• Journal of Yeungnam Medical Science
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• v.39 no.2
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• pp.153-160
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• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.746-754
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• 1996

Objective: Diffuse interstitial lung disease (DILD) is a group of diverse diseases that share conUTIon clinical, radiologic, and pulomonary function features. Open lung biopsy (OLB) has been regarded as gold standard in differential diagnosis of DILD. However open lung biopsy is a invasive diagnostic tool not free of its own risk or complications. These days, high-resolution computed tomography (HRCf) has become an important diagnostic tool in DILD through its precise image analysis. In many instances, HRCT could provide specific diagnosis or, at least, provide infonnation on the disease activity of DILD. The authors re-evaluate the role of open lung biopsy in this "HRCT era" by investigating the additional diagnostic gain and impacts on the treannent plan in patients who have undergone high-resolution CT. Method : Diagnoses obtained by high-resolution CT and open lung biopsy were compared and changes of treatment plans were evaluated retrospectively in 30 patients who had undergone open lung biopsy for the purpose of diagnosis of diffuse interstitial lung disease from March 1988 to June 1994. Results : High-resolution cr suggeted specific diagnoses in 22 out of 28 patients (78.6%) and the diagnoses were confinned (0 be correct by open lung biopy in 20 of those 22 cases (91%). Open lung biopsy could not give specific diagnosis in 5 out of 30 cases (16.7%). In 5 out of 6 cases (83.3%) in whom high reolution cr was not able to suggest specific diagnosis, open lung biopsy gave specific diagnoses. Treatment plan was altered by the result of open lWlg biopsy in only 2 cases. Conclusion: The aoove fmdings suggest that in "HRCT era", when HRCT could suggest specific diagnosis, the need for open lung biopsy should be re-evaluated.

• 대한핵의학회:학술대회논문집
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• 1989.06a
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• pp.124.1-124.1
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• 1989

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.770-790
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• 2021

Idiopathic pulmonary fibrosis (IPF), based on the 2018 international clinical practice guidelines, can be diagnosed with a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) and compatible clinical findings. Given that imaging is pivotal for IPF evaluation and diagnosis, more emphasis should be placed on the integration of clinical, radiological, and pathologic findings for multidisciplinary diagnosis. Interstitial lung abnormality (ILA), on the other hand, has a purely radiological definition based on the incidental identification of CT abnormalities. Taken together, differentiation between ILA and clinically significant interstitial lung disease (ILD) must be based on proper clinical evaluation. With this review, the recent updates in IPF diagnosis and the radiologic considerations for ILA can be well understood, which can be helpful for the proper diagnosis and management of patients with diffuse interstitial pulmonary fibrosis.

• Tuberculosis and Respiratory Diseases
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• v.41 no.6
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• pp.597-603
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• 1994

Background: Because of the power of HRCT to detect the minute changes in lung parenchyme and the advantage of noninvasiveness, it may be the ideal method of follow-up evaluation of the patients with diffuse interstitial pulmonary fibrosis. So the aim of the study is to find out whether the change of HRCT during the course of the disease can represent the change in symptom and pulmonary function test. Method: Thirteen patients with diagnosis of diffuse interstitial pulmonary fibrosis who had more than two times of HRCT during the course of disease were studied. The extent of the disease in HRCT was measured at three levels using image analyzer. The ralationship between the percent change in the disease extent in HRCT, symptom score, and pulmonary function tests were analysed. Results: The change of disease extent in HRCT has good correlation with the change in dyspnea and diffusion capacity(r=0.716, p=0.0012). But there was no correlation between the change of HRCT and FVC or TLC. Also significant inverse correlation was noted between the change in dyspnea and diffusion capacity(r=-0.707, p=0.0047). Conclusion: These data suggest that HRCT may be a good method of follow-up in diffuse interstitial pulmonary fibrosis.

• Tuberculosis and Respiratory Diseases
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• v.50 no.6
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• pp.732-739
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• 2001

Systemic lupus erythematosus frequently has thoracic involvement among connective tissue diseases. One of the pleuropulmonary manifestations is diffuse interstitial lung disease including nonspecific interstitial pneumonia(NSIP). NSIP is a newly classified disease among interstitial lung diseases. Systemic lupus erythematosus has a better prognosis than usual interstitial peumonia(UIP) and responds well to steroids. In this report, a 34 year-old woman who complained of a dry cough, and exertional dyspnea for 2 months is described. The chest X-ray showed fine reticular opacities and a mild honeycomb appearance in both basal lungs. High resolution computed tomography(HRCT) showed bilateral patchy areas of ground-glass attenuation and a mild honeycomb appearance in the subpleural of both the lower and the middle portion of the lung fields. An open lung biopsy showed prominent lymphocytic interstitial inflammation and fibrosis with small areas with a honeycomb appearance. This case was diagnosed as NSIP associated with systemic lupus erythematosus and was managed with oral steroids. Here we report a case of nonspecific interstitial pneumonia associated with systemic lupus erythematosus confirmed by HRCT and an open lung biopsy with a review of the relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.45 no.2
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• pp.360-368
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• 1998

