• Tuberculosis and Respiratory Diseases
• /
• v.83 no.4
• /
• pp.312-320
• /
• 2020

Background: Systemic sclerosis (SSc) involves multiple organ systems and has the highest mortality among connective tissue diseases. Interstitial lung disease is the most common cause of death among SSc patients and requires closer studies and follow-ups. This study aimed to identify lung function changes and predictors of progressive disease in systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: A retrospective study extracted SSc patients from an electronic database January 2002-July 2019. Eligible cases were SSc patients >age 15 diagnosed with SSc-ILD. Factors associated with progressive disease were analyzed by univariate and multivariate logistic regression analyses. Results: Seventy-eight SSc-ILD cases were enrolled. Sixty-five patients (83.3%) were female, with mean age of 44.7±14.4, and 50 (64.1%) were diffuse type SSc-ILD. Most SSc-ILD patients had crackles (75.6%) and dyspnea on exertion (71.8%), and 19.2% of the SSc-ILD patients had no abnormal respiratory symptoms but had abnormal chest radiographic findings. The most common diagnosis of SSc-ILD patients was non-specific interstitial pneumonia (43.6%). The lung function values of diffusing capacity of the lung for carbon monoxide (DLCO) and DLCO per unit alveolar volume declined in progressive SSc-ILD during a 12-month follow-up. Male and no previous aspirin treatment were the two significant predictive factors of progressive SSc-ILD with adjusted odds ratios of 5.72 and 4.99, respectively. Conclusion: This present study showed that short-term lung function had declined during the 12-month follow-up in progressive SSc-ILD. The predictive factors in progressive SSc-ILD were male sex and no previous aspirin treatment. Close follow-up of the pulmonary function tests is necessary for early detection of progressive disease.

• Journal of Chest Surgery
• /
• v.37 no.12
• /
• pp.1032-1035
• /
• 2004

The specific diagnosis in diffuse interstitial lung disease may be obtained through open lung biopsy. Diffuse interstitial lung disease is often associated with lung cancer. We report one case of lung adenocarcinoma with idiopathic pulmonary fibrosis in whom previous open lung biopsy had been performed. We need general concepts about sites of open lung biopsy in these patients. Therefore, we report this case and document other references.

• Journal of Yeungnam Medical Science
• /
• v.36 no.1
• /
• pp.8-15
• /
• 2019

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

• Journal of Chest Surgery
• /
• v.27 no.10
• /
• pp.850-853
• /
• 1994

Open lung biopsy was performed in thirty patients for the diagnosis and staging evaluation of interstitial lung disease during the period from January 1987 until December 1992. The age of the patients ranged from 14 to 71 years [mean 48 years], and the patients consisted of 14 males and 16 females. Preoperative FEV1`s were from 0.80 liter to 3.88 liters [mean 1.66]. Other non-invasive diagnostic studies such as PCNA, bronchoalveolar lavage, TBLB, and gallium scan were also done in addition to X-ray and high-resolution chest CT. Tweaty-eight were correctly diagnosed and 2 cases were not [diagnostic yield rate 93.3%]. Among the 28 cases,pathologic diagnosis influenced further treatment regimens and prognostic expectations in 23 cases [82.1%]. The diagnostic non-invasive studies other than open lung biopsy yielded a correct diagnosis without staging only in 5 cases. There was no mortality and only one complication, ARDS ; however, the patient recovered after 5 days ventilator support. Open lung biopsy, which is the gold standard for the diagnosis and staging evaluation of interstitial lung disease can be done safely and has value in clinical decision making. Also knowledge of the involvement of the lesion is important for proper selection of the biopsy site.

• Tuberculosis and Respiratory Diseases
• /
• v.74 no.2
• /
• pp.70-73
• /
• 2013

Dyskeratosis congenita is a rare congenital disorder characterized by a triad of reticular pigmentation of the skin, dystrophic nails, and leukoplakia of the mucous membrane. Sometimes it is associated with bone marrow failure, secondary malignancy and interstitial lung disease. Though it is rare, Dyskeratosis congenita is diagnosed relatively easily when clinicians suspect it. It can be diagnosed just by gross inspection with care. Dyskeratosis congenita should be considered as one cause associated with interstitial lung disease. In Korea, interstitial lung disease with dyskeratosis congenita has not been reported. We report a case and review the literature.

