类风湿关节炎继发不同类型肺间质病变临床分析

目的比较类风湿关节炎(RA)继发不同类型肺间质病变(ILD)患者的临床、肺部影像以及肺功能特点。方法选择2007年1月至2013年4月北京大学第一医院风湿免疫科或呼吸科住院经高分辨CT(HRCT)确诊、资料完整的RA-ILD的患者51例,收集临床、血气分析、RA血清学及肺功能等指标,比较不同肺HRCT分类患者临床特点的差异。结果肺HRCT下寻常型间质性肺炎(UIP)改变13例,非特异性间质性肺炎(NSIP)改变10例,机化性肺炎(OP)改变8例,无法分类20例,各组在性别、年龄、吸烟史、血清学指标方面差异无统计学意义;12例患者在确诊RA前出现ILD,其余39例患者ILD出现在RA诊断后或与RA同时出现,RA诊断后继发NSIP的中位时间间隔为1.45年(IQR 4.37,范围0~9.75年),早于其他类型ILD患者(P0.05),OP患者的Pa O2[(7.65±1.38)k Pa]低于其他类型ILD患者[UIP(10.23±1.58)mm Hg,NSIP(9.56±3.05)mm Hg,无法分类(10.41±2.39)mm Hg,P0.05]。不同类型ILD患者的肺功能水平比较差异无统计学意义。Logistic回归分析发现,ILD病程(OR=1.451,95%CI1.032~2.040,P=0.032)、NSIP(OR=8.326,95%CI 1.243~55.778,P=0.029)以及OP(OR=17.431,95%CI 1.901~159.811,P=0.011)是RA-ILD出现Ⅰ型呼吸衰竭的独立危险因素。结论 UIP是RA-ILD患者经肺HRCT可以明确分型间质病中最常见的类型,部分患者在RA发病后短期内出现ILD、NSIP、OP以及ILD病程是出现Ⅰ型呼吸衰竭的危险因素,因此,RA患者应积极筛查ILD。     

皮肌炎多发性肌炎患者间质性肺病病理资料推断性分析

目的 分析皮肌炎(DM)、多发件肌炎(PM)合并间质性肺病(ILD)患者的胸部X线、胸部高分辨CT(HRCT)及肺功能等肺部表现临床特点及其预后.方法 回顾性分析上海长海医院风湿免疫科1999年1月至2007年1月期间收住院的33例DM/PM合并ILD除外感染患者的临床资料.结果 33例ILD患者经胸部X线及HRCT证实.根据临床-影像学特点并结合血气分析、肺功能测定,参照美国胸科学会和欧洲呼吸协会问质性肺炎的分类诊断标准,推断分析病理类型主要为非特异性间质性肺炎(NSIP)(57%)和寻常性间质性肺炎(UIP)(25%). UIP类型预后差、病死率高(70%).结论 结合患者的影像学资料、肺功能、血气分析推断病理类型主要为NSIP和UIP,其中UIP病死率高、预后差.     

ILD为首发症的抗Ro-52抗体阳性抗合成酶综合征延迟诊断

目的 分析以间质性肺疾病(ILD)为首发症的抗Ro-52抗体阳性的抗合成酶综合征(ASS)患者的诊断,提高对该疾病的认识以做到早诊断、早治疗。方法 回顾2018～2022年7例以ILD为首发症的抗Ro-52抗体阳性ASS患者延误诊断临床资料,对照相关文献探讨抗Ro-52抗体阳性ASS和ASS临床特征,判断延误诊断的可能原因。结果 7例患者中女6例、男1例,诊断年龄55～74岁,平均(65.29±7.41)岁。发病至确诊ASS间隔2.42～16.00年,其中6.00年及以内6例,平均(4.27±2.84)年;全部诊断为抗Ro-52抗体阳性的ASS,抗Ro-52抗体和抗ARS抗体同时检测为阳性,其中抗Jo-1抗体阳性6例,抗PL-7阳性1例。确诊为ASS时双手关节受累5例,技工手3例,肌酸、肌痛、肌无力3例,雷诺现象3例,患者均有肺间质改变,表现为非特异性间质性肺炎(NSIP)型。肝酶、肌酶、C反应蛋白(CRP)、血气、铁蛋白、超敏肌钙蛋白、D-二聚体等多项指标出现异常。7例首诊为ILD后均使用了糖皮质激素对症治疗。确诊抗Ro-52抗体阳性ASS后2例使用利妥昔单抗,病情稳定。结论 在抗R...     