Background: Endothelin(ET), a potent vasoconstrictor peptide produced by endothelial cells and degraded predominantly in the pulmonary vasculature, have been implicated in the development of various organ dysfunctions. Plasma concentrations of ET-1 are reported to be elevated in patients with diffuse interstitial lung disease(DILD). But, there is no study to establish the exact source and mechanisms involved in the increased plasma ET-1 concentrations in DILD patients. Methods: 12 patients with IPF, 2 patients with sarcoidosis, 2 patients with scleroderma, 1 patient with SLE and 11 healthy volunteers were studied. ET was detected by radioimmunoassay in plasma and bronchoalveolar lavage fluid(BALF) as well as in 24-hr urine specimens. For each subjects, arterial/venous(A/V) ET ratio and renal ET clearance were calculated. Results: Elevated plasma, urine and BALF ET concentrations were found in patients with DILD compared with controls. But, no significant difference was observed in ET A/V ratio and ET renal clearance between patients with DILD and controls. Conclusion: We observed that plasma ET concentrations were elevated in patients with DILD, and that the main site of ET production may be lung parenchyme.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.529-534
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• 2002

Interstitial pneumonia is a heterogenous group of inflammatory and fibrosing lesions that manifest themselves as infiltrative lung disease. Of these, nonspecific interstitial pneumonia is characterized as a variable degree of interstitial inflammation with or without fibrosis and is distinguished from usual interstitial pneumonia and desquamative interstitial pneumonia, histologically. The influx of inflammatory cells and the responses of immune effector cells injury to the alveolar wall and these initial injuries results in alveolitis and fibrosis. Consequently, the gas exchange throughout the alveolar wall is impaired and the patients suffer from lung diseases of a restrictive pattern. The chief complaints represented are dyspnea and dry cough. We experienced a case of nonspecific interstitial pneumonia in a 10-year old girl. The patient had been healthy and had not been exposed to organic dusts or other toxic materials. The pathology of lung biopsy tissue showed that the alveoli were thickened by a mixture of chronic inflammatory cells and collagen type fibrosis. High resolution computed tomography(HRCT) found the patchy areas of ground-glass opacity with patchy consolidation and irregular reticular opacity, and diffuse distribution without zonal predominance. The forced vital capicity(FVC) was 31%, forced expiratory volume in one second ($FEV_1$) 29% and $FEV_1/FVC$ 90%, so a restrictive pulmonary insufficiency was found.

• Journal of Chest Surgery
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• v.39 no.11 s.268
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• pp.844-849
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• 2006

Background: The diffuse infiltrative lung disease requires surgical lung biopsy for its final diagnosis. We evaluated the effect of surgical lung biopsy for final diagnosis of duffuse interstitial lung disease and compared video assisted thoracoscopic lung biopsy(TLB) with open lung biopsy(OLB). Material and Method: We evaluated the patients who underwent surgical lung biopsy from March 2000 from December 2005, retrospectively, We divide to two groups(OLB and TLB group) and compared them. Result: There were 36 patients and cough was the most common pre- operative symptom. Surgery time, anesthetic time, hospital stay, duration of chest tube indwelling, specimen volume and the rate of post-operative complication were not significantly different between two groups. Histologic diagnosis was confirmed in all cases. There was one post-operative death who had suffered from respiratory failure since pre-operative period. Conclusion: Surgical lung biopsy is effective method in final diagnosis for diffuse infiltrative lung disease. Video assisted thoracoscopic lung biopsy is lesser invasive method than open lung biopsy and provide similar results, so it is basic diagnostic method of surgical lung biopsy.

• Clinical and Experimental Pediatrics
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• v.48 no.3
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• pp.327-332
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• 2005

Interstitial lung disease refers to a group of pulmonary disorders characterized by inflammation of the interstitium, derangements and loss of alveolar capillary units leading to disruption of alveolar gas exchange, which induces symptoms of restrictive lung disease. Cases of interstitial pneumonia in children are uncommon and mostly have unknown causes. We have experienced an 8-year old boy who had symptoms of cyanosis, dry cough, dyspnea and abrupt weight loss. He had not been exposed to organic dusts, allergens or any other systemic disease infections. Chest radiology showed diffuse ground glass opacity in both lung fields. High resolution computed tomography(HRCT) showed multiple small patchy areas of consolidation with an underlying ground glass appearance in both lungs. The pathologic findings of lung biopsy tissue showed patchy areas of interstitial fibrosis, alveolar obliteration and nodular fibrotic areas, strongly suggesting interstitial pneumonia. No specific finding of viral inclusion or any other evidence of infection was found under electromicroscopy. We used peak flow meters to compare functional improvement. Forced expiratory volume in one second ($FEV_1$) was decreased to 25 percent of predicted value. The boy was given treatment with prednisone and showed improvements in HRCT findings after two months. He was able to tolerate easy exercise in school and showed clinical improvements after one year of follow up.