CHARACTERIZED BY A DESQUAMATIVE INTERSTITIAL PNEUMONIA WITH GIANT CELLS. COEXISTS WITH SYMPTOMS pdf

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1 1: Pneumonia, Desquamative Interstitial GIP is characterized by patchy interstitial chronic inflammation with a centrilobular accentuation, numerous intraalveolar collections of macrophages (as in desquamative interstitial pneumonia), and bizarre syncytial giant cells that often contain macrophages and neutrophils in their cytoplasm (Figs. and ). There is often black. Case Rep Gastrointest Med. Published online Jun This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Symptoms remained virtually unchanged following administration of antibiotics. A final diagnosis of organizing pneumonia OP was made based on findings of intra-alveolar buds of granulation tissue and fibrous thickening of the alveolar walls on pathological examination and patchy consolidations and ground glass opacities on computed tomography. Immediate administration of prednisolone provided rapid, sustained improvement. Although a rare complication, OP is a pulmonary manifestation that requires attention in CD patients. Noninfectious pulmonary diseases associated with CD are uncommon. These associations are generally considered to arise from extraintestinal manifestations of the disease itself and adverse effects to therapeutic drugs prescribed to ameliorate inflammation [ 3 ]. Organizing pneumonia OP is a particular form of pneumonia presenting as diffuse interstitial lung disease that affects the distal bronchioles, respiratory bronchioles, alveolar ducts, and alveolar walls [ 4 â 6 ]. OP appearing with CD is extremely rare. Only a single report has described nonresolving pneumonia in 3 CD patients receiving immunosuppressive agents: Case Report This patient was a year-old man who had previously undergone partial ileal resection due to intestinal obstruction and partial peritonitis at the age of He had since been admitted to other hospitals because of repeated temporary obstructions at the anastomotic site. At the age of 62, the patient was referred to our hospital with recurrent severe abdominal pain and exacerbation of CD. The patient had no history of lung disease, occupational exposures, or extraintestinal manifestations and no family history of CD. Physical examination revealed the following: A small bowel series showed longitudinal ulcers and strictures at the ileal anastomotic site. Colonoscopy and pathological examination of a biopsied ulcer specimen showed moderate to severe inflammatory cell infiltrates with some epithelioid granulomas in the lamina muscularis mucosae and submucosa. Based on these findings, CD was initially diagnosed. Two years later, the patient was admitted to our hospital due to rapid exacerbation of productive cough, accompanied by fever of up to Laboratory investigations showed the following: Low albuminemia was due to severe inflammation and loss of appetite. Chest computed tomography CT revealed patchy consolidations, ground glass opacities, and small nodular opacities, predominantly in the middle, lingula, and bilateral lower lobes Figures 1 b and 1 c. Based on these findings, we considered a diagnosis of bacterial pneumonia due to streptococcal species. Despite antibiotic treatment with intravenous piperacillin-tazobactam and meropenem and supportive care for 14 days, respiratory symptoms remained unimproved and radiographic findings gradually deteriorated. A transbronchial lung biopsy from a right lung lesion showed fibrous thickening of the alveolar wall and intra-alveolar buds of granulation tissue associated with fibroblasts Figure 2. Based on these findings, a final diagnosis of OP associated with CD was made though postinfectious OP could not be completely ruled out. The patient has remained well for a year. Page 1

2 2: Pathology Outlines - Desquamative interstitial pneumonitis (DIP) interstitial pneumonia, desquamative chronic pneumonia with desquamation of large alveolar cells and thickening of the walls of distal air passages; marked by dyspnea and nonproductive cough. double pneumonia that affecting both lungs. To begin with, pneumonia is inflammation of the lungs due to infection. The lungs are two organs in the body that help people breathe. Interstitial pneumonia is a long-term inflammatory lung disease that affects the interstitial connective tissue of the lungs. The inflammation is due to the build up of white blood cells and protein -rich plasma the watery part of blood throughout the alveoli. White blood cells help protect the body against diseases and fight infections. Alveoli are tiny sacs where gases are exchanged in the lungs so that breathing can take place. The inflammation in interstitial pneumonia extends beyond the end of the bronchioles. Bronchioles are small airways that branch off the other small airways connected to the