• Tuberculosis and Respiratory Diseases
• /
• v.65 no.4
• /
• pp.328-333
• /
• 2008

Desquamative interstitial pneumonia is an uncommon form of interstitial lung diseases and it has a good prognosis compared with other types of idiopathic interstitial pneumonia. A 69-year old man was admitted to our hospital because of a 3-month history of dyspnea. The patient presented with hypoxemia. High-resolution computerized tomography of the patient showed ground glass opacity and traction bronchiectasis with subpleural early honeycombing on the both lung fields. The pathologic findings of the video-assisted thoracoscopy lung biopsy were compatible with desquamative interstitial pneumonia, and irregularly distributed interstitial fibrosis and inflammation were observed at the peripheral parenchyme. Oral predinsolone was started; his symptoms and chest x-ray were improved, and so he stopped taking the prednisolone. Ten months later, the desquamative interstitial pneumonia recurred. We report here on a case of recurrent desquamative interstitial pneumonia with fibrotic lung disease.

• Journal of the Korean Institute of Oriental Medical Informatics
• /
• v.13 no.1
• /
• pp.26-38
• /
• 2007

Interstitial lung disease (ILD) is characterized by progressive scarring of the lung leading to restriction and diminished oxygen transfer. Clinically, the presenting symptoms of ILD are nonspecific (cough and progressive dyspnea on exertion) and are often attributed to other diseases, thus delaying diagnosis and timely therapy. In this study, I analyzed the 10 chinese papers of interstitial lung diseases(ILD). The etiology are body resistance weakness(本虛) and pathogenic factor prevailing(標實). The body resistance weakness(本虛) including deficiency of the lung(肺虛), deficiency of the kidney(腎虛), deficiency of the spleen(脾虛), deficiency of Qi and Yin(氣陰兩虛), pathogenic factor prevailing(標實) including stagnation of phlegm(痰濁), blood stasis(瘀血), noxious heat(熱毒). As an treatment aim at supplementing lung and kidney(益肺腎), resolving phlegm and blood stasis(化痰瘀).

• Tuberculosis and Respiratory Diseases
• /
• v.77 no.2
• /
• pp.81-84
• /
• 2014

A patient treated with venlafaxine for major depression developed an interstitial lung disease (ILD) with the characteristic clinical, radiological and pathological features of chronic hypersensitivity pneumonitis. A high resolution computed tomography scan demonstrated ground glass opacity, mosaic perfusion with air-trapping and traction bronchiectasis in both lungs. The pathological findings were consistent with a nonspecific interstitial pneumonia pattern. Clinical and radiological improvements were noted after the discontinuation of venlafaxine and the administration of a corticosteroid. This report provides further evidence that the anti-depressant venlafaxine can cause ILD.

• Tuberculosis and Respiratory Diseases
• /
• v.84 no.4
• /
• pp.255-262
• /
• 2021

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

• Proceedings of the Korea Inteligent Information System Society Conference
• /
• 2001.01a
• /
• pp.378-382
• /
• 2001

The purpose of this study was to develop an expert system supporting the diagnosis of diffuse interstitial lung disease by high resolution computed tomography. CLIPS(C language integrated production system) with rule-based reasoning was used to develop the system. Development of expert system had three stages knowledge acquisition, knowledge representation, and reasoning. Knowledge was obtained and integrated, from tables and figure legends of a representative textbook in the domain of this expert system, High-Resolution CT of the Lung, by Webb WR, Mueller NL, and Naidich DP. The acquired knowledge was analyzed to form a knowledge base. Overlapping knowledge was eliminated, similar pieces of knowledge were combined and professional terms were defined. The most important knowledge of findings was then selected for each disease. After groupings of combined findings were made, disease groups were analyzed sequentially to determine final diagnoses. The system was based upon the input of 69 diseases, 185 findings, 73 conditions, 387 status, and 62 rules. The system was set up to determine the diagnoses of diseases from the combination of findings using forward reasoning. In an empirical trial, the system was applied to support the diagnosis of 40 cases of diffuse interstitial lung diseases. The performance of two doctors with support of the system was compared to that of another two doctors without support of the system. The two doctors with the support of the system made more accurate diagnoses than the doctors without the support of the system. The system is believed to be useful for the diagnosis of rare diseases and for cases with many possible differential diagnoses. In conclusion, an expert system supporting the high resolution computed tomographic diagnosis of diffuse interstitial lung disease was developed and the system is thought to be useful for medical practice.