【Abstract】 Objective To investigate the clinical characteristics and prognosis of secondary lymphocytic interstitial pneumonia ( LIP) . Methods Clinical data of 9 cases with secondary LIP diagnosed from1990 to 2010 were retrospectively analyzed. Results Of 9 patients there were 3 males and 6 females,the range of age was 7-64 years. In the 6 adult patients there were 5 females. 2 cases were infected by EB virus and 1 by recurrent pulmonary infection in 3 non-adult patients. In the adult patients, 1 case was diagnosed with Sjogren’s syndrome, 1 case with overlapping syndrome, 2 cases with primary biliary cirrhosis,1 case was probably caused by infection, and 1 case was complicated with eosinophilia. Dominant symptoms of pulmonary system were cough, expectoration, and shortness of breath on exertion. Dominant systematic symptoms were asthenia, pyrexia, weight lose, and arthralgia. CT revealed diffuse ground glass opacities with a lower lung zone predominance. Pathologic feature of LIP was a diffuse, polyclonal lymphoid cell infiltration surrounding airways and extending to the lung interstitium. The patients were treated by glucocorticoid and immunosuppressants. Two cases died with secondary infection. Follow-up did not comfirm malignant tumors in the survivors. Conclusions The clinical features of LIP are characteristic, but lacking of specificity. The final diagnosis depends on pathological examination. Treatment targeted on primary diseases can probably have a good efficacy, and the clinical outcome is favorable.
Objective To highlight the characteristics of giant cell interstitial pneumonia ( GIP) . Methods The clinical, radiological, and pathological data of two patients with GIP pathologically proven by open lung and TBLB biopsy were presented respectively, and relevant literatures were reviewed. Results Patients with GIP usually had a history of exposure to metal dust. Clinical presentations included cough and dyspnea on exertion, and pulmonary function testing showed a restrictive abnormality. On chest radiography and high-resolution CT scans, it presented as bilateral areas of ground-glass attenuation, areas of consolidation, diffuse small nodules, extensive reticular opacities, and traction bronchiectasis. The main pathological findings included a desquamative interstitial pneumonia ( DIP) -like reaction with intra alveolar macrophages and numerous large multinucleated histiocytes that ingested inflammatory cells were admixed with macrophages. The finding of GIP was almost pathognomonic for hard metal pneumoconiosis. Conclusions GIP is a very rare chronic interstitial pneumonia, and has no characteristic clinical manifestations. Radiographic findings are similar to other idiopathic interstitial pneumonias. Careful collection of the occupational history can help to minimize misdiagnosis.
Objective To investigate the role of transbronchial lung biopsy ( TBLB) in the diagnosis and prognosis evaluation of idiopathic interstitial pneumonia ( IIP) . Methods Clinical data of IIP patients admitted to Peking University First Hospital from January 2005 to April 2009 were analyzed retrospectively. The pathologic manifestations of TBLB samples of these patients had been reviewed by pathologists and respirologists. Chest high-resolution computer tomography ( HRCT) of the patients had been reviewed, and subsequent follow-ups were performed. The influence factors on diagnosis value of TBLB were analyzed. The relationship between TBLB findings and prognosis were observed. Results 29 IIP patients had been investigated, among them 21 were male and 8 were female, ranging in age from 43 to 79 years, with an average of 63 ±10 years.16 TBLB samples had diagnostic value, among themthe total number of the samples was from 2 to 7, with an average of 4.2 ±1.8. 13 TBLB samples had no diagnostic value, among them the total number of the samples was from0 to 5, with an average of 2.1 ±1.6. There was significant differences between two groups( P lt;0.005) . Among the 16 cases with diagnostic value of TBLB, non-specific interstitial pneumonia ( NSIP) pattern was showed in 9 cases, usual interstitial pneumonia ( UIP) pattern in 6 cases and organizing pneumonia ( OP) in 1 case. Among 29 cases, UIP-like pattern was showed on HRCT in 14 cases, while non-UIP pattern showed on HRCT in 15 cases. On follow-up of the 16 patients, 1 patient aggravated and 5 patients died among 6 cases with UIP pattern, 8 patients got better and 1 patient died among 9 cases with NSIP pattern, and the patient with OP got better. The patients who show non-UIP pattern in TBLB survived longer than UIP pattern with significant difference between two groups ( P lt; 0.005) . Conclusions The diagnosis value of TBLB is bly related to the number of samples and the patterns of HRCT. When the number of samples is more than or equal to 3, the diagnosis value of TBLB is better. It is easier to get valuable TBLB samples in patients with non-UIP pattern on HRCT than that with UIP pattern. The patients with non-UIP pattern in TBLB have better prognosis than those with UIP pattern. TBLB is useful in diagnosis and prognosis evaluation of IIP.