lungs. These other small airways are known as bronchi. If the inflammation lasts long enough it can cause fluid in the lungs to harden, resulting in the production of firm connective tissue scar tissue. The production of this firm tissue is known as fibrosis. There is also collagen formation in the walls of the alveoli. Collagen is a fiber -like protein found in the skin. A cyst is an abnormal lump, swelling, or sac that contains fluid, a part solid material, or a gas, and is covered with a membrane. A membrane is a thin layer of flexible tissue that covers something. As the disease progresses, the bronchi and the walls of the bronchi eventually become widened, the walls of the bronchi are destroyed, and the lungs shrink. When patients with interstitial pneumonia die and the lungs are examined, there are characteristic large cells found lining the alveoli. The giant cells that line the alveoli have multiple nuclei. A nucleus is the structure at the center of a cell. More than one nucleus is known as nuclei. The presence of these large cells in the alveoli is why interstitial pneumonia is also known as giant cell pneumonia. Interstitial pneumonia is most common between the ages of 40 and There are different types of interstitial pneumonia which are each sufficiently different enough from each other to be considered distinct diagnoses. The different types of interstitial pneumonia are described below: This is the most common type of interstitial pneumonia. UIP is a deadly disease that commonly occurs in people 60 years or older that were otherwise in good health. It is not uncommon in the 5th decade of life. Males and females are affected in equal numbers. UIP begins slowly, with inflammation of the alveoli and increased numbers of cells in the walls of the alveoli. As the disease progresses, it becomes more difficult to breath. By the time the diagnosis is made, breathing ability has significantly decreased. The lung tissue will have an abnormal, honeycombed appearance, much like a bee hive. This is due to the disease causing patchy distortion and destruction of the lung tissue structure. The honeycombs are actually holes in the lung that are about one centimeter wide. The presence of honeycombs usually represents the end stage of many forms of lung disease. In about half of UIP patients, x-rays of the lungs, show hazy appearance of ground glass in the lungs. The ground glass appearance is known as ground glass opacities and is usually due to inflammation of the alveoli, thickening of the alveoli walls, or early fibrosis an overgrowth of connective tissue. Small amounts of old fibrosis see above are also present. There is also recent granulation tissue present. Granulation tissue is new connective tissue and tiny blood vessels that is commonly seen on the surfaces of wounds during the healing process. A pneumothorax is the presence of air or gas in the pleural space, causing a lung to collapse. Part of the lung tissue will also look normal in UIP. The pleura is the smooth, moist double layer of flexible tissue that lines the lungs and the chest wall. The pleural space is a thin, fluid-filled opening between the pleura of the lungs and the pleura of the chest wall. Over time, lung size decreases in UIP, which can also be seen on x-ray. On CT scans, a network of more transparent areas known as reticular opacities can be seen, which is due to lung destruction, honeycombing, fibrosis see above, and bronchiectasis. CT scanning is an advanced imaging technique that uses x-rays and computer technology to produce more clear and detailed pictures than a traditional x-ray. Bronchiectasis is irreversible widening and destruction of the walls of the bronchi. It is unclear if bronchiectasis is due to interstitial pneumonia or the frequent bacterial infections that these patients often Page 2

3 experience. These findings are present in almost all patients with UIP and are usually found beneath the pleura and towards the bottom of both sides of the lungs. Remember, the pleura is the smooth, moist double layer of flexible tissue that lines the lungs and the chest wall. This type of interstitial pneumonia occurs in current or past smokers. Current smokers are usually affected in the 30s or 40s and feel out of breath after only mild physical exertion. Although this condition is called pneumonia, there is no evidence that the inflammation is caused by an infection. The lesions in this type of pneumonia are spread throughout the tissue and are usually the same size. There is mild inflammation in the interstitial tissue and fibrosis see last section is present. There are many macrophages present in the alveoli tiny air sacs with a light brown coloring. A macrophage is a type of white blood cell that eats bacteria. Hyperplasia of the lung occurs, which means that there is an increased number of cells present. Chest x-rays generally