Interstitial lung diseases

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Interstitial lung diseases(part2):

Interstitial lung diseases(part2) Dr Neelam


Connective tissue diseases These are chronic inflammatory autoimmune diseases. The may involve any tissue in any part of the body.


Conventionally they comprise rheumatoid arthritis,SLE,systemic sclerosis,polyarteritis nodosa and dermatomyositis/polymyositis.


SLE More common in females and in blacks Features include butterfly rash,arthralgia,Raynauds phenomenon,renal involvement and cns disease.


Lungs and pleura are involved in 50% cases. Pleuritic pain with a small pleural effusionis common manifestation and may occur bilaterally.


Movement of diaphragm is decreased secondary to pleurisy and may cause areas of atelectasis in lower lobes. This may produce bilateral,horizontal basal band shadows and elevation of diaphragm on chest radiograph.


Patchy consolidation may be seen sometimes with cavitation and is most often due to infection,pulmonary edema or pulmonary infarction.


Enlargement of cardiac shadow May be due to pericardial effusion,myocarditis or endocarditis and should be investigated by cardiac ultrasound.


Rheumatoid disease Lung involvement more often in males .


Presents as Pleural effusion ,,uni or bilateral,may resolve with fibrosis

A 52-year-old male with rheumatoid arthritis (RA): (a) PA chest radiograph shows a moderate right sided pleural effusion (arrow):

A 52-year-old male with rheumatoid arthritis (RA): (a) PA chest radiograph shows a moderate right sided pleural effusion (arrow)


2)Rheumatoid pulmonary nodules these are uncommon but are characteristic of this condition They are usually associates with subcutaneous nodules They are well defined round opacities upto 7cm in diameter,may be single or multiple and may cavitate. In caplans syndrome pneumoconiosis with rheumatoid nodules .They may resemble mets.The solid fibrotic lesions eventually may calcify

caplan syndome:

caplan syndome


Posteroanterior radiograph shows a multitude of fairly well-circumscribed nodules and masses ranging in diameter from 1 to 5 cm, scattered randomly throughout both lungs with no notable anatomic predilection. No cavitation is apparent, and there is no evidence of calcification. This patient, a 56-year-old man, had been a coal miner for many years and in recent years had developed arthralgia, which proved to be due to rheumatoid arthritis. As a means of establishing the nature of the pulmonary nodules, a percutaneous needle aspiration was performed on the large mass situated in the lower portion of the left lung (arrowheads): Several milliliters of inky black fluid were aspirated.


3)Fibrosing alveolitis Reticulonodular shadowing may progress to honeycombing and severe volume loss..Obliterative bronchiolitis is a potential cause of respiratory failure and chest radiograph may be normal although evidence of small airway disease may be evident on HRCT. Progressive upper lobe fibrosis with bullous cystic changes seen


CT lung demonstrate changes of advance rheumatoid lung disease which include interstitial fibrotic changes, honeycombing and traction bronchiectasis with bibasilar and peripheral distribution.


Systemic sclerosis Highest incidence of fibrosis among connective tissue diseases. Pulmonary arterial hypertension may be seen with or without fibrosis




Basal reticulonodular shadowing with progressive pulmonary volume loss.Eventually honey comb change may develop. Eggshell lymphnode calcification is a recognized feature.Increase prevalence of lung cancer.


Esophageal involvement result in abnormal motility may cause reflux and aspiration Occasionally a dilated esophagus sometimes demonstrating an air fluid level is visible on chest radiograph


HRCT of the chest demonstrates characteristic changes of pulmonary fibrosis, particularly in the bases and in the subpleural lung. Honeycomb change, intralobular septal thickening and traction bronchiectasis are all present. No pleural effusion or obvious nodal enlargement is visible. The oesophagus is dilated and air filled.


Dermatomyositis. . .basal fibrosing alveolitis and involvement of pharangeal muscles. Ankylosing spondylityis 1 -2 % cases...bilateral apical pleural thickening,upper lobe fibrosis,Aspergillus colonize Sjogern syndrome. ..dry moth and eyes...pleural effusion,fibrosing alveolitis,recurrent chest infections and lymphocytic interstitial pneumonia,,no characteristic pattern


Cystic lung disease Lung cysts are defined as radiolucent areas with a wall thickness of less than 4mm. Cystic lung diseases as listed in the table on the left. Cavities are defined as radiolucent areas with a wall thickness of more than 4mm and are seen in infection (TB, Staph, fungal, hydatid), septic emboli, squamous cell carcinoma and Wegener's disease.