胸部高分辨率CT新分型对特发性肺纤维化早期诊断的影响

胸部高分辨率CT (HRCT) 在特发性肺纤维化(IPF) 的诊断中起核心作用。根据现有指南和专家共识,临床 怀疑IPF 患者的HRCT 表现可分为肯定寻常型间质性肺炎(UIP)、可能UIP 和不符合UIP 三个基本类型,而对一些 不典型病例可考虑为不确定UIP 型。当患者具有典型的IPF 临床表现且HRCT 表现为肯定UIP 型或可能UIP 型, 可作出有信心的IPF 的诊断。表现为不符合UIP 型的患者多考虑为非IPF,针对不确定型UIP 可采用积极活检获取 病理诊断或密切观察。HRCT 这种分型方法便于临床上分层管理,将有助于IPF 早期诊断 。     

血清抗中性粒细胞胞质抗体阳性肺间质病患者临床及预后分析

目的探究血清抗中性粒细胞胞质抗体(ANCA)阳性的间质性肺疾病患者的临床特点及预后。方法回顾性纳入2006年3月至2016年3月就诊于北京协和医院的间质性肺疾病患者274例,其中男81例,女193例,年龄(53±11)岁,根据诊断分为血清ANCA阳性肺间质病(ANCA-ILD)、结缔组织病相关性肺间质病和自身免疫特征的间质性肺炎(CTD-ILD/IPAF)及特发性间质性肺炎(IIP)组,分析3组患者在临床表现、血清学、肺功能、影像学、生存及复发方面的差异。结果274例患者中ANCA阳性38例(38/274,14%),发病年龄(59±10)岁,随访时间(52±31)个月,死亡7例(7/38,18%)。ANCA阳性患者发病年龄高于CTD-ILD/IPAF[(52±10)岁]和IIP[(53±11)岁,H=19.29,P<0.001],血红蛋白[(129±21)mg/L]低于CTD-ILD/IPAF[(138±15)mg/L]和IIP[(140±19)mg/L,H=8.17,P=0.017],ESR[(46±35)mm/1 h]高于CTD-ILD/IPAF[(26±24)mm/1 h]和IIP[(19±22)mm/1 h,H=19.73,P<0.001],治疗后肺功能FVC改善率(31%)低于CTD-ILD/IPAF(59%)和IIP(39%,χ^2=11.74,P=0.003),胸部CT病变吸收率(61%)低于CTD-ILD/IPAF(82%)和IIP(67%,χ^2=9.23,P=0.010),病死率(18%)高于CTD-ILD/IPAF(6%)和IIP(12%,χ^2=7.16,P=0.028)。结论ANCA阳性患者与其他类型肺间质病患者在临床特征方面存在差异,治疗效果不佳,预后较差。     