show less severe changes than in UIP see above. For example, in this type of pneumonia, there is little honeycombing. There is also a decreased amount of oxygen in the blood. This is an uncommon lung disease in which mature lymphocytes and plasma cells build up in the alveoli see above and in the spaces between alveoli. A lymphocyte is a type of white blood cell present in the blood. Plasma is the watery part of blood. LIP occurs in children, but rarely in adults. LIP progresses slowly and leads to the formation of cysts in the lungs. Pulmonary fibrosis can be a long-term complication of patients with LIP and often develops slowly. Respiratory distress has been reported, especially in children with LIP. Lymphoma can also result from lymphoid interstitial pneumonia. Lymphoma is cancer of the lymphatic tissue. The lymphatic system is a system of vessels that drain lymph from all over the body and back into the blood. Lymph is a milky fluid that contains proteins, fats, and white blood cells which help the body fight off diseases. A condition known as pseudolymphoma is a variant of LIP that causes the build-up of harmless abnormal tissue masses. Pseudolymphoma is not a disease, but is an inflammatory reaction to certain known or unknown stimuli. AIP is a type of interstitial pneumonia that comes on suddenly acute and with great severity. It has no known cause. AIP is also known as severe interstitial lung disease, idiopathic adult respiratory distress syndrome, and Hamman-Rich Disease. CIP is a type of interstitial pneumonia with the following characteristics. First is the presence of polyps that contain granulation tissue in the tubular space of bronchioles the smallest airways of the lungs and alveolar ducts. The alveolar ducts are part of the airway passages that are positioned away from the bronchioles. Polyps are a type of growth that projects from the lining of mucous membranes. A mucous membrane is one of four major types of thin sheets of tissue that line or cover various parts of the body. Granulation tissue is a combination of new connective tissue and tiny blood vessels. The second characteristic of CIP is the presence of patchy areas of pneumonia, consisting largely of mononuclear cells and foamy macrophages a type of white blood cell that eats bacteria. Mononuclear cells are cells with one nucleus. RB-ILD is a mild type of interstitial lung disease seen in heavy cigarette smokers. Symptoms include cough, phlegm production, and uncomfortable breathing. Two thirds of patients have crackles that can be heard when the doctor taps the chest area and listen for sounds. Pulmonary function tests show a restrictive defect. After the patient stops smoking, damage in the lungs seen on CT scan appears to remain stable or resolve slowly without progressing to lung disease with fibrosis. NSIP is a type of interstitial pneumonia that cannot be classified into one of the above types. NSIP begins somewhat quickly. The signs and symptoms of interstitial pneumonia include progressively worse discomfort while breathing, fever, occasional wheezing, blue discoloration of the skin, and an abnormal enlargement of the end of the fingers known as clubbing. There also tends to be a non-productive cough for one to three years. For people with lymphoid interstitial pneumonia LIP, enlargement of lymph nodes or lymph vessels may occur. Lymph nodes are small egg shaped structures in the body that help fight against infection. Lymph vessels are tube-shaped structures that carry lymph throughout the body. People with LIP may also experience enlargement of the liver, spleen, and parotid glands. Page 3

4 3: Organizing Pneumonia in a Patient with Quiescent Crohn's Disease Desquamative interstitial pneumonia (DIP) is characterised by the accumulation of numerous pigmented macrophages within most of the distal airspace of the lung and, sometimes, the presence of giant cells. Diagnosis of DIP is not easy and requires surgical lung biopsy. DIP is usually associated with. RB-ILD and DIP are different entities and not two junctures in a continuum, based on their distinct clinical presentation, radiographic features, histology, response to treatment, and prognosis. Histologically, DIP is characterized by an alveolar accumulation of macrophages followed by interstitial inflammation and fibrosis. It usually portends a poorer prognosis. The original authors also did not report the distinctive association with smoking which was first highlighted by Carrington et al. The characteristic findings of pigmented macrophages, therefore, have a distinct bronchiolocentric distribution with centrilobular ground-glass opacities in chest imaging. The extensive alveolar infiltration of macrophages renders them an eosinophilic stain due to the abundant eosinophilic cytoplasm of the macrophages. Radiologically, cystic spaces may be present in areas of