Pulmonary arterial hypertension(PAH) is one of the most severe complications of systemic lupus erythematosus(SLE).Recent studies emphasized the role of T and B lymphocytes in the pathogenesis of PAH.Rituximab (RTX),not only targeting at B lymphocytes,but also been proven to affect T cells,thus could be a potential treatment for SLE-PAH.

Objective To review the ophthalmic features of granulomatosis with polyangiitis (GPA).Methods Clinical data of 164 GPA patients diagnosed in Peking Union Medical College Hospital during the past 10 years were retrospectively reviewed.Results Among 164 patients,48 (29.3％) had ocular involvement with a male to female ratio of 1.4:1 and an average age of (45 ± 15) years.Nine patients (5.5％) were first diagnosed at the ophthalmology department.Scleritis (15.9％,26/164),orbit lesions (13.4％,22/164) and peripheral corneal ulcer (9.1％,15/164) were the three most common ophthalmic complications.Twenty-two patients (13.4％) had positive findings in the orbit imaging.Six patients (3.7％,6/164) lost their vision due to GPA.Conclusion The ocular manifestations of GPA are diverse and complicated.GPA may affect every component of the eye,sclera,orbit and cornea are the most frequently affected sites of the eye.

A 29-year-old woman was admitted to the Department of Rheumatology,Peking Union Medical College Hospital due to intermittent rashes,fever and headache.Palpable purpura were symmetrically distributed on the extremities and trunk.Other manifestations included headache with nausea and vomiting.Elevated white blood cell (WBC) count,platelet (PLT) count,erythrocyte sedimentation rate (ESR) and C-reactive protein were the main laboratory findings.Antinuclear antibodies and antineutrophil cytoplasmic antibodies were negative.Examination of the cerebrospinal fluid (CSF) revealed high intracranial pressure,while routine cytology and biochemical tests of CSF were normal.Head MRI scan and PET-CT did not detect remarkable findings.A diagnosis of systemic vasculitis was confirmed by the biopsy of skin lesion which showed inflammatory infiltration of the muscular vessel wall.Combination therapy of corticosteroids and cyclophosphamide lead to a rapid improvement in clinical symptoms and laboratory parameters.The patient was in stable remission till 6 month follow-up.

With the recognition of antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and widespread vaccination against viral hepatitis B, the prevalence of polyarteritis nodosa (PAN) varied considerably. In our study, patients diagnosed as polyarteritis nodasa (PAN)based on the 1990 American College of Rheumatology(ACR) criteria were reclassified using 2007 European Medicines Agency(EMA) algorithm modified by 2012 Chapel Hill Consensus Conference(CHCC) definitions, aiming to evaluate the new classification criteria for the diagnosis of PAN. A total of 113 PAN patients admitted to Peking Union Medical College Hospital from January 2002 to December 2018 were retrospectively analyzed, who were classified into three subtypes including 9 patients with cutaneous, 80 with classic and 24 Hepatitis B virus (HBV) associated PAN. All patients were reclassified according to 2007 EMA algorithm using CHCC 2012 definitions. As a result, 7 patients were diagnosed as microscopic polyangiitis(MPA) and 19 patients with unclassified vasculitis based on the new classification criteria. The diagnostic rate of PAN was gradually declined as the classification criteria of vasculitides was update. However, there are quite a few PAN patients in China, whom rheumatologists should pay attention to the early diagnosis and treatment.