Objective To analyze the clinical symptoms, imaging, laboratory tests, efficacy and other indicators of patients diagnosed as diffuse parenchymal lung disease ( DPLD) , in order to provide a reference for differential diagnosis of secondary DPLD and idiopathic interstitial pneumonia ( IIP) .Methods Sixtytwo patients diagnosed as DPLD were retrospectively analyzed. Results In 62 patients with DPLD, 19 patients ( 30. 6% ) were diagnosed as secondary DPLD, 42 cases ( 67. 7% ) as IIP, 1 case ( 1. 6% ) as Langerhans cell histiocytosis. The smoking rate of the DPLD patient was 33. 8% , which was higher than general population ( 29. 7% ) . 94. 7% of the secondary DPLD patients had cough, which was higher than the IIP patients ( 71. 4%) . The average age of onset of the secondary DPLD and IIP was ( 45. 9 ±16. 8) years and ( 60. 5 ±7. 7) years respectively, without significant difference ( P gt; 0. 05) . Etiological factors of secondary DPLD were dust, pets, drugs, pesticides, decoration material, etc. The secondary DPLD patients had higher response rate to steroid therapy, but had no statistical difference compared with the IIP patients ( 46. 2% vs. 37. 5% , P gt;0. 05) . Conclusions As a group of diseases of known causes, history taking is very important for DPLD diagnosis and differential diagnosis. Clinical symptoms, imaging, and laboratory tests may provide reference for differential diagnosis of secondary DPLD and IIP.
ObjectiveTo compare the clinical characteristics,high-resolution computed tomography (HRCT) manifestations,pulmonary function results,serum autoantibodies and treatment modality of connective tissue diseases related interstitial lung diseases (CTD-ILDs) and idiopathic interstitial pneumonias (ⅡPs). MethodsPatients explicitly diagnosed with CTD-ILDs and ⅡPs were retrospectively selected from Nanjing Drum Tower Hospital between January 2004 and December 2012.The clinical features were abstracted,including age,gender,symptoms,signs,serum autoantibody results,HRCT findings,and treatment.Patient characteristics were compared between CTD-ILDs and ⅡPs using a Pearson's χ2 test for categorical variables,and a Student's t test for continuous variables. ResultsA total of 692 patients with complete data were included in this study,with 240 CTD-ILDs cases and 452 ⅡP cases.CTD-ILDs could exist in different types of CTDs,which were mainly shown in Sjogren's syndrome,rheumatoid arthritis,and dermatomyositis/polymyositis.Age,gender,connective tissue diseases related characteristics such as dry eyes,dry mouth,and arthralgia,and several autoantibodies such as ANA,SSA,SSB all showed significantly difference between CTD-ILDs and ⅡPs (P<0.05).However there were no significant differences in cough,dyspnea after exertion,velcro crackles on auscultation,or finger clubbing between two groups (P>0.05). The HRCT manifestations of CTD-ILDs were reticular opacities,patchy consolidation,band-like shadows,and pleural thickening.Pulmonary function tests commonly showed restrictive lung function and decreased diffusing capacity.The histopathologic findings of lung biopsies of CTD-ILDs were mostly chronic inflammatory cell infiltration,as well as hyperplasia of fibrous tissue and septal thickness.The finding of chronic inflammatory cell infiltration showed significant difference between CTD-ILDs and ⅡPs (P<0.05),while the HRCT manifestations,pulmonary function results or other histopathologic findings did not(P>0.05).The current treatment modality was corticosteroids plus immunosuppressants. ConclusionDespite the similarities,CTD-ILDs show distinct clinical,laboratory and imaging features from from ⅡPs in clinical practice.
