Objective:To investigate the clinical characteristics of adult patients with autoimmune disease-associated hemophagocytic syndrome (AID-HPS) and enhance clinical recognition of this condition.Methods:A retrospective analysis was performed on 85 adult AID-HPS patients admitted to the department of rheumatology and immunology at Peking Union Medical College Hospital from January 2012 to December 2020. Clinical data included demographics, predisposing factors, manifestations, laboratory/imaging findings, treatments, and outcomes. Patients were stratified into three groups based on underlying AIDs: adult-onset Still′s disease with HPS (AOSD-HPS) group, systemic lupus erythematosus with HPS (SLE-HPS) group, and other AID with HPS (other AID-HPS) group. Comparative analyses were conducted to identify intergroup differences. Continuous variables were analyzed using one-way ANOVA, Welch′s test, or Kruskal-Wallis′s test based on data distribution and homogeneity of variance. Categorical variables (rates and proportions) were compared with the chi-square test or Fisher-Freeman-Halton exact test according to expected cell frequencies.Results:Among 85 patients, 67 were female. Underlying AIDs included AOSD (32 cases, 37.6%), SLE (32 cases, 37.6%), and other AIDs (21 cases, 24.7%). Infections (≥1 type) were identified in 54 patients (63.5%), predominantly viral (48 cases, 56.5%), including cytomegalovirus (CMV) (40 cases), Epstein-Barr virus (EBV) (11 cases), and 6 cases were coinfected with both CMV and EBV. All patients presented with fever; hepatomegaly, splenomegaly, and lymphadenopathy were observed in 39 (45.9%), 59 (69.4%), and 70 (82.4%) cases, respectively. Additional manifestations included arthralgia (63 cases, 74.1%) and rash (65 cases, 76.5%). Pancytopenia in 31 cases (36.5%) and bicytopenia in 29 cases (34.1%). Liver dysfunction was noted in 79 cases (92.9%). Elevated lactate dehydrogenase (LDH) (83 cases, 97.6%), elevated triglyceride (TG) (76 cases, 89.4%), decreased fibrinogen (Fbg) (55 cases, 64.7%), and elevated serum ferritin (SF) (84 cases, 98.8%) were common. Elevated soluble CD25(sCD25) (53cases) and reduced NK cell activity (49 cases) were observed. Bone marrow examination revealed hemophagocytosis in 49 cases. There were statistically significant differences in age( F=3.763, P=0.031), lymphadenopathy( χ2=7.098, P=0.029), rash( χ2=12.816, P=0.002), reductions in WBC( H=22.284, P<0.001)、NEU( H=18.882, P<0.001)、PLT( H=15.127, P=0.001), and elevations in LDH( H=7.842; P=0.020)、TG( H=6.177, P=0.046)、CRP( H=6.915, P=0.032)、SF( H=9.661, P=0.008)、sCD25( χ2=5.154, P=0.035) among the three groups: (1) The SLE-HPS group was significantly younger [(28.1 ± 10.4) years) than the other AID-HPS group [(39.5 ± 17.3) years, P=0.028]. (2) The AOSD-HPS group had higher incidence rates of lymphadenopathy (93.8%) and rash (93.8%) than the SLE-HPS group (68.8% and 56.3%, respectively), (lymphadenopathy: χ2=7.098, P=0.029; rash: χ2=12.816, P=0.002). (3) ① WBC in the SLE-HPS group [1.62 (1.18, 2.92) ×10 9/L] were significantly lower than those in the AOSD-HPS group [5.66 (2.75, 11.57)×10 9/L] and the other AID-HPS group [6.05 (2.49, 14.55)×10 9/L] ( Z=-4.032, P<0.001; Z=3.993, P<0.001). ② NEU in the SLE-HPS group [1.10 (0.60, 1.93)×10 9/L] were markedly reduced compared to the AOSD-HPS group [3.73 (1.54, 9.04)×10 9/L] and the other AID-HPS group [2.23 (1.43, 11.57)×10 9/L] ( Z=-3.859, P<0.001; Z=3.506, P=0.001). ③ PLT in the SLE-HPS group [59.50 (28.50, 81.00)×10 9/L] were significantly lower than those in the AOSD-HPS group [109.00 (65.75, 232.00)×10 9/L] and the other AID-HPS group [150.00 (55.00, 221.00)×10 9/L] ( Z=-3.421, P=0.002; Z=3.179, P=0.004). (4) LDH levels in the AOSD-HPS group [1 178 (645, 1 875) U/L] were significantly higher than those in the other AID-HPS group [598 (410, 771) U/L] ( Z=2.795, P=0.016). (5) TG levels in the SLE-HPS group [3.61 (2.46, 6.09) mmol/L] were significantly higher than those in the other AID-HPS group [2.68 (1.71, 3.30)mmol/L] ( Z=2.402, P=0.049). (6) CRP and SF levels in the AOSD-HPS group [79.20 (28.02, 179.53)mg/L and 30 225 (13 494, 53 598)μg/L, respectively] were significantly higher than those in the SLE-HPS group [26.05 (9.41, 83.31)mg/L and 9 862 (4 467, 22 315) μg/L, respectively] ( Z=2.547, P=0.033; Z=3.069, P=0.006 ). (7) The incidence rates with elevated sCD25 in the AOSD-HPS group (100.0%) was significantly higher than that in the other AID-HPS group (76.9%), ( χ2=5.154, P=0.035). After treatment, 83 patients improved, while 2 deaths occurred in the other AID-HPS group. Conclusion:Adult AID-HPS predominantly affects young to middle-aged females, with SLE and AOSD being the most common underlying AIDs. The condition manifests with severe clinical features, frequently triggered by viral infections (particularly CMV and EBV). Distinct differences in clinical and laboratory profiles exist among AID-HPS subtypes. Early recognition and aggressive treatment are critical for improving prognosis.

Objective:To investigate the clinical characteristics of adult patients with autoimmune disease-associated hemophagocytic syndrome (AID-HPS) and enhance clinical recognition of this condition.Methods:A retrospective analysis was performed on 85 adult AID-HPS patients admitted to the department of rheumatology and immunology at Peking Union Medical College Hospital from January 2012 to December 2020. Clinical data included demographics, predisposing factors, manifestations, laboratory/imaging findings, treatments, and outcomes. Patients were stratified into three groups based on underlying AIDs: adult-onset Still′s disease with HPS (AOSD-HPS) group, systemic lupus erythematosus with HPS (SLE-HPS) group, and other AID with HPS (other AID-HPS) group. Comparative analyses were conducted to identify intergroup differences. Continuous variables were analyzed using one-way ANOVA, Welch′s test, or Kruskal-Wallis′s test based on data distribution and homogeneity of variance. Categorical variables (rates and proportions) were compared with the chi-square test or Fisher-Freeman-Halton exact test according to expected cell frequencies.Results:Among 85 patients, 67 were female. Underlying AIDs included AOSD (32 cases, 37.6%), SLE (32 cases, 37.6%), and other AIDs (21 cases, 24.7%). Infections (≥1 type) were identified in 54 patients (63.5%), predominantly viral (48 cases, 56.5%), including cytomegalovirus (CMV) (40 cases), Epstein-Barr virus (EBV) (11 cases), and 6 cases were coinfected with both CMV and EBV. All patients presented with fever; hepatomegaly, splenomegaly, and lymphadenopathy were observed in 39 (45.9%), 59 (69.4%), and 70 (82.4%) cases, respectively. Additional