A 17-year-old young man with a history of swollen leg and intermittent jaundice was presented to Peking Union Medical College Hospital with acute fever and mental disturbance.He developed deep venous thrombosis,acute myocardial infarction and plantar skin necrosis during the past four years,and was presented with an acute episode of fever,thrombocytopenia,acute kidney injury,acute myocardial infarction,mental disturbance,and obstructive jaundice.Laboratory tests showed schistocytes on peripheral blood smear.High titer of antiphospholipid antibodies was detected.Strikingly,the activity of a disintegrin and metalloprotease with a thrombospondin type 1 motif,member 13 (ADAMTS13)was significantly decreased without the production of inhibitors.Images indicated stenosis of the common bile duct,common hepatic duct,and cystic duct,which caused dilation of bile ducts and the gall bladder.Corticosteroids and anticoagulation therapy were effective at first,but the disease relapsedonce the corticosteroids tapered down.Plasma exchange was administrated for 17 times,which was effective temporarily during this episode.Methylprednisolone pulse therapy,intravenous immunoglobulin,rituximab,anticoagulation therapy,and bile drainage,were all tried but still could not control the disease.The patient's family agreed to withdraw treatment after he developed septic shock.

Objective To determine the clinical significance of pulmonary function test(PFT)in evaluating the features and severity of lung impairments associated with connective tissue diseases(CTD)by comparing the differences of pulmonary function test parameters among groups of CTD associated pulmonary disorders.Methods Cases of CTD associated pulmonary disorders were prospectively enrolled and assigned into 3 groups according to their lung impairments:CTD associated pulmonary arterial hypertension group (CTD-PAH,n=29),CTD associated interstitial lung disease group(CTD-ILD,n=35),CTD associated PAH plus ILD group(CTD-PAH+ILD,n=16)and CTD control group(n=34).Pulmonary function test parameters,including total lung capacity(TLC % predicted),forced vital capacity(FVC % predicted),forced expiratory volume in the first second(FEV_(1.0)% predicted),FE_(1.0)%/FVC and diffusing capacity of the lung for carbon monoxide(DLco,% predicted)were measured and compared among the four groups.Results One hundred and forteen eases were included and predominantly female with average onset age of 35～39 years old.CTDs that were predisposed to lung diseases were mixed connective disease(MCTD),systemic sclerosis(SSc),systemic lupus erythematosus(SLE)and primary Sj(o)ren syndrome(pSS),in order.There were 10,29 and 46 percent of patients presented with decreased TLC% in CTD-PAH,CTD-ILD and CTD-PAH+ILD group respectively,50,36 and 71 percent of patients with decreased FVC% respectively,54,47 and 71 percent of patients with decreased FEV_(1.0)% respectively,and 100,82 and 100 percent with decreased DLco% respectively.ANOVA analysis demonstrated that TLC%,FVC%,FEV_(1.0)%,DLco% had significant differences between CTD control group and each of the CTD associated lung disease group(P＜0.05),although none of them was lack of difference between the PAH and ILD groups.TLC% was significantly higher in CTD-PAH group than CTD-PAH+ILD group[(89±15)% vs(79±12)%,P＜0.05],while FVC% was significantly lower in CTDPAH+ILD group either than CTD-PAH group or than CTD-ILD group[(81±13)%,(80±16)% vs(65±22)%,P＜0.05].ConclusionPulmonary function test may be valuable in early screening for CTD associated lung disorders than distinguishing CTD-PAH from ILD,which usually reveal restrictive ventilation dysfunction and/or diffusing capacity dysfunction.

Objective To analyze the clinical information of a family with one patient who have systemic lupus erythematosus (SLE) and Fabry disease,as well as the enzymatic activity and gene mutation in these family members.Methods Clinical characteristics were collected from the proband and her family members.Peripheral blood samples from three members of this family were collected and the enzymatic activity was measured by fluorimetrie substrate assay.Genomic DNA was extracted from one male member with significantly decreased enzyme activity,the 7 exons and their flanking introns of GLA gene were amplified by PCR and directly sequenced.Results The enzyme activity of two family members was significantly decreased,the genetic analysis of the male member revealed a missense mutation in exon 2:c.334C＞T (CGC＞TGC)( p.R112C ).Family members except the proband had no definite evidence to support the presence of SLE.Conclusion The coexistence of SLE and Fabry disease is extremely rare.Immunological test,enzymatic activity and gene mutation analysis seem to be helpful for the differential diagnosis.