Langerhans cell histiocytosis (LCH) is an idiopathic disease characterized in its early stages by granulomatous nodules containing Langerhans histiocytes and eosinophils. In its later stages, the granulomas are replaced by fibrosis and the formation of cysts. It is an uncommon condition. The majority of patients are young or middle-aged adults presenting with nonspecific symptoms of cough and dyspnea. Up to 20% of patients present with pneumothorax and over 90% of patients are smokers. Most cysts appear round, but can also have bizarre shapes (bilobed or clover-leaf shaped). An upper lobe predominance in the size and number of cysts is common.


case with multiple round and bizarre shaped cysts. There was an upper lobe predominance. The patient had a long history of smoking. This combination of findings is typical for Langerhans cell histiocytosis.


Lymphangiomyomatosis is a rare disease characterized by progressive proliferation of spindle cells, resembling smooth muscle. Proliferation of these cells along the bronchioles leads to air trapping and the development of thin-walled lung cysts. Rupture of these cysts can result in pneumothorax. Other features of LAM include adenopathy and pleural effusion. Lymphangiomyomatosis occurs only in women, usually of child-bearing age, between 17 and 50 years. Identical clinical, radiologic, and pathologic pulmonary changes are seen in about 1% of patients with tuberous sclerosis. Most patients die within 10 years of the onset of symptoms.


case with multiple cysts that are evenly distributed througout the lung ( in contrast to LCH).Notice the pneumothorax.There was no history of smoking and this was a 40 year old female. This combination of findings is typical for Lymphangiomyomatosis (LAM).




Goodpasture's syndrome. (A) Computed tomographic (CT) image at the level of the aortic arch shows centrilobular ground-glass opacities throughout the upper lobes due to diffuse pulmonary hemorrhage.


An abnormal chest x-ray shows abnormal tissue (arrows) in both lungs (L), predominantly in the middle and lower areas. This finding is typical of Goodpasture's syndrome. A normal chest x-ray is shown on the right for comparison; the heart (H), lungs (L), vertebrae (v), and collarbone (C) can be seen.


Goodpasture syndrome in 41-year-old man. Lung window of thin-section CT scan at level of main bronchi shows diffuse GGO in both lungs representing DAH. (Image courtesy of Nestor L. Muller, MD, PhD.)


PAP in a 61-year-old man with chronic myelogenous leukemia and recent onset of fatigue and cough. Posteroanterior chest radiograph shows symmetric, perihilar ground-glass and reticulonodular opacities. Note the relative sparing of the costophrenic angles.


PAP in a 17-year-old boy with mild cough and dyspnea that had persisted for several years. Chest radiograph shows dense bilateral consolidation with relative sparing of the apices and right costophrenic angle.


PAP in a 36-year-old man with a history of inhalational exposure to beryllium. Chest radiograph shows symmetric perihilar consolidation with sparing of the costophrenic angles and apices.


PAP in a 50-year-old man with severe dyspnea. CT scan (lung window) acquired through the upper lobes demonstrates widespread ground-glass opacity with focal areas of sparing and strikingly prominent septal lines (crazy-paving pattern).


Crazy-paving in PAP. (a) Coronal reformatted image (lung window) obtained in a 35-year-old man shows geographic areas of ground-glass opacity and septal thickening in an asymmetric distribution


PAP in a 50-year-old man. (a) CT scan (lung window) obtained prior to therapeutic BAL shows patchy areas of ground-glass opacity, thickened septal lines, and consolidation.


Post-BAL CT scan (lung window) obtained at the same level reveals residual septal lines but near-complete resolution of the ground-glass opacity.