manifestations included arthralgia (63 cases, 74.1%) and rash (65 cases, 76.5%). Pancytopenia in 31 cases (36.5%) and bicytopenia in 29 cases (34.1%). Liver dysfunction was noted in 79 cases (92.9%). Elevated lactate dehydrogenase (LDH) (83 cases, 97.6%), elevated triglyceride (TG) (76 cases, 89.4%), decreased fibrinogen (Fbg) (55 cases, 64.7%), and elevated serum ferritin (SF) (84 cases, 98.8%) were common. Elevated soluble CD25(sCD25) (53cases) and reduced NK cell activity (49 cases) were observed. Bone marrow examination revealed hemophagocytosis in 49 cases. There were statistically significant differences in age( F=3.763, P=0.031), lymphadenopathy( χ2=7.098, P=0.029), rash( χ2=12.816, P=0.002), reductions in WBC( H=22.284, P<0.001)、NEU( H=18.882, P<0.001)、PLT( H=15.127, P=0.001), and elevations in LDH( H=7.842; P=0.020)、TG( H=6.177, P=0.046)、CRP( H=6.915, P=0.032)、SF( H=9.661, P=0.008)、sCD25( χ2=5.154, P=0.035) among the three groups: (1) The SLE-HPS group was significantly younger [(28.1 ± 10.4) years) than the other AID-HPS group [(39.5 ± 17.3) years, P=0.028]. (2) The AOSD-HPS group had higher incidence rates of lymphadenopathy (93.8%) and rash (93.8%) than the SLE-HPS group (68.8% and 56.3%, respectively), (lymphadenopathy: χ2=7.098, P=0.029; rash: χ2=12.816, P=0.002). (3) ① WBC in the SLE-HPS group [1.62 (1.18, 2.92) ×10 9/L] were significantly lower than those in the AOSD-HPS group [5.66 (2.75, 11.57)×10 9/L] and the other AID-HPS group [6.05 (2.49, 14.55)×10 9/L] ( Z=-4.032, P<0.001; Z=3.993, P<0.001). ② NEU in the SLE-HPS group [1.10 (0.60, 1.93)×10 9/L] were markedly reduced compared to the AOSD-HPS group [3.73 (1.54, 9.04)×10 9/L] and the other AID-HPS group [2.23 (1.43, 11.57)×10 9/L] ( Z=-3.859, P<0.001; Z=3.506, P=0.001). ③ PLT in the SLE-HPS group [59.50 (28.50, 81.00)×10 9/L] were significantly lower than those in the AOSD-HPS group [109.00 (65.75, 232.00)×10 9/L] and the other AID-HPS group [150.00 (55.00, 221.00)×10 9/L] ( Z=-3.421, P=0.002; Z=3.179, P=0.004). (4) LDH levels in the AOSD-HPS group [1 178 (645, 1 875) U/L] were significantly higher than those in the other AID-HPS group [598 (410, 771) U/L] ( Z=2.795, P=0.016). (5) TG levels in the SLE-HPS group [3.61 (2.46, 6.09) mmol/L] were significantly higher than those in the other AID-HPS group [2.68 (1.71, 3.30)mmol/L] ( Z=2.402, P=0.049). (6) CRP and SF levels in the AOSD-HPS group [79.20 (28.02, 179.53)mg/L and 30 225 (13 494, 53 598)μg/L, respectively] were significantly higher than those in the SLE-HPS group [26.05 (9.41, 83.31)mg/L and 9 862 (4 467, 22 315) μg/L, respectively] ( Z=2.547, P=0.033; Z=3.069, P=0.006 ). (7) The incidence rates with elevated sCD25 in the AOSD-HPS group (100.0%) was significantly higher than that in the other AID-HPS group (76.9%), ( χ2=5.154, P=0.035). After treatment, 83 patients improved, while 2 deaths occurred in the other AID-HPS group. Conclusion:Adult AID-HPS predominantly affects young to middle-aged females, with SLE and AOSD being the most common underlying AIDs. The condition manifests with severe clinical features, frequently triggered by viral infections (particularly CMV and EBV). Distinct differences in clinical and laboratory profiles exist among AID-HPS subtypes. Early recognition and aggressive treatment are critical for improving prognosis.