抗Ro52抗体与Ⅰ型自身免疫性肝炎患者病情的相关性研究

[目的]探究抗Ro52抗体与I型自身免疫性肝炎患者病情的相关性。[方法]选取68例于2014年8月之2016年5月入我院诊治的Ⅰ型自身免疫性肝炎(autoimmune hepatitis,AIH)患者,运用免疫印迹法检测患者血清抗Ro52抗体,依据检测结果,将所有患者分为抗Ro52抗体阳性组(26例)和抗Ro52抗体阴性组(42例),收集2组患者的抗核抗体(ANA)滴度、抗可溶性肝原(抗SLA/LP)抗体、抗Ro60抗体、肝病相关并发症、肝外自身免疫性疾病、免疫球蛋白等资料,并进行对比。[结果]2组AIH患者的ANA抗体滴度主要分布于1:320~1:1 000之间;在抗Ro52抗体阳性组中,有7例合并有抗SLA/LP抗体阳性、有16例合并有抗Ro60抗体阳性;在肝病相关并发症方面,抗Ro52抗体阳性组发生率要高于阴性组,但差异无统计学意义(χ~2=0.11,P=0.75);在肝外自身免疫性疾病方面,抗Ro52抗体阳性组发生率显著高于阴性组,差异具有统计学意义(χ~2=5.13,P=0.02);在免疫球蛋白水平方面,抗Ro52抗体阳性组IgG水平显著高于阴性组,差异具有统计学意义(t=3.67,P0.01)。[结论]抗Ro52抗体阳性的Ⅰ型AIH患者合并有肝病相关并发症和肝外自身免疫性疾病的可能性较高,Ⅰ型AIH的发病可能与抗Ro52抗体和IgG共同作用有关。     

电视辅助胸腔镜肺手术对特发性间质性肺炎的诊断价值

目的探讨电视辅助胸腔镜肺手术(VATS)对特发性间质性肺炎病理诊断的价值。方法我院收治的间质性肺病(ILD)患者353例,经VATS取得53例特发性间质性肺炎(IIP)的肺组织病理标本,进行病理诊断分类血清、血液动力学、影像学和肺功能检查,回顾性分析IIP患者的治疗、活检情况,术后并发症、肺组织取材和手术住院的费用。结果353例患者中进行VATS比例为18.1%(53/353)。53例中特发性非特异性间质性肺炎(NSIP)23例,普通性间质性肺炎(UIP)9例,闭塞性细支气管炎伴机化性肺炎(BOOP)5例,呼吸性细支气管炎间质性肺炎(RBILD)3例,淋巴细胞间质性肺炎(LIP) 特发性脱屑型间质性肺炎(DIP)2例,UIP NSIP1例,NSIP RBILD1例,LIP1例,不能分类的IIP8例。术前平均PaCO235.3mmHg,PaO275.4mmHg。胸部CT检查中毛玻璃样见于各种类型的ILD,蜂窝样主要见于UIP和NSIP。手术后未见并发症。手术住院费用平均24253元。影响手术病理结果的因素有病灶的取材部位和数量。22例术前送检风湿科作相关检查,有4例不同指标低滴度的阳性。结论VATS具有安全、损伤小、病理诊断率高的特点。多叶取肺组织标本可以提高VATS对IIP的诊断率。     

特发性间质性肺炎小气道功能异常研究

目的探讨特发性间质性肺炎(idiopathic interstitial pneumonia,IIPs)患者的小气道功能改变。方法收集1999年1月至2007年2月北京朝阳医院住院确诊为IIPs的患者159例。特发性肺纤维化(IPF)患者113例,非特异性间质性肺炎(NSIP)患者23例,隐源性机化性肺炎(COP)患者23例。检测所有患者肺通气、肺换气以及小气道功能。结果 IIPs患者小气道功能指标明显降低,25%和50%肺活量时的最大呼气流速(V25%,V50%)均预计值的80%。IPF、NSIP、COP患者小气道功能障碍的发生率分别为51.32%、73.91%和69.56%,其中NSIP与IPF比较,差异有统计学意义(P0.05)。结论 IIPs患者不仅存在限制性通气功能和弥散功能障碍,也存在小气道功能障碍,不同类型IIPs小气道功能障碍发生率不同,NSIP发生率较IPF高;小气道功能测定可能会增加对IIPs患者生理功能异常的理解。     

