普通型间质性肺炎与非特异性间质性肺炎急性加重的临床与病理表现

目的 研究间质性肺疾病患者急性加重的临床和病理表现.方法 回顾性总结北京协和医院自1999年4月至2007年6月间经肺部病理活检证实的普通型间质性肺炎(UIP)和非特异性间质性肺炎(NSIP)急性加重患者各3例的临床资料.其中5例为急性加重前的间质性肺炎病理的结果,1例为急性加重后的尸检结果.结果 6例中男2例,女4例,年龄29～57岁(中位年龄51岁).急性加重前的病理表现:3例为UIP(1例为特发性,1例为皮肌炎继发,1例为结缔组织病继发),2例为NSIP(1例为特发性,1例为皮肌炎继发),1例尸检为弥漫性肺泡损伤合并NSIP(皮肌炎继发).6例中2例在急性加重前1周曾行胸腔镜肺活检术,5例发热,2例白细胞数增高,5例中性粒细胞比例升高.6例的痰培养和血培养无阳性发现.急性加重时氧合指数为200 mm Hg(四分位间距为158～237 mm Hg,1 mm Hg=0.133 kPa),4例ESR增快,2例C反应蛋白升高.胸部CT示在原有病变基础上出现磨玻璃影和实变影.5例使用糖皮质激素35～1000 mg/d,2例使用静脉人血丙种球蛋白,住院期间死亡4例,3例使用有创机械通气的患者均死亡,存活出院者1例稳定,1例好转,其后2例均失访.结论 UIP和NSIP都可出现急性加重,临床表现为突然出现的呼吸困难及发热,无特异性,诱因不清,广谱抗生素治疗无反应,大剂量糖皮质激素及静脉人血丙种球蛋白治疗可能有效.     

A case of group II non-specific interstitial pneumonia developed during corticosteroid therapy after acute respiratory distress syndrome

Non-specific interstitial pneumonia/fibrosis (NSIP) is a relatively new entity among idiopathic interstitial pneumonias. This disease is pathologically characterized by varying proportions of interstitial inflammation and fibrosis that appear to occur over a single, short period of time, and is clinically characterized by a generally good prognosis. Although corticosteroid therapy is widely considered to be effective for treatment of this disease, we report a case of Group II NSIP which developed during treatment with corticosteroid 4 months after acute respiratory distress syndrome (ARDS). This case suggests that any group of NSIP may be developed after ARDS, and that low-dose corticosteroid treatment is not sufficient to prevent the development of NSIP.     

Idiopathic non‐specific interstitial pneumonia

Non‐specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid‐adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High‐resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground‐glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi‐disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP.     

COVID-19 Pneumonia Resembling an Acute Exacerbation of Interstitial Pneumonia

An 84-year-old man was admitted with hypoxemia and ground-glass opacities with traction bronchiectasis in both lungs and mild fibrosis on computed tomography. We first suspected that he had acute exacerbation of interstitial pneumonia and initiated methylprednisolone pulse therapy. On day 4, he was diagnosed with coronavirus disease 2019 (COVID-19) pneumonia. Although the ground-glass opacities were improved with corticosteroid treatment alone, the hypoxemia persisted, and the plasma D-dimer level increased. Anticoagulant therapy was initiated, and the hypoxemia was improved. COVID-19 pneumonia may result in radiological findings similar to those of acute exacerbation of interstitial pneumonia, and corticosteroids and anticoagulant therapy may lead to favorable outcomes.     

