Association Between Peripheral Eosinophilia and Clinical Characteristics of Adult-onset Still's Disease with Persistent Eruption: A Retrospective Study.

Persistent eruption occurs in a subset of patients with adult-onset Still's disease. In our experience, a considerable proportion of these patients present with peripheral eosinophilia. The aims of this study were to summarize the clinical and histological features of patients with adult-onset Still's disease with persistent eruption in the current study cohort, and to assess the association between peripheral eosinophil levels and disease characteristics. A total of 21 patients with adult-onset Still's disease with persistent eruption were included in this retrospective study. Koebner signs, an important diagnostic clue, were found in 85.7% of patients. The proportion of patients presenting with eosinophilia within the disease course was 57.1%. Skin histology revealed infiltration of eosinophils in 90.5% of patients. Peripheral eosinophil levels were positively associated with involved body surface area. Patients with normal peripheral eosinophil counts were more likely to achieve complete remission than those with abnormal peripheral eosinophil counts. Eosinophils may be involved in the pathogenesis of skin eruption. Abnormal peripheral eosinophil counts in these patients may indicate a more refractory disease course.

Distinctive Clinical Characteristics and Outcome of ILD-Onset Rheumatoid Arthritis and ACPA-Positive ILD: a Longitudinal Cohort of 282 Cases.

The aim of this study is to investigate the clinical features and outcome of interstitial lung disease (ILD)-onset rheumatoid arthritis (RA) and anti-citrullinated protein antibody (ACPA)-positive ILD-only patients. Arthritis-onset and ILD-onset RA-ILD and ACPA-positive ILD-only patients consecutively admitted to Peking Union Medical College Hospital from January 2008 to December 2017 were enrolled and followed-up. Their demographic, clinical, and laboratory features as well as outcome were collected and analyzed. Compared with arthritis-onset RA-ILD (n = 166, median arthritis-to-ILD interval: 60 months), the ILD-onset RA-ILD (n = 75, median ILD-to-arthritis interval: 2 months) had less rheumatoid nodules and higher titer of ACPA, and manifested more stable ILD (median estimated progression-free survival: 120 vs. 100 months, p = 0.019). Elder age (≥ 65 years) at ILD diagnosis and UIP pattern were associated with ILD progression by both univariate and Cox hazards modeling analysis (p < 0.05). In ACPA-positive ILD-only patients (n = 41), arthritis developed in 7 (17.1%) female patients after a median interval of 24 months. ACPA-positive ILD who subsequently developed arthritis exhibited higher frequency of rheumatoid factor (RF), higher titer of ACPA, and higher levels of ESR and CRP (p < 0.05). Multivariate regression analysis showed that positive RF (OR 12.55, 95% CI 1.31 to 120.48) was the independent risk factor for arthritis development in ACPA-positive ILD-only patients. ILD-onset RA-ILD had more stable ILD compared with arthritis-onset RA-ILD. ACPA-positive ILD patients with positive RF are at increased risk of developing RA.

[Chest High-resolution Computed Tomography Imaging Features of Lung Involvement in Rheumatoid Arthritis].

Objective To explore the chest high-resolution computed tomography (HRCT) features in patients with rheumatoid arthritis (RA) complicated with pulmonary involvement. Methods Totally 161 patients with RA with lung involvement were collected from June 2014 to May 2018. The chest HRCT findings were retrospectively analyzed. According to the imaging features as well as the results of history taking,pulmonary function test,pathology,and bronchoalveolar lavage fluid test,RA-related lung diseases (RA-LD) were classified and their clinical characteristics were compared. Results These 161 RA-LD patients (56 males and 105 females) whose mean age at diagnosis was (60.7±12.8) years (14-85 years) included 87 cases of usual interstitial pneumonia (UIP) (including 16 cases of possible UIP),44 cases of non-specific interstitial pneumonia (NSIP),10 cases of organizing pneumonia,7 cases of lymphocytic interstitial pneumonia,9 cases of small airway disease (SAD) (including 8 cases of bronchiolitis obliterans and 1 case of follicular bronchiolitis),and 4 other lung manifestations (including 3 cases of diffuse alveolar hemorrhage and 1 case of rheumatoid nodules). The UIP group had the oldest average age [(63.3±12.1) years old] and the highest smoking rate (41.4%). The SAD group had the youngest average age [(54.7±15.1) years old] and there was no smoking history. There were significant differences between these two groups (P=0.020,P<0.001). Seventy patients (43.5%) with RA-LD were complicated with pleural lesions. Conclusions RA involving the lung is common and has varied imaging manifestations,with interstitial lung diseases (mainly UIP and NSIP) being the most important manifestations. RA patients should undergo lung HRCT as early as possible to identify the lung involvement and related types.