ObjectiveTo evaluate the effectiveness of different biopsy methods in the diagnosis of idiopathic interstitial pneumonia (IIP). MethodsA retrospective analysis was performed in patients hospitalized and diagnosed as IIP between January 2010 to December 2013 in the Department of Respiratory Medicine, the First Affiliated Hospital of Guangzhou Medical University. The patients were divided based on initial diagnoses. The performance of invasive examinations including both transbronchoscopic lung biopsy (TBLB) and surgical lung biopsy (SLB) and final diagnoses were recorded. ResultsThree hundred and ninety-one patients were included in the study with mean age of 62.5±11.9 years. There were 299 patients receiving TBLB, 43 patients receiving SLB, and 49 patients receiving non-invasive examinations. Among 299 patients who underwent TBLB, parenchymal tissue was not obtained in 132 (44.1%) but successfully obtained in 167 (55.9%) patients, with 79 (47.3% out of 167) clinically helpful. TBLB-based specific diagnoses were as follows:30 cases of non-specific interstitial pneumonia (NSIP), 22 cases of cryptogenic organizing pneumonia (COP), 18 cases of idiopathic pulmonary fibrosis (IPF), 4 cases of acute interstitial pneumonia (AIP), 2 cases of respiratory bronchiolitis-interstitial lung disease (RB-ILD) and lymphocytic interstitial pneumonia (LIP) for each, 1 case of desquamative interstitial pneumonia (DIP), respectively. Among 43 patients who underwent SLB, parenchymal tissue was obtained in all cases with 40(93.0% out of 43) pathologically diagnostic, including 20 cases of NSIP, 12 cases of IPF, 3 cases of COP and LIP for each, and 2 cases of RB-ILD and DIP for each. Finally, among 181 patients who failed to obtain parenchymal tissue or did not undergo invasive examination, specific diagnoses could be established in 40 patients with all finally diagnosed as IPF. ConclusionsThe pathological diagnosis, either based on TBLB or SLB, is very important in the diagnostic procedure of IIP. The biopsy technique of hospital and patient conditions should be considered when making the decision of biopsy method.
ObjectiveTo retrospectively analyze the clinical,pathological and imaging features of one case pathologically diagnosed as cryptogenic organizing pneumonia(COP) to improve clinical diagnosis and treatment. MethodsWith a case report and review of the related literatures,the clinical manifestations,radiological features,pathological features,differential diagnosis,management and prognosis of COP were discussed. ResultsThe clinical manifestations of COP had no specificity. The imaging manifestations were real shadows,ground glass shadows,nodules and all kinds of tape. Pathological features of lung specimen biopsy showed buds of granulation tissue within alveolar ducts and alveoli consisting of fibroblasts. Remarkable response to corticosteroids was found in this patient. The prognosis of COP was good. ConclusionsCOP is diagnosed on basis of clinical,pathologic,and imaging findings. The radiological features of COP which show mass with cavity are rare. It can be easy misdiagnosed as lung infection or tumor. The effects of ordinary anti-bacteria therapy are limited,while the corticosteroids therapy shows preferable effects. Therefore,it's important to acquire pathological evidences as early as possible to guide the diagnose and treatment.
ObjectiveTo investigate the clinical characteristics of polymyositis (PM)/dermatomyositis (DM) with acute interstitial pneumonia (AIP) as the presenting symptoms, and identify characteristics of such disease. MethodsA retrospective analysis was conducted on the hospitalized patients with PM/DM with AIP as the presenting symptoms, from October 2009 to June 2015 in the Departemnt of Respiratory Medicine, Guangzhou Institute of Respiratory Diseases. ResultsThey were two males and six females with a mean age of 54.8±7.5 years. The common clinical features included fever (8 cases), shortness of breath (8 cases), rapidly progressive exertional dyspnea (8 cases), dry cough (6 cases), decreased muscle strength (8 cases), and typical rash (7 cases). Electromyography showed neurogenic or myogenic leision in these 8 cases. Muscle biopsy revealed myositis in 7 cases. High resolution CT (HRCT) revealed widespread ground glass patterns in all patients. All patients received noninvasive positive pressure mechanical ventilation on the first hospital day. High dose of methylprednisolone or combination with intravenous cyclophosphamide were initiated on 2.3±1.4 hospital day. Six patients survived to hospital discharge and two patient died. ConclusionsThe most common symptoms in patients of PM/DM with AIP are shortness of breath, progressive exertional dyspnea, and dry cough. Typical rash is seen in most of the patients.The diagnosis can be established by combinating the characteristics of HRCT, electromyography and muscle biopsy. Earlier intervention with noninvasive positive pressure mechanical ventilation and immunosuppressive may improve clinical outcome in patients of PM/DM with AIP.