非特异性间质性肺炎八例临床病理分析

目的：探讨非特异性间质性肺炎（NSIP）的病理和临床特点，方法：对8例经电视胸腔镜或小切口开胸肺活检诊断为NSIP的病例进行光镜观察和临床病理资料回顾性分析。结果：NSIP多见于女性（男：女＝1：3，2／6），平均年龄48岁，临床表现为渐进性呼吸困难，咳嗽，咳痰，双下肺闻及爆裂音，肺功能主要为限制性通气障碍，高分辨CT表现为双下肺野为主的网状，片状磨玻璃样改变，病理特征为均一的间质炎症和纤维化，4例有闭塞性细支气管炎伴机化性肺炎样改变，3例有纤维母细胞灶，1例有窝蜂肺，组织学类型，混合型6例，纤维化型2例，对皮质激素反应：显效组与有效组各4例，与纤维化型相比，大部分混合型对皮质激素有较好的反应，结论：NSIP的组织学类型与疗效有密切的关系，纤维化性的NSIP与普通型间质性肺炎在病理组织学上不易鉴别，诊断需密切联系临床。     

Cyclosporin treatment in steroid-resistant and acutely exacerbated interstitial pneumonia

OBJECTIVE: The aim of this study was to evaluate the efficacy of cyclosporin A (CsA) in patients with interstitial pneumonia (IP). DESIGN: Retrospective comparative study. PATIENTS: We reviewed 33 patients (23 males and 10 females with a mean age of 62.5 years) with histologically-proven progressive IP who were treated with CsA. All patients had corticosteroid-resistant IP or developed acute exacerbation of IP in their courses. RESULTS: The underlying systemic diseases were: idiopathic interstitial pneumonias (IIPs) in 19 patients, and collagen vascular diseases (CVDs) in 14. The histopathological patterns and underlying diseases of IP were classified as usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) in 10 patients, cellular-nonspecific interstitial pneumonia (NSIP)/IIPs in 3, fibrotic-NSIP/IIPs in 5, organizing pneumonia (OP)/IIP in 1, UIP/CVDs in 4, cellular-NSIP/CVDs in 7, fibrotic-NSIP/CVDs in 2, and diffuse alveolar damage (DAD)/CVD in 1, respectively. They received a low dosage of CsA combined with corticosteroids. The prognoses after treatment with CsA were well correlated with histopathological patterns. Cellular-NSIP and OP showed better prognoses than fibrotic-NSIP, UIP or DAD. In addition, CVDs had better prognoses than IIPs, when compared on the basis of the same histopathological patterns. Furthermore, the prognoses in the CsA-treated group were significantly better than in those without CsA treatment in regard to acute exacerbation of UIP/IPF. CONCLUSIONS: CsA combined with corticosteroids may be an efficacious treatment for corticosteroid-resistant IP and for acute exacerbation of IPF.     

Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP

In the 2013 reclassification of the idiopathic interstitial pneumonias (IIPs), two rare IIPs (idiopathic lymphoid interstitial pneumonia (LIP), idiopathic pleuroparenchymal fibroelastosis (IPPFE)) and two rare histologic patterns (acute fibrinous and organizing pneumonia (AFOP), bronchiolocentric pattern of interstitial pneumonia (BPIP)) are described. All these entities are rare with small series published to date, mostly containing primary and secondary forms of disease. LIP is histologically characterized by diffuse polyclonal lymphoid cell infiltrate surrounding the airways and expanding the interstitium. Thin‐walled cysts and diffuse ground glass are considered the typical radiologic features. The clinical course is highly variable with corticosteroid responsiveness evident in approximately half of cases. IPPFE is defined histologically by coexisting upper lobe pleural and intra‐alveolar fibrosis with elastosis. Dense subpleural irregular fibrosis and consolidation are the cardinal radiologic features. A history of recurrent lower respiratory tract infection is frequent. Responses to immunomodulation have not been reported and the rate of progression appears to be highly variable. AFOP is a rare histologic pattern lying within the spectrum of acute/subacute lung injury, characterized by organizing pneumonia and intra‐alveolar fibrin deposition without hyaline membranes. BPIP is characterized histologically by fibrosis and/or inflammation confined to the alveolar interstitium around bronchovascular bundles, overlapping with peribronchial metaplasia and fibrosis in some series. Currently, AFOP and BPIP are both best viewed as histological entities rather than true clinical disorders, in the absence of characteristic associated imaging patterns and clinical features.     