丙型肝炎病毒基因型与丙型肝炎患者自身抗体的相关性

目的探讨湖州地区丙型肝炎病毒(hepatitis C virus,HCV)感染患者HCV基因型与自身抗体变化的关系,同时分析HCV含量与抗核抗体(anti-nuclear antibody,ANA)检出率的关系。方法收集湖州市中心医院2008年1月至2010年1月传染科门诊和住院HCV RNA阳性患者血清,采用DNA测序法检测HCV基因型,间接免疫荧光法检测ANA和抗平滑肌抗体,斑点免疫印迹法检测抗线粒体抗体M2、Sm、SSA、RO52、SSB及抗着丝点抗体,散射比浊法检测类风湿因子。血清样本按照HCV RNA病毒载量分为高、中、低载量3组。结果共纳入94例HCV患者,其中1a型5例(5.3%),1b型77例(81.9%),2a型9例(9.6%),3a型1例(1.1%),6a型2例(2.1%)。94例HCV患者自身抗体阳性检出率39.4%,其中1b型检出率46.8%,2a型检出率11.1%,1b型与2a型检出率比较差异有统计学意义(P0.05),其他基因型未检出自身抗体阳性。1b型77例患者可检出ANA、类风湿因子、抗平滑肌抗体、M2、SSA、RO52、SSB、抗着丝点抗体8种自身抗体,2a型9例患者检出ANA、类风湿因子和M23种自身抗体。HCV患者ANA以低滴度颗粒型为主,ANA 1∶100滴度占59.5%(2237),颗粒型占ANA的35.1%(1337),其次是着丝点型。3个载量组分别与对照组比较,差异均有统计学意义(χ2分别为6.4、19.9、53.1,均P0.05)。高载量组和低、中载量组比较,差异均有统计学意义(χ2分别为19.5、9.8,均P0.05)。结论湖州地区HCV基因型以1b型为主,HCV不同基因型与自身抗体之间存在密切的关系,其载量高低与自身抗体检出率成正比。     

普通型间质性肺炎和非特异性间质性肺炎肺组织中不同细胞因子的表达及其意义

目的　研究转化生长因子 (TGF) β1、碱性成纤维细胞生长因子 (b FGF)、白细胞介素 8(IL 8)、白细胞介素 13(IL 13)、γ干扰素 (IFN γ)在普通型间质性肺炎 (UIP)和非特异性间质性肺炎(NSIP)肺组织中的分布、表达及意义。方法　经胸腔镜或开胸肺活检获取 5例UIP和 8例NSIP患者的肺组织。对照组 5例 ,来自手术切除的远离肺癌原发灶的周边肺组织。用免疫组化法半定量分析细胞因子的分布及表达。结果　TGF β1、IL 8、b FGF主要分布在肺泡上皮细胞、肺泡巨噬细胞、细支气管上皮细胞 ,UIP组表达强于NSIP组和对照组。IL 13主要分布在肺泡上皮细胞、肺泡巨噬细胞、间质单个核细胞 ,UIP、NSIP组表达无明显差异 ,但均强于对照组。IFN γ主要分布在间质单个核细胞 ,NSIP组表达强于UIP组和对照组。UIP组的IL 13/IFN γ比值为 (2 18± 0 76 ) ,NSIP组为(0 95± 0 2 8) ,对照组为 (0 91± 0 16 ) ,3组比较差异均有显著性 (P值均 <0 0 5 ) ,而NSIP组与对照组比较差异无显著性。对照组只有肺泡巨噬细胞表达上述各细胞因子。结论　TGF β1、IL 8、b FGF在UIP和NSIP患者肺组织中表达强度的不同和IL 13/IFN γ的是否平衡可能参与了UIP和NSIP不同的发病过程。     

Clinical features,risk factors,and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study