Objective To explore the potential role of brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide(NT-proBNP) in the assessment of patients with connective tissuediseases(CTD) associated pulmonary arterial hypertension (PAH). Methods Thirty patients with CTD associated PAH were evaluated by WHO function, echocardiography, right heart catheterization and blood biomarkers. All the clinical data was analyzed statistically. Results All patients [age (39.5 ± 11.6) yr]were female. Both NT-proBNP and BNP were significantly increased and significantly correlated ( all P ＜0. 05 ) respectively with mean pulmonary arterial pressure ( r = 0. 53 and r = 0. 40 ), right arterial pressure ( r = 0. 55 and r = 0. 42 ), pulmonary vascular resistance ( r = 0. 69 and r = 0. 61 ), cardiac index ( r = - 0. 58and r = - 0. 46), mixed venous blood oxygen saturation ( r = - 0. 62 and r = - 0. 54 ), pericardial effusion ( r = 0. 46 and r = 0. 43 ), right atrial sizes ( r = 0. 40 and 0. 53, and r = 0. 39 and 0. 45 ) and right ventricular size ( r = 0. 55 and r = 0. 37 ). Furthmore, NT-proBNP, but not BNP, significantly correlated with WHO function class ( r = 0. 55 ). Conclusion Blood NT-proBNP and BNP were elevated in patients with CTD associated PAH and paralleled the extent of function class, pulmonary hemodynamic changes and right ventricular remodeling.

Objective To explore the efficacy and safety of tacrolimus among Chinese Takayasu arteritis (TAK) patients. Methods This was a single center, prospective study of active TAK patients treated with tacrolimus. Clinical manifestations, white blood cell count, hemoglobin level, erythrocyte sedimentation rate (ESR), hypersensitivity C reactive protein (hsCRP), alanine and aspartate aminotransferase and serum creatinine were recorded before and during tacrolimus treatment. Vascular changes were repeated every 6 months during tacrolimus treatment. All data were analyzed by statistical product and service solutions (SPSS) 20.0 statistical software, unpaired t test and Fisher exact probability and Kruskal-Wallis H test were used for statistical analysis. Results A total of 19 consecutive patients with an average age of (26 ±6) years were analyzed in this study. Sixteen of them were women. Pulselessness, fatigue, asymmetric blood pressure and fever were the most common clinical findings. Cervical and subclavian artery were more vulnerable. The most common artery involvement pattern was Numano type Ⅰ, followed by type Ⅱa and type Ⅴ. The median tacrolimus dosage was 2(2, 3) mg. Tacrolimus was effective in 9 out of the 19 patients. Patients who responded to tacrolimus tended to have lower mean ESR [(33±29) mm/1 h vs (42±20) mm/1 h, t=-0.776, P=0.448] and hsCRP [(20 ±31) mg/L vs (54 ±45) mg/L, t=-1.758, P=0.099] levels. However, no statistical significance was observed. During tacrolimus treatment, no drug related side effect was observed. Conclusion Tacrolimus is an alternative and effective therapy for some of the TAK patients.

A 53-year-old woman presented with arthralgia and dyspepsia on exertion.Symmetrical joint swelling and pain of bilateral hands with pigmentation sedimentation in the articular surfaces,dry cough and short of breath were the main clinical manifestations.Elevated levels of erythrocyte sedimentation rate (89 mm/1 h),C-reactive protein (64.45 mg/L),IgG (31.22 g/L) and IgA (5.44 g/L).Rheumatoid factor was positive and anti-cyclic citrullinated peptide antibody was negative.Chest high-resolution computed tomography scan found that diffusely distributed lung nodules,round cysts with varying sizes and patchy ground glass opacities.A significant plasma cell infiltration and amyloid deposition were seen in lung tissue.The patient was finally diagnosed as rheumatoid arthritis,interstitial lung disease and pulmonary nodules.Combination therapy of prednisone 7.5 mg qodpo,thalidomide 50 mg qdpo,tocilizumab 560 mg iv once per month for 1 month.Chest computed tomography showed stable lung nodules,however,pulmonary interstitial lesions gradually aggravated.