Desquamative interstitial pneumonia may progress to lung fibrosis as characterized radiologically

Background and objective: In some patients, desquamative interstitial pneumonia may progress to lung fibrosis. The aim of this study was to assess the long‐term radiological follow‐up results in patients with desquamative interstitial pneumonia. Methods: Among 75 patients suspected of having desquamative interstitial pneumonia, 31 who fulfilled the criteria were included in this study. Clinical characteristics at presentation, responses to treatment and long‐term follow‐up were evaluated. Results: The 31 patients were predominantly males (94%), and the mean age was 55 years; 93% (28/30) had a history of smoking. The clinical findings included high serum levels of lactate dehydrogenase and immunoglobulin G. Bronchoalveolar lavage (26 patients, 84% of cases) frequently showed an increased percentage of eosinophils (mean 17%). Computed tomography (CT) or high resolution (HR) CT at presentation showed ground glass opacities and/or consolidation in all patients, with one third of patients also showing thin‐walled cysts within the ground glass opacities. There was no honeycombing on CT or HRCT scans at presentation. Corticosteroid therapy was effective early in the course of the disease; long‐term follow‐up (mean 99 months) of 31 patients showed only one death due to progression of the disease, but long‐term follow‐up of 14 patients (mean 125 months) by HRCT showed the development of new thin‐walled cysts and honeycombing in five and lung cancer in four patients, respectively. Conclusions: In a proportion of patients, desquamative interstitial pneumonia may progress to lung fibrosis with honeycombing on HRCT, despite therapy.     

Anti-endothelial cell antibodies in patients with interstitial lung diseases

BACKGROUND: Anti-endothelial cell antibodies (AECA) are circulating antibodies that bind to endothelial antigens and induce endothelial cell damage. AECA have been detected in patients with collagen vascular disease (CVD) and their presence is associated with interstitial lung disease (ILD) in cases of CVD. However, the prevalence of AECA in patients with idiopathic interstitial pneumonias (IIPs) is not known. METHODS: We investigated the prevalence of AECA in patients with IIPs. We also examined whether the expression of AECA differed among the histologic subgroups usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP), and compared the values with those of CVD-associated ILD (CVD-ILD). Twenty patients with IIPs and 24 patients with CVD-ILD were studied. Serum samples were examined for AECA by cellular enzyme-linked immunosorbent assay (ELISA) using human umbilical vein endothelial cells. Values are expressed as ELISA ratios (ER). RESULTS: All sera from patients with idiopathic pulmonary fibrosis (IPF)/UIP were negative for AECA, whereas 5 out of 10 with idiopathic NSIP, 5 out of 14 with CVD-UIP and 4 out of 10 with CVD-NSIP tested positive (p<0.05). ER values were significantly lower in patients with IPF/UIP than idiopathic NSIP, CVD-UIP or CVD-NSIP (p<0.05). Among idiopathic NSIP, CVD-NSIP and CVD-UIP patients, the ER values did not differ. CONCLUSIONS: Among IIP patients, only those with idiopathic NSIP, not IPF/UIP, tested positive for AECA. The prevalence of AECA in idiopathic NSIP patients was similar to that in CVD-ILD patients. These results may provide important information to understand the distinct pathophysiology of each form of IIPs.     

Corticosteroid therapy against treatment-related pulmonary toxicities in patients with lung cancer

Background

With the recent increased use of new anti-neoplastic agents, molecular-targeted drugs and radiation in patients with lung cancer, there has been an increase in the occurrence drug-induced or radiation-induced pulmonary toxicities. We conducted this study to evaluate the clinical characteristics of patients with lung cancer who presented with treatment-related pulmonary toxicities and to analyze the dosage pattern of corticosteroid therapy against them.

Methods

To collect the baseline data from the patients with lung cancer who developed treatment-related pulmonary toxicities, we initially selected those who were prescribed corticosteroids between January 1, 2008 and December 31, 2012. Depending on clinical and radiological diagnoses, we classified pulmonary toxicities into drug-induced interstitial lung disease (DILD), radiation pneumonitis, acute exacerbation of chronic obstructive pulmonary disease (AE COPD) and others.