Objective: To analyze the correlation between the Spondyloarthritis Research Consortium of Canada (SPARCC) magnetic resonance imaging (MRI) sacroiliac joint inflammation score (SPARCC score)/structural score (SSS) and the disease activity as well as the functional indexs. The correlation between the MRI score and inflammatory indicators [erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)] in patients with active axial spondyloarthritis (axSpA) before and after treatment was explored. In addition, the contribution of the two MRI scoring method in evaluating conditions was also explored. Methods: According to the inclusion criteria, 24 patients with active axial SpA were recruited and received the recombinant hauman tumor necrosis factor (TNF)-α receptor Ⅱ: IgG Fc fusion protein(rhTNFR:Fc), sulfasalazine and thalidomide for 12 weeks. Subjects were scored at week 0 and 12 by SPARCC/SSS scores. Bath ankylosing spondylitis disease activity index (BASDAI), Assessment of Spondyloarthritis Intemational Society (ASAS)-endorsed disease activity score(ASDAS)-CRP, bath ankylosing spondylitis functional index (BASFI). Bath ankylosing spondylitis metrology index(BASMI), ESR and CRP. The correlation between the SPARCC/SSS scores and that of clinical indicators were analyzed. Paired sample t test, Wilcoxon signedrank test, Spearman correlation analysis and Pearson correlation analysis were used for statistical analysis, and receiver operating characteristic curve (ROC) was used to evaluate the effectiveness of SPARCC score decline as a response to treatment. Results: ① Compared with baseline, after 12 weeks treatment, SPARCC scores [(15±4) and(33±10)], BASDAI [(3.2±0.9) and (5.2±1.1)], BASFI [(2.3±0.6) and (4.6±1.0)], BASMI [(2.3±0.7) and (4.1±1.1)], ASDAS-CRP scores [(2.0±0.8) and (3.7±0.9)], ESR [(16±12) mm/1 h and (49±26) mm/1 h], CRP [(7.2±2.8) mg/L and (30.4±19.3) mg/L] were significantly decreased (t values were 7.822, 6.950, 10.707, 7.204, 6.281,-4.015 and-4.257, respectively), and the differences were statistically significant (P=0.000). There was no significant difference in SSS scores between baseline [(20±6) and (19±7)] and after 12 weeks treatment (t=-0.761, P=0.455). ② Before treatment, SPARCC score showed positive linear correlation with BASDAI (r=0.630, P=0.001), ASDAS-CRP (r=0.646, P=0.001), CRP (r=0.574, P=0.003) and ESR (r=0.559, P=0.004), and the correlation of the above indexes disappeared after treatment (P>0.05). The association between SPARCC structral scores and the above indicators was not significant before and after treatment. ③ Areas under curve(AUC) of ROC for assessing treatment response by reduced SPARCC scores was 0.809. The cut-off value for response to treatment was 20.5, with the sensitivity of 68.8% and specificity of 75.0%. Conclusion The SPARCC MRI SIJ inflamm-ation score has certain value in evaluating disease activity and efficacy, while the SPARCC SSS is not.

