OBJECTIVE: To investigate the clinical and immunological characteristics of anti-synthetase syndrome (ASS) patients overlap with rheumatoid arthritis (RA). METHODS: A retrospective analysis was conducted on ASS patients with arthritis who were treated at Peking University People' s Hospital. Data collected included demographic information, clinical manifestations, laboratory features, lymphocyte subsets in peripheral blood, and treatments. The patients with ASS were divided into two groups based on the presence or absence of RA for comparative analysis. RESULTS: A total of 104 ASS patients with arthritis were included, among whom 23.1% (24/104) were diagnosed with RA. The ASS with RA group had a significantly higher incidence of rapidly progressive interstitial lung disease (RP-ILD) (41.7% vs. 17.6%, P=0.032), number of tender joints [10 (7, 14) vs. 4 (0, 8), P < 0.001], number of swollen joints [4 (2, 8) vs. 2 (0, 4), P=0.012], and rate of bone erosion (47.8% vs. 2.5%, P < 0.001) compared with the non-RA group. Levels of platelets [(289.57±68.74)×10³/μL vs. (247.94±77.04)×10³/μL, P=0.022], erythrocyte sedimentation rate (ESR) [43 (19, 59) mm/h vs. 18 (10, 44) mm/h, P=0.019], and C-reactive protein (CRP) [19.20 (4.80, 55.36) mg/L vs. 5.68 (1.10, 14.96) mg/L, P=0.006] were found significantly higher in the ASS with RA group than those in non-RA group. Analysis of immune cells in peripheral blood mononuclear cell (PBMC) showed that significantly decreased proportions of CLA⁺ Treg cells [(11.12±4.10)% vs. (17.22±8.49)%, P=0.003], B cells [8.56% (4.80%, 11.90%) vs. 14.55% (8.75%, 20.29%), P=0.025], and natural killer (NK) cells [7.56% (4.65%, 13.20%) vs. 13.25% (7.46%, 19.25%), P=0.045] in the overlap group compared with non-RA group. Proportion of Naïve Th cells [(52.66±17.66)% vs. (40.76±14.96)%, P=0.033)] was significantly increased in overlap group compared with non-RA group. Overlap group had lower rate of complete clinical response than non-RA group (16.7% vs. 43.8%, P=0.031). CONCLUSION Among ASS patients with arthritis, those with RA have more severe lung and joint involvement and a lower treatment response rate, highlighting the need for early recognition and aggressive intervention.
Humans ; Arthritis, Rheumatoid/immunology* ; Retrospective Studies ; Lung Diseases, Interstitial/immunology* ; Male ; Myositis/blood* ; Female ; Middle Aged ; Autoantibodies/blood*

BACKGROUND/AIMS: To evaluate the impact on mortality of anti-tumor necrosis factor (anti-TNF) treatment of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). METHODS: We retrospectively reviewed the medical records of 100 RA-ILD patients who visited our tertiary care medical center between 2004 and 2011, identified those treated with an anti-TNF agent, divided patients into non-survivor and survivor groups and evaluated their clinical characteristics and causes of death. RESULTS: A total of 24 RA-ILD patients received anti-TNF therapy, of whom six died (25%). Mean age at initiation of anti-TNF therapy was significantly higher in the nonsurvivor versus survivor group (76 years [range, 66 to 85] vs. 64 years [range, 50 to 81], respectively; p = 0.043). The mean duration of anti-TNF treatment in the non-survivor group was shorter (7 months [range, 2 to 14] vs. 23 months [range, 2 to 58], respectively; p = 0.030). The duration of anti-TNF therapy in all nonsurviving patients was < 12 months. Pulmonary function test results at ILD diagnosis, and cumulative doses of disease-modifying drugs and steroids, did not differ between groups. Five of the six deaths (83%) were related to lung disease, including two diffuse alveolar hemorrhages, two cases of acute exacerbation of ILD, and one of pneumonia. The sixth patient died of septic shock following septic arthritis of the knee. CONCLUSIONS: Lung complications can occur within months of initial anti-TNF treatment in older RA-ILD patients; therefore, anti-TNF therapy should be used with caution in these patients.
Adult ; Aged ; Aged, 80 and over ; Antirheumatic Agents/adverse effects/*therapeutic use ; Arthritis, Rheumatoid/complications/diagnosis/*drug therapy/immunology/mortality ; Female ; Humans ; Lung Diseases, Interstitial/diagnosis/etiology/*mortality ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Tertiary Care Centers ; Time Factors ; Treatment Outcome ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors

PURPOSE: Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. MATERIALS AND METHODS: A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. RESULTS: Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. CONCLUSION: We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category.
Aged ; Autoantibodies/*blood/immunology ; Female ; Humans ; Lung Diseases, Interstitial/*diagnosis/immunology/physiopathology ; Male ; Middle Aged ; Myositis/*immunology/physiopathology ; Respiratory Function Tests

We describe an unusual case of systemic lupus erythematosus with pulmonary manifestations presenting as hypoglycemia due to anti-insulin receptor antibodies. A 38-year-old female suffered an episode of unconsciousness and was admitted to hospital where her blood glucose was found to be 18 mg/dL. During the hypoglycemic episode, her serum insulin level was inappropriately high (2,207.1 pmol/L; normal range, 18 to 173) and C-peptide level was elevated (1.7 nmol/L; normal range, 0.37 to 1.47). Further blood tests revealed the presence of antinuclear antibodies, anti-double-stranded DNA antibodies, and anti-Ro/SSA, anti-La/SSB, anti-ribonucleoprotein, and anti-insulin receptor antibodies. A computed tomography scan of the abdomen, aimed at tumor localization, such as an insulinoma, instead revealed ground-glass opacities in both lower lungs, and no abnormal finding in the abdomen. For a definitive diagnosis of the lung lesion, video-associated thoracoscopic surgery was performed and histopathological findings showed a pattern of fibrotic non-specific interstitial pneumonia.
Adult ; Autoantibodies/*blood ; *Autoimmunity ; Biological Markers/blood ; Blood Glucose/metabolism ; Female ; Humans ; Hypoglycemia/blood/*complications/immunology ; Insulin/blood ; *Insulin Resistance ; Lung Diseases, Interstitial/diagnosis/*etiology/immunology/surgery ; Lupus Erythematosus, Systemic/*complications/diagnosis/immunology ; Receptor, Insulin/*immunology ; Thoracic Surgery, Video-Assisted ; Tomography, X-Ray Computed ; Treatment Outcome

BACKGROUND: This study was purposed to find out the differences in the lymphocyte subsets and differential cell counts of the bronchoalveolar lavage (BAL) fluid in patients with interstitial lung disease (ILD) and to analyze the differences according to their ages, gender and smoking habits. METHODS: BAL fluid samples of 141 ILD patients were examined for lymphocyte subsets and differential cell counts, and the differences among the patients were analyzed according to their diseases. Then, within the three most common disease groups, the differences were further analyzed by the age, gender and smoking habit of the patients. RESULTS: There were no statistically significant differences in total cell counts (per millimeters of BAL fluid) among the patient groups with each ILD. However, significant differences were observed in the percentages of neutrophils, lymphocytes, eosinophils, and macrophages of BAL fluid. Also, in lymphocyte subset analyses, the percentages of total T cells, B cells, CD4+ T cells, CD8+ T cells, CD4/CD8 T cell ratios, and NK cells were significantly different among the patients with each ILD. However, within the same disease group, there were no differences in differential cell counts and lymphocyte subset analyses according to the age, smoking habit, and gender of the patients. CONCLUSIONS: Although the age, smoking habit and gender did not have an effect on the BAL fluid analyses among the patients with the same ILD, there were significant differences among the patients with each ILD; therefore, the differential cell counts and lymphocyte subset analyses of BAL fluid can be useful in differential diagnosis for determining the types of ILD.
Adult ; Aged ; Bronchoalveolar Lavage Fluid/*cytology ; Diagnosis, Differential ; Female ; Humans ; Lung Diseases, Interstitial/diagnosis/*epidemiology/etiology ; Lymphocyte Count ; Lymphocyte Subsets/immunology ; Male ; Middle Aged ; Smoking

Intravascular lymphomatosis (IL) is a rare and generally fatal disease characterized by proliferation of large lymphoma cells almost exclusively within the lumen of small blood vessels. The skin and central nervous systems are typically affected, but involvement of other organs, such as lung, has been described. Predominant lung involvement without cutaneous and neurologic manifestation is very rare and difficult to diagnose. Originally considered as an endothelial disorder, IL has recently been reclassified as lymphoma. Most of the cases reported are of B cell lineage with a few cases of T cell type. We describe a case of the T-cell type IL manifested clinically as an interstitial lung disease without involvement of skin and central nervous systems. Immunohistochemical studies showed the T-cell nature of the neoplastic cells in open lung biopsy sample.
Blood Vessels/pathology ; Case Report ; Diagnosis, Differential ; Fatal Outcome ; Human ; Lung Diseases, Interstitial/pathology* ; Lymphoma, T-Cell/physiopathology ; Lymphoma, T-Cell/pathology* ; Lymphoma, T-Cell/immunology ; Male ; Middle Age ; Tomography Scanners, X-Ray Computed