Results

We divided total patients (n=398) into four groups, and these include 88 cases (22%) of DILD, 189 cases (47%) of radiation pneumonitis, 47 cases (12%) of AE COPD and 74 cases (19%) of others. The prescribed rate of pulse or high-dose steroid was measured as 73%, 20%, 40% and 38%, respectively (P<0.001). In DILD radiologic findings, the 2-month mortality was significantly higher in the patients with the diffuse alveolar damage (DAD) pattern (100%) as compared with those with the non-specific interstitial pneumonia (NSIP) or bronchiolitis obliterans with organizing pneumonia (BOOP) one (62% or 42%, respectively) (P=0.032).

Conclusions

This study showed that the natural course of DILD had more unfavorable outcome requiring higher dose steroid therapy as compared with those with radiation pneumonitis or AE COPD. According to a subgroup analysis of the patients with DILD, BOOP and NSIP radiographic patterns showed more favorable outcomes.     

急性间质性肺炎

急性间质性肺炎是一种临床上引起快速呼吸衰竭的间质性肺疾病,可区别于其他慢性间质性肺炎,又称为Hamman-Rich综合征,主要发生在没有肺基础疾病的患者.组织病理学表现为弥漫性肺损伤.急性间质性肺炎的放射学和生理学改变与急性呼吸窘迫综合征相似,并且被认为代表了一小部分特发性急性呼吸窘迫综合征.急性间质性肺炎经常与其他快速进展的间质性肺炎相混淆,特别是继发性急性间质性肺炎、急性加重的隐源性机化性肺炎.本文阐述了急性间质性肺炎相关的临床和病理学问题.     

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.     

Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis

Amyopathic dermatomyositis (ADM) is occasionally complicated by rapidly progressive interstitial pneumonia (RPIP), and in such cases, diffuse alveolar damage (DAD) is usually diagnosed at autopsy. Here, we present three patients with RPIP accompanied by ADM in whom lung disease was assessed at an early stage. High-resolution computed tomography (HRCT) carried out before the onset of dyspnoea revealed uniformly subpleural reticular opacity with faint ground-glass attenuation. At that stage, surgical lung biopsies from two patients showed histological patterns typical of cellular nonspecific interstitial pneumonia (NSIP). Despite pulse methylprednisolone and subsequent high-dose oral administration of prednisolone, lung disease progressed in all patients, with extensive areas of ground-glass opacity and consolidation observed in HRCT scans. DAD was confirmed histologically in one case. Additional administration of cyclosporine, pulse cyclophosphamide or high-dose intravenous administration of immunoglobulin rescued all patients. Our data suggest that ADM-associated interstitial pneumonia takes an aggressive course even when the radiological and histological features are consistent with NSIP. Aggressive combination therapy with high-dose steroids and immunosuppressive agents is required as early as possible for patients with this life-threatening disorder.     

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.     

Osteopontin levels are elevated in patients with eosinophilic pneumonia

Background and objective: Osteopontin is a key cytokine involved in pro‐inflammatory T helper type 1 (Th1)‐associated immune responses, which has recently been implicated in allergic diseases. We investigated the pathogenic role of osteopontin in eosinophilic pneumonia. Methods: The concentrations of osteopontin and Th1‐ or Th2‐associated cytokines were measured in BAL fluid (BALF) from 41 patients with eosinophilic pneumonia, including those with acute (AEP, n = 12), chronic (CEP, n = 16), or drug‐induced eosinophilic pneumonia (DEP, n = 13). The results were compared with those from patients with other interstitial lung diseases. Immunocytochemistry and double immunofluorescence labelling were performed to determine the cellular source of osteopontin. Results: Osteopontin was significantly elevated in BALF from patients with eosinophilic pneumonia as compared with BALF from patients with drug‐induced interstitial pneumonia, hypersensitivity pneumonitis, idiopathic interstitial pneumonia, or sarcoidosis, and also compared with BALF from healthy volunteers. Osteopontin concentrations elevated at the time of exacerbation decreased during clinical improvement, either spontaneously or as a result of corticosteroid therapy. Elevated concentrations of CXCL10, CCL17 and IL‐10 were also detected in BALF from patients with eosinophilic pneumonia. Osteopontin concentrations in BALF of AEP patients were correlated with IL‐5, as well as IL‐10, CCL11, CCL17 and CXCL10 concentrations. In AEP and DEP patients, serum osteopontin concentrations were also elevated. Double immunofluorescence labelling showed that in patients with eosinophilic pneumonia, osteopontin was expressed in lung eosinophils. Conclusions: Osteopontin is likely to contribute to the development of inflammation in patients with eosinophilic pneumonia.     