ground glass opacities. Those small cystic air spaces seen in chest imaging in DIP have been shown to correlate histologically with dilated alveolar ducts and bronchiectasis. Toward late-stage disease, production of transforming growth factor beta-1 results in fibrosis around alveolar interstitium. Unlike usual interstitial pneumonia, however, fibroblastic foci are almost never seen. Multinucleated giant cells are also often reported. History and Physical An overlap of clinical and histopathological features are common among smoking-related interstitial pneumonia. Individuals with a history of relevant exposure to cigarettes, inhalational drugs of abuse, and occupational risk factors commonly present with a dry cough and exercise-induced shortness of breath. Although childhood disease has been rarely reported as mentioned above, the most common age of presentation is 40 to 60 years. Hemoptysis is very rare. Evaluation Peripheral blood may reveal an increase in neutrophil and eosinophil count. Pulmonary function tests show a restrictive defect, with impaired diffusion capacity unlike usual obstructive pattern in respiratory bronchiolitis. Chest x-ray may show a reticulonodular infiltrate in lower lung zones but appears fairly subtle and nonspecific. High-resolution CT chest is the imaging modality of choice. It shows diffuse bilateral lower lobe predominant ground glass changes in a homogenous distribution, and without honeycombing- the last two features distinguishing it from usual interstitial pneumonia or idiopathic pulmonary fibrosis. In progressive disease, cysts and traction bronchiectasis may be seen, but it is not exclusively subpleural. However advanced DIP is reported to be indistinguishable from NSIP with a temporally uniform pattern of fibrosis beyond the initial accumulation of alveolar macrophages. Interestingly in a study reported by Cartier et al. A surgical lung biopsy is the gold standard of diagnosis. In recent years, transbronchial cryobiopsy is being explored as an alternative diagnostic approach with an increase in sample size by non-invasive means. Similarly if associated with specific occupational exposures, avoidance of exposure is also key to preventing disease progression. Systemic corticosteroid therapy over months has been reported as the most effective pharmacologic intervention. With corticosteroids and immunosuppressive therapy azathioprine being the most commonly used agent, most patients remain stable or improve with reports of even complete recovery. Finally, with progressive and severe lung disease causing hypoxemia, pulmonary hypertension, severe reduction in TLC or DLCO, lung transplantation evaluation is indicated [1] [26] ; although, there are reports of recurrence of DIP post lung transplantation. Idiopathic pulmonary fibrosis Idiopathic nonspecific interstitial pneumonia NSIP Cryptogenic organizing pneumonia Acute interstitial pneumonia formerly Hamman-Rich syndrome Idiopathic lymphoid interstitial pneumonia. Page 4

5 4: Interstitial Pneumonia: Signs, Symptoms, & Treatment- Desquamative interstitial pneumonia (DIP) Intra-alveolar macrophage accumulation Not a true desquamation of epithelial cells Almost always seen in smokers Clinical. Background Interstitial pneumonias are a confusing and frustrating set of diseases both for the treating physician and for the diagnostic pathologist. One source of the confusion has been the lack of overlapping terms that treating physicians and pathologists use. For example, idiopathic pulmonary fibrosis IPF is a clinical term describing a slowly progressive, chronic interstitial pneumonia. To complicate matters even further, European clinicians utilize the term cryptogenic fibrosing alveolitis for IPF. Pathologists also share in the confusion. Terms that were commonly used by pathologists just a few years ago have also undergone an evolution. Bronchiolitis obliterans with organizing pneunomina BOOP is no longer used because it has been considered a mixture of terms. Lymphocytic interstitial pneumonia LIP is now considered a lymphoproliferative disease. Giant cell interstitial pneumonitis GIP is now considered a hard metal pneumoconiosis. The symptoms vary for each of the pneumonias but most are characterized by a slowly progressive shortness of breath. Chest radiographs reveal a hazy ground glass appearance with linear opacities. Most of the diseases are progressive and are treated with corticosteroids. Usual interstitial pneumonia UIP is a form of lung disease characterized by progressive scarring of both lungs. The scarring fibrosis involves the supporting framework interstitium of the lung. UIP is thus classified as a form of interstitial lung disease. The term "usual" refers to the fact that UIP is the most common form of interstitial fibrosis. The term used for UIP in the British literature is cryptogenic fibrosing alveolitis, a