To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint. Patients with IPAF were much common in young female and had lower percentage of ever smoking and a significantly shorter survival than those with non-IPAF (P?<?0.001). Subgroup analysis revealed that IPAF cohort survival was worse than that in non-IPF (P?<?0.001), but better than that in IPF (P?<?0.001). In IPAF cohort, the most common systemic symptom and serological abnormality were Raynaud’s phenomenon (12.9%) and ANA ≥?1:320 (49.2%); the most frequent high-resolution computed tomography (HRCT) pattern was nonspecific interstitial pneumonia (NSIP) (61.6%). Multivariate analysis indicated that several factors including age, smoking history, organizing pneumonia (OP) pattern in HRCT, and anti-RNP positivity were independently associated with significantly worse survival. IPAF had the distinct clinical features and outcomes compared with other groups of ILD. Additional studies should be needed to explore the underlying autoimmune mechanism and to determine risk stratification in future clinical research.     

A retrospective analysis of distinguishing features of chest HRCT and clinical manifestation in primary Sjögren’s syndrome-related interstitial lung disease in a Chinese population

To characterize the distinctive chest high-resolution computerized tomography (HRCT) features and clinical manifestations of primary Sjögren syndrome (pSS)-related interstitial lung disease (ILD). The demographic data, clinical manifestations, and laboratory and radiological findings of 527 pSS patients were retrospectively analyzed. ILD was defined based on the presences of pulmonary signs in HRCT. Two hundred six of 527 patients were diagnosed as pSS-ILD, and the prevalence was 39.1%. The three most frequent abnormalities in HRCT were reticular pattern (92.7%), ground-glass attenuation (87.4%), and bronchovascular bundle thickening (82%). One hundred twenty-four cases (60.2%) of the pSS-ILD patients had only a single HRCT pattern, which involved 86 non-specific interstitial pneumonitis (NSIP) cases (41.7%), 22 usual interstitial pneumonia (UIP) cases (10.68%), 8 organizing pneumonia (OP) cases (3.9%), and 8 lymphocytic interstitial pneumonia (LIP) cases (3.9%), respectively. Besides, the more important observation was that 82 cases had no less than two HRCT patterns, and NSIP admixed with OP (43.9%), NSIP admixed with UIP (35.4%), and NSIP admixed with LIP (19.5%) were the most frequent. HRCT of pSS-ILD patients demonstrated bilateral infiltrates (99%), with abnormalities predominantly in the lower lobes (89.3%) and subpleural areas (81.1%), and a few lesions were characterized by hilum distributed (8.7%). Pulmonary function tests (PFTs) revealed impaired diffusion capacity for carbon monoxide and total lung capacity, and the rate of small airway lesions in the pSS-ILD patients was 3.5 times higher in patients of pSS. Logistic regression analysis showed that dry cough (OR 59.05), clubbing (OR 6.26), elevated lactate dehydrogenase (OR 21.38) and positive anti-Ro (OR 7.86) were relevant factors of pSS-ILD. ILD is the common pulmonary involvement of pSS and the prevalence of pSS-ILD is 39.1%. The single pattern of NSIP and UIP in HRCT are the commonest, and about 40% of the pSS-ILD patients possess multiple patterns in HRCT. The classification of idiopathic pulmonary fibrosis cannot completely include the pulmonary imaging features of pSS-ILD.     