Obstructive uropathy associated with rheumatoid arthritis successfully treated with steroids and immunosuppressive therapy: A case report.

RATIONALE: Urinary obstruction are relatively rare complications of autoimmune diseases including systemic lupus erythematosus and systemic vasculitis. It has never been reported in rheumatoid arthritis (RA). PATIENT CONCERNS: We report a case of a female patient with seropositive RA who presented with gross hematuria associated with worsening joint symptoms, found to have acute kidney injury (AKI), bilateral hydronephrosis with bilateral renal pelvis, and ureteral wall thickening. Uroscopy with biopsy demonstrated inflammation without evidence of malignancy. DIAGNOSES: Rheumatoid arthritis related inflammation and obstruction of the urinary tract. INTERVENTIONS: Prednisone 50 mg daily (tapering began 1 month later), iguratimod 50 mg daily, and leflunomide 20 mg daily were prescribed. OUTCOMES: The patient responded well to steroids and immunosuppressive therapy with complete resolution of hematuria, renal injury, and hydronephrosis. LESSONS: Our case showed that RA might cause local inflammation involving the urinary tract which leads to obstruction and AKI.

The efficacy and safety of antithrombotic therapy in patients with positive antiphospholipid antibodies receiving invasive procedures: experience from a single tertiary center.

OBJECTIVES: To evaluate the efficacy and safety of antithrombotic prophylaxis and to explore potential risk factors for thrombotic/bleeding events in patients with positive antiphospholipid (aPL) antibodies receiving invasive procedures. METHOD: All aPL-positive patients who underwent invasive procedures in Peking Union Medical College Hospital, from January 2002 to April 2018, were retrospectively enrolled. Demographic features, clinical features, antiphospholipid antibody profiles, types of invasive procedures, and antithrombotic management, as well as complications and outcomes, were systematically reviewed and recorded. RESULTS: A total of 111 aPL-positive patients with 130 invasive procedures were enrolled. One hundred nine (83.8%) cases were on regular antithrombotic therapy which started at least 1 month prior to the invasive procedures, with 58 (44.6%) receiving anticoagulation therapy, 27 (20.8%) receiving antiplatelet therapy, and 24 (18.5%) receiving both. During the periprocedural period, the median time free of antithrombotic therapy was 2.5 days (interquartile range 1.5-6.0 days). Two (1.5%) periprocedural thrombotic events and 18 (13.8%) bleeding events were identified. Large open/laparoscopic surgeries of the thorax and abdomen were associated with a higher risk of bleeding (OR 3.46, 95% CI 1.24-9.67, p = 0.014). All bleeding events were manageable and not life-threatening. CONCLUSIONS: Aggressive antithrombotic therapy was associated with fewer thrombotic events in aPL-positive patients receiving invasive procedures, but might contribute to an increased bleeding rate, especially in large open surgeries. This study justifies more caution in prophylactic antithrombotic therapy in periprocedural aPL-positive patients.

The renal manifestations of type 4 familial partial lipodystrophy: a case report and review of literature.