term that has fallen out of favor since the basic underlying pathology is now thought to be fibrosis, not inflammation. Causes The cause of the scarring in UIP may be known or unknown. The latter situation is more common. Symptoms The typical symptoms of UIP are progressive shortness of breath and cough for a period of months. In some patients, UIP is diagnosed only when a more acute disease supervenes and brings the patient to medical attention. Prognosis Regardless of etiology, UIP is relentlessly progressive, usually leading to respiratory failure and death. Some patients do well for a prolonged period of time, but then deteriorate rapidly because of a superimposed acute illness so-called "accelerated UIP". The outlook for long-term survival is poor. In most studies, the median survival is 3 to 4 years. How is interstitial lung disease treated? The choice of treatment depends upon a number of factors including the cause of the interstitial lung disease and the overall health status of the patient. Most commonly, corticosteroid drugs are given in an attempt to reduce the inflammation. Sometimes, immune-suppressing drugs, such as asazathioprine Imuran or cyclophosphamide Cytoxan are also given, either in combination with steroids or following a course of steroid treatment. Smoking cessation is critical for those with interstitial lung disease. Pneumonia What is pneumonia? Pneumonia is an infection of the tiny air sacs of the lungs. There are many different types of pneumonia, which can range from mild to severe. An incurable, deadly lung disorder. Medical Marijuana Medical marijuana should never be smoked by anyone with lung problems. My suggestion is that you acquire a Sativa x Indica hybrid and then make yourself a whole plant tincture. Place a drop under the tongue twice a day to start. These you should also make yourself using: Or if making an oil: There are articles on our website: Acquire organically grown medical marijuana free of any pathogens. Make your own whole plant extracts. This way you will be taking the best medicine possible. General principles and recommendations. Sumikawa H, et al Human Pathology 39 9: Coalition for Pulmonary Fibrosis: Page 5

6 5: Interstitial Pneumonia In desquamative interstitial pneumonia, the biopsy usually shows a distinctive pattern of widespread and evenly distributed lung inflammation. The most striking feature of the lung biopsy is the presence of. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Symptoms remained virtually unchanged following administration of antibiotics. A final diagnosis of organizing pneumonia OP was made based on findings of intra-alveolar buds of granulation tissue and fibrous thickening of the alveolar walls on pathological examination and patchy consolidations and ground glass opacities on computed tomography. Immediate administration of prednisolone provided rapid, sustained improvement. Although a rare complication, OP is a pulmonary manifestation that requires attention in CD patients. Noninfectious pulmonary diseases associated with CD are uncommon. These associations are generally considered to arise from extraintestinal manifestations of the disease itself and adverse effects to therapeutic drugs prescribed to ameliorate inflammation [ 3 ]. Organizing pneumonia OP is a particular form of pneumonia presenting as diffuse interstitial lung disease that affects the distal bronchioles, respiratory bronchioles, alveolar ducts, and alveolar walls [ 4 â 6 ]. OP appearing with CD is extremely rare. Only a single report has described nonresolving pneumonia in 3 CD patients receiving immunosuppressive agents: Case Report This patient was a year-old man who had previously undergone partial ileal resection due to intestinal obstruction and partial peritonitis at the age of He had since been admitted to other hospitals because of repeated temporary obstructions at the anastomotic site. At the age of 62, the patient was referred to our hospital with recurrent severe abdominal pain and exacerbation of CD. The patient had no history of lung disease, occupational exposures, or extraintestinal manifestations and no family history of CD. Physical examination revealed the following: A small bowel series showed longitudinal ulcers and strictures at the ileal anastomotic site. Colonoscopy and pathological examination of a biopsied ulcer specimen showed moderate to severe inflammatory cell infiltrates with some epithelioid granulomas in the lamina muscularis mucosae and submucosa. Based on these findings, CD was initially diagnosed. Two years later, the patient was admitted to our hospital due to rapid exacerbation of productive cough, accompanied by fever of up to Laboratory investigations showed the following: Low albuminemia was due to severe inflammation and loss of appetite. Chest computed tomography CT revealed patchy consolidations, ground glass