Bronchoalveolar lavage in fibrotic idiopathic interstitial pneumonias

The purpose of this study was to assess the role of bronchoalveolar lavage (BAL) in differentiating usual interstitial pneumonia (UIP) from non-specific interstitial pneumonia (NSIP) and in predicting the prognosis in fibrotic idiopathic interstitial pneumonia (IIP). A retrospective review of 122 patients (age 58+/-8 years, 70 male) with UIP (n=87) and NSIP (n=35) was carried out. Prior to surgical lung biopsy, all of them underwent BAL and high-resolution-computed tomography (HRCT) of the chest. Neutrophil count in BAL fluid was higher in UIP (7.0%) than NSIP (3.0%) (P=0.027). In contrast, BAL lymphocyte count was significantly higher in NSIP (29.0%) than UIP (5.5%) (P<0.0001). In 62 patients whose HRCT findings were atypical for UIP, BAL lymphocytosis was more frequently observed in NSIP (20/33) than UIP (4/29) (P<0.001) and the absence of BAL lymphocytosis suggested a diagnosis of UIP rather than NSIP (odds ratio 12.7, P<0.001). Pathologic diagnosis of NSIP was the only independent factor predicting a longer survival of our patients (median follow-up 21 months) (hazard ratio (HR) 0.035, P=0.005). When NSIP was not included in the survival analysis, higher BAL lymphocyte count was the only independent predictor of a longer survival (HR 0.909, P=0.029). BAL is an useful non-invasive tool in fibrotic IIP, not only for excluding a variety of specific non-IIP diseases but also for narrowing the differential diagnosis and predicting the prognosis in the absence of the histopathologic diagnosis.     

Overlap of interstitial pneumonia with autoimmune features with undifferentiated connective tissue disease and contribution of UIP to mortality

Background and objective

Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome.

Methods

Patients with prior UCTD‐interstitial lung disease (ILD) were screened by ERS/ATS criteria for IPAF. Clinical data along with all‐cause mortality were collated and compared with selected idiopathic pulmonary fibrosis (IPF) patients from the same study period. Survival was compared between IPAF subgroups with and without UIP features.

Results

One hundred and one UCTD‐ILD subjects (91%) evaluated from 2005 to 2012 also met strict criteria for IPAF. Frequent clinical findings included Raynaud’s phenomenon, positive anti‐nuclear antibody (ANA) and non‐specific interstitial pneumonia (NSIP) pattern on chest computed tomography (CT). Nineteen had features of UIP either on histopathology or CT imaging. As compared with IPF, IPAF patients had overall better survival except in those with UIP features.

Conclusion

Current IPAF criteria encompassed the majority of broadly defined UCTD‐ILD and included those with UIP findings. Survival compared with IPF in those with UIP was similar. Further studies are necessary to refine IPAF definitions for clinical use and guide directed management strategies.

     

Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis

Background

Nonspecific interstitial pneumonia (NSIP) has recently been proposed as a histologic type of idiopathic interstitial pneumonia (IIP), but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. It is particularly unclear how NSIP and usual interstitial pneumonia (UIP) are related. The present study investigated the clinicopathologic features and prognosis of NSIP, and its differential diagnosis from UIP.

Methods

The clinicopathologic findings and prognosis in 21 NSIP and 18 UIP patients who underwent surgical or video-assisted thoracoscopic lung biopsy were reviewed.

Results

NSIP was more frequent in women and showed nonspecific clinical manifestations. High-resolution computed tomography (HRCT) demonstrated ground-glass, net-like, and patchy attenuation in both lungs. Semiquantitative HRCT showed a median fibrosis score of 3 (range, 0 to 7) in NSIP patients and 5 (range, 2 to 7) in UIP patients (P<0.01). On histopathologic examination, NSIP cases were heterogeneous and the findings could be categorized into cellular and fibrosing patterns. The mean age of the NSIP and UIP patients was 48 and 60 years, respectively. The frequencies of fibroblast foci, myogelosis, honeycomb lesions, and pulmonary structural destruction in NSIP and UIP patients were 16.7% and 100% (P<0.001), 22.2% and 85.7% (P<0.05), 16.7% and 92.9% (P<0.001), and 27.8% and 100% (P<0.05), respectively. The responses to glucocorticoid treatment and the prognosis were significantly greater in NSIP than those in UIP.

Conclusions

NSIP was difficult to be differentiated from UIP by general clinical manifestations, but HRCT can be helpful for this purpose. Definitive diagnosis depends on the results of surgical lung biopsy.     