Objective To summarize the clinical characteristics of patients with interstitial pneumonia with positive myeloperoxidase (MPO-IP). Methods The clinical data of 15 patients hospitalized with MPO-IP from June 2013 to January 2016 were analyzed retrospectively, including clinical manifestations, laboratory test, lung function test, chest high resolution computed tomography (HRCT) and management. Simultaneously, 11 patients with IPF were recruited as control. Results The onset age of MPO-IP was older than that in IPF patients (74.07±6.31 yearsvs. 66.73±6.80 years,P＜0.01). There was no manifestation of vasculitis in kidney in all included patients, and the differences were not significant in gender, smoking, cough and expectoration, wheezing, shortness of breath, HRCT manifestations, FVC%pred, TLCO%pred, PaO2 or PaCO2 between the two groups. In 15 MPO-IP patients, HRCT revealed 12 cases of usual interstitial pneumonitis (UIP), 2 cases of nonspecific interstitial pneumonia (NSIP), 1 case of organizing pneumonia (OP), and 5 patients with overlapped emphysema. Eleven IPF patients were all in UIP pattern and 4 with overlapped emphysema. One MPO-IP patients and 5 IPF patients had clubbed-fingers. The differences in clubbed-finger and treatment were significantly different between the two groups. Nine IPF patients received symptomatic treatment and7 MPO-IP patients were administered with glucocorticoids and immunosuppressants. In addition 2 MPO-IP patients were treated with pirfenidone. Conclusions MPO-IP patients have older onset age of disease and lower incidence of clubbed-finger than IPF patients. UIP is the main HRCT change both in MPO-IP and IPF. Beside glucocorticoid and immunosuppressants, pirfenidone is a choice and worthy of further research in management of MPO-IP.
Objective To investigate the clinical characteristics of interstitial pneumonia patients with positive anti-signal recognition particle antibody (SRP-IP), and compare those with interstitial pneumonia patients with positive anti-Jo-1 antibody (Jo1-IP). Methods Clinical data of SRP-IP patients admitted to Department of Respiratory and Critical Care Medicine of Drum Tower Hospital affiliated to Nanjing University Medical School from May 2017 to May 2021, including clinical manifestations, laboratory examinations, pulmonary function tests and radiographic types, were retrospectively analyzed. The results were compared with those of Jo1-IP patients admitted during the same period. Results The SRP-IP patients were older than Jo1-IP patients (P=0.044). There were no significant differences in clinical manifestations or pulmonary function tests results between the two groups. The proportion of SRP-IP patients combined with positive anti-EJ antibody (P<0.001) or perinuclear anti-neutrophil cytoplasmic antibody (P=0.028) was significantly higher than that of Jo1-IP patients, while the proportion of SRP-IP patients combined positive anti-Ro-52 antibody was significantly lower than that of Jo1-IP patients (P=0.009). The erythrocyte sedimentation rate (ESR) of SRP-IP patients was faster than that of Jo1-IP patients (P=0.026). The serum IgM level (P=0.039) and peripheral NK cell counts (P=0.013) of SRP-IP patients were significantly lower than those of Jo1-IP patients. The most common chest CT findings in SRP-IP patients were organizing pneumonia and the proportion of usual interstitial pneumonia in SRP-IP patients was higher than that of Jo1-IP patients (P=0.032). The levels of creatine kinase (P=0.010), creatine kinase myocardial brand (P=0.025) and alanine aminotransferase (P=0.045) in interstitial pneumonia patients with high titer (++~+++) SRP antibody were higher than those in interstitial pneumonia patients with low titer (+) SRP antibody. SRP-IP and Jo1-IP patients were mainly treated with glucocorticoids combined with or without immunosuppressants, and there was no significant difference in the choice of treatment between the two groups. The proportion of patients with Jo1-IP evaluated as improved was significantly higher than that of patients with SRP-IP (p=0.005), while the proportion of patients with SRP-IP evaluated as stable was significantly higher than that of patients with Jo1-IP (P=0.035). The mortality of SRP-IP patients within 3 months was significantly higher than that of Jo1-IP patients (P=0.028). Conclusion Compared with Jo1-IP patients, SRP-IP patients are older, have faster ESR, are more likely to be combined with other autoantibodies, have lower serum IgM level and peripheral blood NK cell count, have more UIP imaging manifestations, and have a worse short-term prognosis.