, 95% CI 7.6–343, p < 0.0001). Finally, the initial KL-6 level correlated with the forced vital capacity (FVC) decline rate (r = 0.58, p < 0.0001).Conclusion.The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.

Download Full-text

A case of antisynthetase syndrome with elevated serum CA19-9 associated with interstitial lung disease

European Journal of Dermatology ◽

10.1684/ejd.2016.2890 ◽

2017 ◽

Vol 27 (1) ◽

pp. 78-80 ◽

Cited By ~ 1

Author(s):

Shino Minami ◽

Takeshi Nakanishi ◽

Toshihiro Tanaka ◽

Yoshinao Muro ◽

Noriki Fujimoto

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Elevated Serum ◽

Antisynthetase Syndrome

Download Full-text

Role of B Cell Activating Factor in CVID Lung Disease

Journal of Allergy and Clinical Immunology ◽

10.1016/j.jaci.2015.12.722 ◽

2016 ◽

Vol 137 (2) ◽

pp. AB180

Author(s):

Paul J. Maglione ◽

Montserrat Cols ◽

Emma Roellke ◽

Lin Radigan ◽

Charlotte Cunningham-Rundles

Keyword(s):

Lung Disease ◽

B Cell ◽

B Cell Activating Factor

Download Full-text

Interstitial Lung Disease in Patients with Diffuse Large B-Cell Lymphoma Receiving CHOP-Based Chemotherapy Is Associated with the Use of Rituximab.

Blood ◽

10.1182/blood.v110.11.3435.3435 ◽

2007 ◽

Vol 110 (11) ◽

pp. 3435-3435 ◽

Cited By ~ 1

Author(s):

Ronald S. Go ◽

Kevin M. Riggle ◽

Sue A. Beier-Hanratty ◽

Jacob D. Gundrum ◽

Jonean E. Schroeder ◽

...

Keyword(s):

Risk Factors ◽

Interstitial Lung Disease ◽

Lung Disease ◽

B Cell ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Imaging Studies ◽

Cardiopulmonary Disease ◽

Large B Cell Lymphoma ◽

Large B Cell

Abstract Background: Several cases of chemotherapy-induced interstitial lung disease (ILD) or pneumonitis have been reported in recent years in patients with lymphoma. The potential roles of rituximab (R) and granulocyte colony stimulating factor (GCSF), agents more commonly used in recent years, are suggested. Objective: We wanted to determine the prevalence of ILD and identify risk factors in patients with diffuse large B-cell lymphoma (DLCL) who received cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP)-based chemotherapy. Methods: Selection criteria included newly diagnosed DLCL patients treated at our institution from 2000–2006 who received CHOP with or without R and had at least 3 serial CT or PET/CT scans during chemotherapy. ILD was defined as new bilateral interstitial pulmonary infiltrates not likely representing fluid overload or fibrosis. One radiologist blinded to clinical data reviewed all imaging studies. Results: Out of a total of 174 new cases of DLCL, 73 met our study criteria. Reasons for exclusion included no chemotherapy (45), <3 imaging studies (46), and non-CHOP-based chemotherapy (10). Among the 73 study patients, 52 (71%) received R in addition to CHOP. Eleven (15.1%) patients developed ILD, all in the subgroup that received RCHOP (P = 0.027). Most occurred between cycles 2 and 4 of RCHOP (81.8%) and persisted until after completion of chemotherapy (63.6%). Nine (81.8%) patients with ILD were asymptomatic and never required treatment or delay of RCHOP. The remaining 2 patients became symptomatic (1 hospitalized), were empirically treated for atypical pneumonia with clinical recovery, and had delay of RCHOP. All patients received the intended number of courses of RCHOP. Univariate analysis of potential ILD risk factors among those who received RCHOP showed a trend with the subgroup that either had GCSF or cardiopulmonary disease (P = 0.09). Multivariate analysis using a two-variable model suggests that the use of GCSF or presence of cardiopulmonary disease (P = 0.065) and a high (3–5) international prognostic index score (P = 0.13) need further investigation as risk factors. Conclusions: In our cohort of DLCL patients receiving CHOP-based chemotherapy, ILD was common and significantly associated with the use of R. While most cases were asymptomatic, self-limited, and did not require delay of chemotherapy, more serious presentation could occur. The mechanism of ILD is unknown and requires further investigation.

Download Full-text

Elevated serum D-dimer level is associated with an increased risk of acute exacerbation in interstitial lung disease

Respiratory Medicine ◽

10.1016/j.rmed.2017.05.009 ◽

2017 ◽

Vol 128 ◽

pp. 78-84 ◽

Cited By ~ 8

Author(s):

Genta Ishikawa ◽

Samuel O. Acquah ◽

Mary Salvatore ◽

Maria L. Padilla

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Acute Exacerbation ◽

Elevated Serum ◽

Increased Risk ◽

D Dimer

Download Full-text

Elevated carcinoembryonic antigen predicts rapidly progressive interstitial lung disease in clinically amyopathic dermatomyositis

Rheumatology ◽

10.1093/rheumatology/keaa819 ◽

2021 ◽

Author(s):

Dingxian Zhu ◽

Jianjun Qiao ◽

Shunli Tang ◽

Yunlei Pan ◽

Sheng Li ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Carcinoembryonic Antigen ◽

Elevated Serum ◽

Multivariate Logistic Regression Analysis ◽

Laboratory Findings ◽

Serum Carcinoembryonic Antigen ◽

Lower Lung ◽

Serum Cea ◽

Lung Zone

Abstract Objectives The present study aimed to determine the correlation between serum carcinoembryonic antigen (CEA) level and the severity of interstitial lung disease (ILD) in clinically amyopathic DM (CADM) patients. Methods We performed a retrospective study including 41 Chinese CADM patients without malignancy. Serum CEA levels, clinical and laboratory findings were collected. Association tests between CEA levels and disease activity parameters were performed. Results Among the 41 patients, 16 (39.0%) developed rapidly progressive (RP)-ILD; of them, 14 (87.5%) had elevated serum CEA levels. Multivariate logistic regression analysis indicated that an elevated serum CEA level was an independent risk factor for RP-ILD. The incidence of elevated CEA level was significantly higher in patients with RP-ILD than in those without RP-ILD (87.5 vs 16.0%, P < 0.001). Furthermore, CEA levels were higher in patients with CADM with RP-ILD [26.87 (6.71) μg/l] than in those without RP-ILD [3.23 (0.64) μg/l] (P < 0.001). CEA levels in CADM patients were associated with the ferritin, alanine aminotransferase, aspartate aminotransferase and lactate dehydrogenase levels, and CT scores of the lungs. Also, elevated CEA levels are related to the organizing pneumonia pattern and lower lung zone consolidation in high-resolution CT. Moreover, the cumulative survival rate was significantly lower (68.4 vs 31.6%, P < 0.001) in the group with a CEA level >8.75 μg/l than that in the group with a CEA level <8.75 μg/l. Conclusions An elevated serum CEA level is common in patients with CADM, and a higher serum CEA level is a powerful indicator of RP-ILD and poor prognosis in those patients.

Download Full-text