D ifferential diagnosis of crazy-paving is broad and includes left heart failure, pneumonia (especially pneumocystis pneumonia), alveolar hemorrhage, bronchoalveolar carcinoma, lymphangitic carcinomatosis, diffuse alveolar damage (adult respiratory distress syndrome), radiation- or drug-induced pneumonitis, hypersensitivity pneumonitis, and pulmonary veno-occlusive disease


Chronic eosinophilic pneumonia is an idiopathic condition characterized by filling of the alveoli with eosinophils. It is associated with an increased number of eosinophils in the peripheral blood and patients present with fever, cough, weight loss, malaise, and shortness of breath. The symptoms are often severe and last three months or more. Patients respond promptly to treatment with steroids.


contrast enhanced CT in a patient with chronic eosinophilic pneumonia. Notice peripheral distribution of the consolidations.




61-year-old man with interstitial fibrosis; patient was receiving chlorambucil for chronic lymphocytic leukemia. High-resolution CT scan shows irregular linear opacities and ground-glass opacities in predominantly subpleural distribution. Differential diagnosis includes drug toxicity, opportunistic infection, and leukemic opacities. Diagnosis was confirmed at lung biopsy. Read More:


47-year-old woman with hypersensitivity reaction; patient was receiving bleomycin for Hodgkin's disease. High-resolution CT scan of chest shows extensive bilateral ground-glass opacities and poorly defined centrilobular nodules (arrows). Primary diagnostic considerations are drug toxicity, opportunistic infection, and pulmonary hemorrhage. Diagnosis was confirmed at open lung biopsy. Read More:


57-year-old man with drug-induced adult respiratory distress syndrome; patient was receiving bleomycin for non—Hodgkin's lymphoma. High-resolution CT scan reveals extensive bilateral ground-glass opacities primarily involving dependent lung regions. Differential diagnosis includes opportunistic infection, drug toxicity, and pulmonary hemorrhage. Diagnosis was confirmed at open lung biopsy. Read More:


58-year-old man with bronchiolitis obliterans organizing pneumonia; patient was receiving busulfan and cyclophosphamide chemotherapy for multiple myeloma. High-resolution CT scan shows peripheral areas of consolidation. Note striking left-sided predominance. Differential diagnosis includes bacterial or fungal pneumonia and adverse drug reaction. Diagnosis was confirmed at lung biopsy. Read More:


60-year-old man with bronchiolitis obliterans organizing pneumonia; patient was receiving amiodarone for ischemic heart disease. High-resolution CT scan of right lung shows irregular linear opacities, ground-glass opacities, and focal areas of consolidation. Differential diagnosis includes pneumonia, adverse drug reaction, and pulmonary edema. Diagnosis was confirmed at lung biopsy. Read More:

Amiodarone is an iodine-containing compound; therefore, parenchymal lesions often show high attenuation, with a range from 82 to 174 H [6] (Fig. 7A,7B). Although this finding is helpful in suggesting amiodarone-induced pulmonary toxicity, it is not pathognomonic. Read More:

Amiodarone is an iodine-containing compound; therefore, parenchymal lesions often show high attenuation, with a range from 82 to 174 H [6] (Fig. 7A,7B). Although this finding is helpful in suggesting amiodarone-induced pulmonary toxicity, it is not pathognomonic. Read More: 77-year-old man with bronchiolitis obliterans organizing pneumonia; patient was receiving amiodarone for treatment of cardiac arrhythmia. High-resolution CT scan shows extensive bilateral ground-glass opacities and dependent areas of consolidation. Diagnostic considerations include pneumonia, pulmonary edema, and adverse drug reaction. Diagnosis was confirmed at lung biopsy. Read More:


74-year-old woman with interstitial fibrosis; patient was receiving nitrofurantoin for recurrent urinary tract infections. High-resolution CT scan shows predominantly basal subpleural reticular and ground-glass opacities. Differential diagnosis includes adverse drug reaction, unrelated interstitial lung disease, and pneumonia. Diagnosis was confirmed at open lung biopsy. Read More:


52-year-old man with hypersensitivity reaction; patient was receiving methotrexate for rheumatoid arthritis. High-resolution CT scan shows poorly defined centrilobular nodules (arrows) and extensive areas of ground-glass attenuation. Differential diagnosis includes interstitial pneumonitis related to rheumatoid arthritis, opportunistic infection, and adverse drug reaction. Diagnosis was confirmed at open lung biopsy. Read More:


27-year-old female IV drug abuser with talcosis. High-resolution CT scan shows magnified view of left lung. Conglomerate mass is seen on background of fine micronodular interstitial pattern. Read More:


27-year-old female IV drug abuser with talcosis. Same image as A obtained with mediastinal windows confirms high attenuation of consolidative mass caused by talc accumulation. Findings are virtually diagnostic of talcosis. Read More:




Posteroanterior radiograph of the chest shows dense branching opacities and numerous basal-predominant calcified pulmonary nodules (arrows).


Transverse thin-section CT images through the chest obtained with (a–c) lung and (d–f) soft-tissue window settings show multiple dense nodules with calcification. Nodules are most numerous in the lung bases and include large lobulated subpleural calcifications (white arrows). There are also basal cysts, and many of the calcified nodules are associated within cyst walls or located within cysts (black arrows). In addition, there are irregular calcified linear opacities, as seen in Figure 1. There are no thickened interlobular septa. Mild centrilobular emphysema is also present.












Nodular parenchymal amyloidosis. CT scan of a patient showing multiple, bilateral, subpleural soft tissue densities, histological analysis of which confirmed the presence of amyloid.


Systemic AL amyloidosis with diffuse alveolar septal parenchymal lung involvement. Digital chest radiograph of a patient who presented with dyspnoea and cough showing diffuse interstitial shadowing causing loss of definition of the cardiac borders. A bronchoscopic biopsy specimen revealed amyloid and bone marrow examination revealed a monoclonal lymphoplasmacytoid population.


thoracic CT scan of a patient who presented with generalised lymphadenopathy and progressive dyspnoea revealing paratracheal and hilar lymphadenopathy associated with diffuse lung infiltration and pleural effusions. Histological examination of the lymph nodes confirmed AL amyloidosis.


Axial CT scan of the chest, lung and mediastinal windows, showing multiple calcified pulmonary nodules and masses secondary to Amyloidosis




UIP Usual Interstitial Pneumonitis (UIP) is a histologic diagnosis. UIP has distinctive HRCT findings and is usually shown at lungbiopsy, when honeycombing is visible. If the UIP pattern is of unknown cause (i.e. idiopathic), the disease is called Idiopathic pulmonary fibrosis (IPF). IPF accounts for more than 60% of the cases of UIP. In the presence of a surgical biopsy showing a UIP pattern the diagnosis of IPF requires exclusion of other known causes of UIP including drug toxicities, environmental exposures (asbest), and collagen vascular diseases like RA, SLE, polyarteritis nodosa and sclerodermia. A long list of drugs have been implicated, but this pattern is most commonly the result of cytotoxic chemotherapeutic agents such as bleomycin, busulfan, vincristine, methotrexate, adriamycin, and carmustine (BCNU). The differentiation between NSIP and UIP has tremendous prognostic implication for the patient. UIP is more progressive and more than 50% of patients with UIP die within 3 years.


chest film of a patient with UIP due to IPF. The findings on the chest film comprise volume loss and fibrotic changes in the basal lung area. The radiographic appearance of honeycombing comprises reticular densities caused by the thick walls of the cysts. Whenever you see a chest film with long standing reticulation with a lower lobe and peripheral preference also think 'UIP'.


HRCT findings in UIP Honeycombing consisting of multilayered thick-walled cysts. Architectural distortion with traction bronchiectasis due to fibrosis. Predominance in basal and subpleural region. Mild mediastinal lymphadenopathy




HRCT of advanced stage of pulmonary fibrosis demonstrating reticular opacities with honeycombing, with predominant subpleural distribution.


Bilateral lower lobe opacities and possible mild decrease in lung volumes. Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM.


High-resolution CT (HRCT) shows increased pulmonary attenuation with distortion of the pulmonary architecture. Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM.


High-resolution CT (HRCT) shows distortion of the pulmonary architecture with thickening of pulmonary interstitium and some areas of ground-glass attenuation. No obvious honeycombing is present. Courtesy of Sat Sharma, MD, FRCPC, FACP, FCCP, DABSM

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