Interstitial lung disease in primary Sjögren syndrome

BACKGROUND: Primary Sj?gren syndrome (pSS) has been associated with various histologic patterns of interstitial lung disease (ILD). METHODS: We retrospectively identified 18 patients with pSS and suspected ILD who underwent lung biopsies (14 surgical biopsies and 9 bronchoscopic biopsies) at our institution during a 13-year period from 1992 through 2004. Histopathologic findings were analyzed and correlated with radiologic features and outcome. RESULTS: Median age was 62 years (range, 34 to 78 years), and 15 patients (83%) were women. Most patients presented with dyspnea and cough. Chest radiographs demonstrated bilateral infiltrates, and high-resolution CT revealed abnormalities of various types including ground-glass, consolidation, reticular, and nodular opacities. The major histopathologic patterns included nonspecific interstitial pneumonia (NSIP) [five patients], organizing pneumonia (OP) [four patients], usual interstitial pneumonia (UIP) [three patients], lymphocytic interstitial pneumonia (three patients), primary pulmonary lymphoma (two patients), and diffuse interstitial amyloidosis (one patient). In four patients (three with OP and one with amyloidosis), the diagnosis was established on transbronchial biopsy results. Treatment commonly included prednisone with or without another immunosuppressive agent. During the follow-up period (median, 38 months), most patients improved or remained stable except three patients with UIP, one patient with NSIP, and one patient with amyloidosis. Seven patients (39%) died, including three deaths from acute exacerbation of interstitial pneumonia. CONCLUSIONS: A variety of histologic patterns can be seen in patients with pSS-associated ILD. Those with UIP tended to have progression of lung disease. Death from acute exacerbation of interstitial pneumonia may occur in patients with pSS-associated ILD.     

非特异性间质性肺炎临床-放射-病理诊断分析

目的对经肺活检诊断的非特异性间质性肺炎(NSIP)病例进行分析，探讨临床一放射一病理诊断的重要性。方法呼吸科、放射科和病理科医师对出院诊断和疑诊的9例NSIP病例的临床资料、影像特征和病理诊断进行回顾性分析，根据美国胸科学会和欧洲呼吸学会(ATS／ERS)的分类标准，重新作出一致的临床和病理诊断。结果出院诊断NSIP患7例，病理标本来自外科肺活检；疑诊NSIP患2例，病理标本来自CT引导下肺穿刺活检。回顾性病理分析发现，外科肺活检诊断的7例中5例符合ATS／ERS诊断标准，确诊为NSIP；1例主要病理特征为弥漫性支气管扩张，1例为机化性肺炎，不能诊断NSIP。经CT肺活检疑诊的2例因组织标本较小，不能进行全面病理评价，但因缺乏其他特征性病变，结合临床和影像表现，仍拟诊NSIP。全部病例的影像学表现以磨玻璃样阴影为主，均不具备特发性肺纤维化(IPF)的典型特征。确诊NSIP的5例中1例存在多肌炎／皮肌炎，临床诊断继发性NSIP；其余4例未发现潜在病因，临床诊断特发性NSIP，其中1例在诊断后3年因肺纤维化进行性加重、呼吸衰竭死亡。2例疑诊病例中1例在人院后20d死亡，1例在2年后确诊多肌炎／皮肌炎。结论NSIP的影像学表现缺乏特征性，外科肺活检是确立诊断的主要手段。NSIP的临床和病理诊断需要临床、放射和病理科医师的共同参与，而进一步寻找潜在病因是诊断过程中的重要目标。     