opacities, and small nodular opacities, predominantly in the middle, lingula, and bilateral lower lobes Figures 1 b and 1 c. Based on these findings, we considered a diagnosis of bacterial pneumonia due to streptococcal species. Despite antibiotic treatment with intravenous piperacillin-tazobactam and meropenem and supportive care for 14 days, respiratory symptoms remained unimproved and radiographic findings gradually deteriorated. A transbronchial lung biopsy from a right lung lesion showed fibrous thickening of the alveolar wall and intra-alveolar buds of granulation tissue associated with fibroblasts Figure 2. Based on these findings, a final diagnosis of OP associated with CD was made though postinfectious OP could not be completely ruled out. The patient has remained well for a year. Laboratory findings on admission. Progressive pulmonary symptoms and manifestations were seen during scheduled ADA maintenance therapy. Treatment with prednisolone induced rapid, sustained improvements. Discussion CD often shows extraintestinal manifestations such as uveitis and ankylosing spondylitis [ 9 ]. On the other hand, primary lung involvement is rare and reportedly includes bronchiolitis obliterans organizing pneumonia BOOP, pulmonary interstitial emphysema, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, fibrosing alveolitis, and eosinophilic pneumonitis [ 2, 3 ]. Lung involvement can also be secondary to the drug used to treat CD [ 10 ]. In this type of idiopathic diffuse interstitial lung disease, granulation tissue obstructs the alveolar ducts and alveolar spaces and chronic inflammation arise in the adjacent alveoli [ 5, 6 ]. Pulmonary interstitial emphysema is characterized by air trapping outside the normal air passages and inside the connective tissue of the Page 6

7 peribronchovascular sheaths, interlobular septa, and visceral pleura; this disease entity is more frequent in premature infants who require mechanical ventilation for severe lung disease [ 12 ]. Desquamative interstitial pneumonia is a chronic lung inflammation characterized by mononuclear cell infiltration of the airspaces. It occurs almost exclusively in current or former cigarette smokers [ 13 ]. Fibrosing alveolitis is characterized by inflammation and thickening of the alveolar walls and usually occurs in individuals over the age of 40 years [ 14 ]. Eosinophilic pneumonitis is a disease characterized by accumulation of eosinophils in bronchoalveolar lavage and lung tissue [ 15 ]. In the present case, the final diagnosis of OP associated with CD was made based on the pathological and CT findings and the clinical course [ 16 â 18 ]. However, in terms of the clinical features, postpneumonic OP and secondary OP, these are actually fairly similar. Differentiating postpneumonic OP from secondary OP based on the radiographic findings was difficult. Despite the use of wide-spectrum antibiotics, respiratory symptoms persisted and findings on chest radiography gradually deteriorated. In general, in the early period of postpneumonic OP, antibiotics therapies are partially effective and yield some degree of improvement in symptoms and radiological findings [ 19, 20 ]. Based on the above, we concluded that secondary OP was more likely than postpneumonic OP. Consensus is currently lacking regarding a definitive approach to diagnosing drug-induced lung disease. In a few cases, drug-induced lung disease can show a clinical course and radiographic findings like OP [ 24, 25 ]. In the present case, at disease onset, the patient was immediately given prednisolone, which provided rapid and sustained improvement. The prednisolone was subsequently tapered while ADA and mesalazine were continued. As a result, the patient has remained well without recurrence of OP. These results suggest that this lung disorder was unlikely to present a drug-induced lung disease and was instead more likely to be OP associated with CD. For patients with progressive symptoms of cryptogenic organizing pneumonia COP and diffuse radiographic changes, initial therapy with oral glucocorticoids is recommended for promising clinical outcomes [ 26, 27 ]. However, one case has been described in which initial treatment with glucocorticoids led to poor outcome, whereas a dramatic response to infliximab was seen. Interestingly, OP cases have not been known to be associated with the disease activity of IBD [ 2, 11, 28 ]. In conclusion, although OP is a rare complication, due vigilance is required regarding the occurrence of pulmonary symptoms and manifestations in CD and IBD patients. Competing Interests The authors declare that there is no conflict of interests regarding the publication of this paper. Corley III, and M. Page 7