BACKGROUND: Lipodystrophy syndromes are rare disorders of variable body fat loss associated with potentially serious metabolic complications. Familial partial lipodystrophy (FPLD) is mostly inherited as an autosomal dominant disorder. Renal involvement has only been reported in a limited number of cases of FPLD. Herein, we present a rare case of proteinuria associated with type 4 FPLD, which is characterized by a heterozygous mutation in PLIN1 and has not been reported with renal involvement until now. CASE PRESENTATION: A 15-year-old girl presented with insulin resistance, hypertriglyceridaemia, hepatic steatosis and proteinuria. Her glucose and glycated haemoglobin levels were within normal laboratory reference ranges. A novel heterozygous frameshift mutation in PLIN1 was identified in the patient and her mother. The kidney biopsy showed glomerular enlargement and focal segmental glomerulosclerosis under light microscopy; the electron microscopy results fit with segmental thickening of the glomerular basement membrane. Treatment with an angiotensin-converting enzyme inhibitor (ACEI) decreased 24-h protein excretion. CONCLUSIONS: We report the first case of proteinuria and renal biopsy in a patient with FPLD4. Gene analysis demonstrated a novel heterozygous frameshift mutation in PLIN1 in this patient and her mother. Treatment with ACEI proved to be beneficial.

Endobronchial Ultrasound-guided Transbronchial Needle Aspiration versus Standard Bronchoscopic Modalities for Diagnosis of Sarcoidosis: A Meta-analysis.

BACKGROUND: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is an effective technique used to precisely detect enlarged mediastinal lymph nodes. The efficacy of EBUS-TBNA versus standard modalities for the diagnosis of sarcoidosis remains to be elucidated. In this meta-analysis, we compared the efficacies of these methods. METHODS: We searched PubMed, Embase, The Cochrane Library, Wanfang, Cpvip, CNKI, and the bibliographies of the relevant references. We analyzed the data obtained with Revman 5.2 (Nordic Cochrane Center, Copenhagen, Denmark) and Stata 12.0 software (Stata Corporation, College Station, TX, USA). The Mantel-Haenszel method was used to calculate the pooled odds ratio (OR) and 95% confidence intervals (CIs). RESULTS: Sixteen studies with a total of 1823 participants met the inclusion criteria, and data were extracted regarding the diagnostic yield of each approach. The ORs for EBUS-TBNA versus transbronchial lung biopsy (TBLB) for the diagnosis of sarcoidosis ranged from 0.26 to 126.58, and the pooled OR was 5.89 (95% CI, 2.20-15.79, P = 0.0004). These findings indicated that EBUS-TBNA provided a much higher diagnostic yield than TBLB. The pooled OR for EBUS-TBNA + TBLB + endobronchial biopsy (EBB) versus TBNA + TBLB + EBB was 1.54 (95% CI, 0.61-3.93, P = 0.36), implying that there was no significant difference between their diagnostic yields. However, clinical heterogeneity was reflected in the nature of the studies and in the operative variables. CONCLUSIONS: The results of this meta-analysis suggest that EBUS-TBNA + TBLB + EBB could be used for the diagnosis of sarcoidosis, if available. At medical centers without EBUS-TBNA, TBNA + TBLB + EBB could be used instead.

Tigecycline-induced Drug Fever and Leukemoid Reaction: A Case Report.

In this study, we describe a patient in whom tigecycline-induced drug fever and leukemoid reaction (LR) after 3 weeks of therapy for pneumonia.A 62-year-old man developed aspiration pneumonia on February 1, 2015. He had received multiple antibiotics at another hospital, but did not respond well. Disease rapidly progressed, and he was referred to our department on February 14. We adjusted the antibiotic therapy to tigecycline + vancomycin, and added voriconazole to empiric antifungal therapy. Pneumonia largely improved, and we discontinued vancomycin and voriconazole on February 28. With tigecycline monotherapy, his clinical status remained stable.On March 7, he developed high fever and LR (white blood cell count: 38.25 × 10(9)/L). Erythrocyte sedimentation rate and C-reactive protein were elevated, and CD8+ T cells had been abnormally activated. After a careful physical examination and laboratory investigation, we confirmed that primary infection did not progress and no other cause was evident. So we figured fever and LR might be induced by tigecycline. After discontinuing tigecycline and adding low-dose steroid, fever and LR totally resolved in 3 days, which further confirmed our diagnosis.According to this case and literature review, drug-induced hypersensitivity should be considered in the differential diagnosis of fever and LR when the therapeutic duration of tetracycline approximates 3 weeks. Monitoring T-cell subsets may facilitate early diagnosis. When necessary, we should discontinue the suspected drug to confirm diagnosis.