Immunophenotyping and remodeling process in small airways of idiopathic interstitial pneumonias: functional and prognostic significance

Background and Aims: To test whether different degrees of immunologic and fibrotic airway remodeling processes occur in idiopathic interstitial pneumonias (IIPs), with impact on functional tests and survival, we studied the collagen/elastic system and immune cell density in the bronchiolar interstitium of lungs with the major types of IIPs. Materials and Methods: Histochemistry, immunohistochemistry and morphometric analysis were used to evaluate collagen/elastic fibers and immune cells in the bronchiolar interstitium of open lung biopsies of patients with cryptogenic organizing pneumonia [COP/organizing pneumonia (OP) = 10], acute interstitial pneumonia [AIP/diffuse alveolar damage (DAD) = 20], nonspecific interstitial pneumonia (NSIP/NSIP = 20) and idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP) = 20. Results: OP lungs presented a significant increase in collagenous/elastic fibers and in the total density of immune cells in the bronchiolar interstitium compared to controls, DAD, NSIP and UIP. We observed a significant increase in CD4, CD8 and CD20 lymphocytes, as well as in neutrophils, macrophages and plasma cells in OP. The increased amount of elastic fibers in the bronchiolar interstitium from OP lungs has a direct association with forced vital capacity (FVC) (r_s = 0.99, P = 0.03). The most important survival predictor was CD20+ lymphocytes in the bronchiolar interstitium. In decreasing order, patients with UIP [Odds Ratio (OR) = 35.01], high forced expiratory volume in 1 s (FEV₁)/FVC FVC (OR = 7.01), increased CD20+ lymphocytes (OR = 4.44) and collagenous/elastic fiber densities (OR = 2.03 and OR = 1.49, respectively) in the bronchiolar interstitium were those who had the greatest risk of death, followed by those with AIP, NSIP and COP. Conclusion: Different degrees of immunologic and fibroelastotic airway remodeling processes occur in the major types of IIPs with impact on physiological tests and survival. Please cite this paper as: Parra ER, Noleto GS, Tinoco LJM, Capelozzi VL. Immunophenotyping and remodeling process in small airways of idiopathic interstitial pneumonias: functional and prognostic significance. The Clinical Respiratory Journal 2008; 2: 227–238.     

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.     

BAL findings in idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia.

Idiopathic pulmonary fibrosis (IPF), which has the histological pattern of usual interstitial pneumonia (UIP), is a progressive interstitial lung disease with a poor prognosis. Idiopathic interstitial pneumonias with a histological pattern of nonspecific interstitial pneumonia (NSIP) have a better prognosis than UIP, and may present with a clinical picture identical to IPF. The authors hypothesised that bronchoalveolar lavage (BAL) findings may distinguish between UIP and NSIP, and have prognostic value within disease subgroups. BAL findings were studied retrospectively in 54 patients with histologically proven (surgical biopsy) idiopathic UIP (n=35) or fibrotic NSIP (n=19), all presenting clinically as IPF. These findings were also compared with the BAL profile of patients with other categories of idiopathic interstitial pneumonias. BAL total and differential cell counts did not differ between the two groups. Survival was better in NSIP. In neither group were BAL findings predictive of survival or changes in lung function at 1 yr, even after adjustment for disease severity, smoking and treatment. BAL differential counts in fibrotic NSIP differed from respiratory bronchiolitis-associated interstitial lung disease, but not from desquamative interstitial pneumonia or cellular NSIP. The authors conclude that bronchoalveolar lavage findings do not discriminate between usual interstitial pneumonia and nonspecific interstitial pneumonia in patients presenting with clinical features of idiopathic pulmonary fibrosis, and have no prognostic value, once the distinction between the two has been made histologically.