8 6: Organizing Pneumonia in a Patient with Quiescent Crohnâ s Disease Three cases of an unusual interstitial pneumonia, designated by Liebow as giant cell interstitial pneumonia, were encountered by us. Dyspnea, weight loss, cough, chest pain, rales in the lungs and clubbing of the fingers were the frequently observed clinical features. Choon Sik Park, M. This article has been cited by other articles in PMC. There are few reports about the clinical features of NIP. Methods The pathologic slides of 66 patients having open lung biopsy OLB for the differential diagnosis of interstitial lung diseases ILD from to were reviewed. Seven cases were confirmed as NIP. Results Six patients with NIP were female. The median age was 56 years. Mean duration of symptoms was 4 months. Five patients had systemic flu-like symptoms. Diffusion capacity of carbonmonoxide DLCO decreased markedly. In bronchoalveolar lavage BAL, total cell counts elevated about three times of normal value. On differntial counts of BAL cells, lymphocytes, neutrophils and eosinophils were higher than those of normal controls. The prominent finding of chest radiology was bilaterally patchy opacifications in parenchyme of lower lung zones. The average period from diagnosis to the last follow-up was Five patients were clinically recovered to the previously well-being state. They also had characteristic findings of radiology and their prognosis seems to be better than UIP. Hamman and Rich reported the clinical and pathologic findings of four cases, who expired due to a fulminant pulmonary fibrosis with rapidly progessing course in 1. Thereafter, many cases of diffuse interstitial pneumonias with chronic courses have been reported. Although the terminology has been used inconsistently, IPF represents the interstitial penumonias without any evidence of underlying causes or other disease. In, Liebow and carrington classified interstitial pneumonias into five subcategories by pathologic findings, usual interstitial pneumonia UIP, desquamative interstitial pneumonia DIP, bronchiolits with interstitial pneumonia BIP, lymphocytic interstitial pneumonia LIP and giant cell interstitial pneumonia GIP 2, 3. In, Katzenstein 4 identified acute interstitial pneumonia AIP as a new subcategory of fibroblasts is a main component in AIP, whereas the dense deposition of collagens is prominent in UPI. The clinical feature to differentiate NIP from UIP is that the former seems to have a better prognosis than the latter. However, there have been few reports about the clinical features of NIP till now. We firstly reported clinical features of three cases at the 77th Conference on Tuberculosis and Respiratory Diseases of Korea in 6. Because of the shortage of clincal data so far, we summarized the clinical features, radiologic and pathologic findings of seven patients with NIP. Study Population Study population was composed of 66 patients having open lung biopsy OLB for the differential diagnosis of interstitial lung diseases ILD from to For pathologic proof, all pathologic specimens were reviewed by two pathologists in our hospital. NIP is characterized by a variable degree of intersitial inflammation with or without fibrosis. However, it appeared temporally uniform within each case and areas of honeycombing were not found. Histologic features for a specific category of interstitial pneumonia were not present. Group I was characterized by a cellular interstitial pneumonia with littly fibrosis. The interstitial infiltrate was minimal to mild and consisted of small lymphocytes Fig. There were patch involvement and accentuation around bronchioles. Group II was characterized by a cellular interstitial pneumonia with significant fibrosis Fig. The alveolar septa were widened by cellular infiltrations, which consisted of lymphocytes, plasma cells and a few eosinophils. An area of germinal center formation was noted. There was a marked hyperplasia of alveolar lining cells. The fibroblasts were composed of mature collagen and proliferating fibroblasts. Large numbers of intraalveolar macrophages and fibroblast foci were found. Group III was characterized by architectural derangement due to dense interstitial collagenous fibrosis and less degree of inflammation Fig. This process was diffuse in one case and patchy in the other case. The alveolar septa were markedly thickened, resulting in narrowing of air spaces. The alveolar lining cells were hyperplastic and alveolar spaces were filled with alveolar macrophages. Areas of patchy interstitial infiltrate were noted, but their degree was mininal compared to that of group II. Page 8

9 7: Idiopathic interstitial pneumonias Radiology Reference Article Lymphocytic interstitial pneumonia is a rare idiopathic interstitial pneumonia characterized by infiltration of alveoli and alveolar septa with small lymphocytes and varying numbers of plasma cells. Non-necrotizing, poorly formed granulomas may be present but are usually rare and inconspicuous. Page 9

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