OBJECTIVE: To explore a simple and accurate method for localization of upper airway obstruction in patients with obstructive sleep apnea-hypopnea syndrome (OSAHS) and provide instructions for surgical treatment. METHOD: Fifty OSAHS patients confirmed by PSG underwent acoustic rhinometric and pharyngometric assessment by Eccovision. The parameters were recorded, including nasal minimal cross-sectional area (NMCA), distance of MCA from the nostril (DCAN), minimum cross-sectional area at the nasal valve(MCA), nasal resistance (NR) and nasal volume from 0 to 6 cm from the nostril (NCV), as well as pharyngeal cross-sectional area (CSA) and volume from 4.8 to 15.0 cm. The sensitivity and specificity of acoustic rhinometry and pharyngometry on localization of airway obstruction was determined by a comprehensive imaging and endoscopic study. RESULT: In 50 cases with severe OSAHS, NMCA, DCAN, MCA, NCV, NR were (0.61 +/- 0.35) cm2, (2.06 +/- 0.12) cm, (0.87 +/- 0.12) cm2, (9.24 +/- 2.31)cm3 and (0.51 +/- 0.32)kPa/(L x min), respectively. Pharyngeal CSA and volume were statistically significantly lower than that in control group (P < 0.01). The value of DCAN was (2.06 +/- 0.12) cm, (9.50 +/- 4.08) cm, (13.10 +/- 2.52) cm in type I II, III patient, respectively. Compared with the control group, the difference was statistically significant. CONCLUSION Acoustic rhinometry and pharyngometry is a simple and safe method in localization of airway obstruction in patients with OSAHS.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; physiopathology ; Nasopharynx ; physiopathology ; Rhinometry, Acoustic ; Sleep Apnea, Obstructive ; physiopathology ; Young Adult

Objective To explore the anatomy for transethmoidal-sphenoid optic nerve decompression under endoscopy and its significance in operation. Methods Fifteen cases (30 sides) of formalin-fixed adult optic canal specimens were dissected under the microscope. The anatomic characteristics of the optic canal and its adjacent were observed, and the relative parameters were evaluated according to nasal endoscopic approach. Results ①The relationship between the optic carotid triangle(OCT)with the optic canal, the ophthalmic artery, the cavernous sinus and the internal carotid artery were invariable, its present ratio were in 66.7%. ②The mean distance from the front margin of nasal columella floor to medial wall of the orbital opening, middle portion and the cranial opening in the optic canal were (72.79 ± 5.40)mm, (75.85 ± 5.10)mm and (79.34 ± 4.95)mm, respectively, and the elevation angles were (39.45 ± 3.68)°, (37.30±4.24)°and (35.45 ± 4.16)°, respectively. ③The mean thickness of sheath in the medial wall of the orbital opening,middle portion and the cranial opening were (0.70 ± 0. 18)mm, (0.51 ± 0.15)mm and (0.49-0.22)mm,respectively. The difference in thickness between the orbital opening and middle portion, the cranial opening were very remarkable(P ＜ 0.01 ). ④The lateral deviate distance from medial wall of the orbital opening, middle portion and cranial opening to sagittal median plane of cadaveric were 1/2 (12.69 ± 2.73)mm、1/2( 19.61± 3.47)mm and 1/2 (25.79 ± 3.23)mm, respectively. Conclusion OCT is the most reliable anatomic landmark to locate the optic canal, and the key point is at the orbital opening of the optic nerve in the optic nerve decompression. It is secure and feasible to cut the sheath from the place where the medial wall crosses the superior wall of the optic nerve.

OBJECTIVE: To provide transnasal endoscopic optic canal decompression with the anatomic reference. METHOD: 15 samples of the adult corpse wet specimen (30 sides for the optic canal) were examined under the endoscope to scrutinize the regional anatomy of the optic canal. RESULT: distance between the spina nasalis anterior and the midpoint of optic canal medial wall is (61.02 +/- 5.83) mm, and the angle between spina nasalis anterior and the midpoint of optic canal medial wall is (45.1 +/- 4.81) degrees. The medial wall of optic canal is longest, with an average length of (11.61 +/- 1.58) mm; the lateral wall of optic canal is thickest, and the medial wall thinnest. 10 traumatic blind patient underwent endoscopic optic canal decompression with satisfactory outcome. CONCLUSION The regional anatomy of the optic canal under endoscope is of importance to endoscopic optic canal decompression. Which is microinvasive with direct approach and clear view thus is widely used in clinical practice.
Adolescent ; Adult ; Blindness ; surgery ; Child ; Endoscopy ; Female ; Humans ; Male ; Middle Aged ; Nose ; anatomy & histology ; surgery ; Optic Nerve ; anatomy & histology ; surgery ; Orbit ; anatomy & histology ; surgery ; Young Adult