Nonspecific interstitial pneumonia (NSIP)

Nonspecific interstitial pneumonia (NSIP) represents one histologic subtype of idiopathic interstitial pneumonia (IIP). NSIP is typified by temporal homogeneity and less profusion of fibroblastic foci than is seen with usual interstitial pneumonia (UIP), the most common IIP. Clinically patients with NSIP present with similar symptoms (cough and dyspnea) when compared to patients with UIP. The duration of these symptoms prior to presentation is variable. The finding of fever may be more common in NSIP and clubbing may be more common in UIP; however, both findings can be seen in either UIP or NSIP. Physiological findings typically demonstrate a restrictive ventilatory defect with decreased gas transfer; little difference exists between UIP and NSIP. High resolution computed tomography (HRCT) scans are more likely to show honeycombing with UIP and a ground-glass pattern with NSIP, however, either of these findings can be seen with UIP or NSIP. The most striking differential feature between NSIP and UIP is the markedly better prognosis for patients with NSIP, a finding that cannot be explained by baseline differences in physiology or radiographic features. In this article we explore the clinical, physiological, and radiographic features of NSIP. We also review available information regarding response to therapy and prognosis.     

Nonspecific interstitial pneumonia pattern as pulmonary involvement of rheumatoid arthritis

The pathologic patterns of lung involvement were evaluated in 16 patients with rheumatoid arthritis (RA). They consisted of six females and ten males, with a median age of 67.5 years and diagnosed according to the American Rheumatism Association revised criteria. High-resolution computed tomography (HRCT) of the lungs was performed in all patients, and honeycomb formation was apparent in seven. Histopathologically, seven patients were diagnosed with usual interstitial pneumonia (UIP) pattern, seven with nonspecific interstitial pneumonia/fibrosis (NSIP) pattern, and two with UIP/NSIP hybrid pattern. There were no apparent honeycomb formations on HRCT in patients diagnosed with NSIP pattern. In conclusion, the present study demonstrates that NSIP pattern is also a significant histologic classification of interstitial pneumonia associated with RA.     

Fibrotic idiopathic interstitial pneumonia: high-resolution computed tomography considerations

The fibrotic idiopathic interstitial pneumonias comprise usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). The characteristic high-resolution computed tomography findings of UIP are reticular abnormality and honeycombing with basal and peripheral predominance. Honeycombing is the strongest predictor of UIP. The extent of fibrosis on computed tomography (CT) is an important prognostic indicator in idiopathic pulmonary fibrosis (IPF). When ground-glass attenuation is seen in IPF it commonly progresses to fibrosis and honeycombing. Imaging may help to detect complications of IPF, including accelerated progression, lung cancer, and secondary infection. Our understanding of the clinical and radiological features of NSIP is still evolving. The CT finding of extensive ground-glass abnormality and some reticular abnormality, with basal and peripheral predominance, is strongly suggestive of NSIP. However, the CT appearances of NSIP overlap with those of UIP, organizing pneumonia (OP), and desquamative interstitial pneumonia (DIP), and biopsy may be necessary to sort this out. Other idiopathic pneumonias that may be associated with CT evidence of lung fibrosis include DIP, OP, acute interstitial pneumonia, and lymphoid interstitial pneumonia.     

Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis

The pathological features of lung disease in nine patients with systemic sclerosis (SSc) were evaluated. The patients comprised one man and eight women, with a median age of 58 years. SSc was diagnosed according to the criteria of the American Rheumatism Association. In all patients, high resolution computed radiographic scanning of the lungs (HRCT) was performed, and apparent honeycomb formation was seen in four patients. Pathologically, four patients were diagnosed with usual interstitial pneumonia (UIP), three with non-specific interstitial pneumonia (NSIP) group II, one NSIP group II-III, and one NSIP group II with diffuse alveolar damage. HRCT showed no apparent honeycomb formations in patients diagnosed with NSIP. This is the first report describing NSIP as a pulmonary complication of SSc.