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V CHRONIC DIFFUSE INFILTRATIVE LUNG DISEASE
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Table 1 Common Causes of Hypersensitivity Pneumonitis. Disease Antigen Source Probable Antigen s, Thermophilic actinomycetes Micropolyspora faeni Thermoactino. Farmer s lung Moldy hay, myces vulgaris, Bagassosis Moldy pressed sugarcane Bagasse Thermophilic actinomycetes T sacchari T vulgaris. Multiple bird handler s diseases Bird droppings products and feathers Bird proteins. Laboratory worker s lung Rat fur Rat urine proteins. Diisocyanates and trimellitic, Chemical exposures Altered proteins. anhydrides, Contaminated humidifiers dehumidifiers Thermophilic actinomycetes T candidus T vulgaris Penicillium. Ventilator lung, air conditioners and heating systems species Amoeba species Klebsiella species and Candida species.
physical examination sents as a diffuse pulmonary disorder together with laboratory. The physical examination has less diagnostic utility than the evidence of diabetes insipidus. history but is important nonetheless Collagen vascular disorders Additional laboratory tests such as autoimmune serology and. may be suggested by detection of synovitis telangiectasia sclero determination of angiotensin converting enzyme ACE level. dactyly or a malar rash Sarcoidosis also involves extrapulmo may prove useful depending on the clinical and radiographic. nary organs and should be suspected if uveitis erythema nodosum features in a given case Antinuclear antibodies or rheumatoid. or plaquelike skin lesions consistent with cutaneous sarcoidosis factor is usually present in the serum of patients with collagen. are detected Malignant diffuse infiltrative lung disease i e lym vascular disorders but it is also found in low titer in up to 50 of. phangitic spread may be associated with findings relevant to the patients with IPF Increased serum levels of ACE are suggestive. primary tumor such as an abdominal or breast mass hepato of sarcoidosis but ACE can also be elevated in miliary tuberculo. megaly or guaiac positive stools Finally cardiovascular exami sis berylliosis asbestosis and silicosis Hypersensitivity pneumo. nation is very important because on occasion chronic pulmonary nitis is nearly always associated with the presence of serum anti. venous congestion resulting from occult mitral stenosis or left body against the offending antigen However the presence of. ventricular failure may present as diffuse infiltrative lung disease antibody against one of the causative agents of hypersensitivity. pneumonitis in serum does not prove that hypersensitivity pneu. laboratory examination monitis is the cause of diffuse infiltrative lung disease but only. The laboratory examination comprises studies that are more or that there has been sufficient exposure to the antigen to elicit an. less routine e g complete blood count biochemical screening immunologic response Resolution of pneumonitis with disap. and those that are more specialized Sarcoidosis may be suggest pearance of antibody after stopping the exposure provides a more. ed by cytopenias if the bone marrow is involved or by the pres conclusive association. ence of hypercalcemia or elevated serum liver enzyme levels. Collagen vascular disease and malignancy may also result in cy chest radiography. topenias Peripheral eosinophilia would suggest chronic eosino Radiographic features are for the most part nonspecific Dif. philic pneumonia Uncommonly eosinophilic granuloma pre fuse infiltrative lung disease may be characterized by bilaterally. Table 2 Drugs That Commonly Induce Chronic Parenchymal Lung Disease. Category Drugs Notes, Bleomycin Dose related worse with O2. Busulfan Induces pulmonary fibrosis with high mortality and occasionally alveolar proteinosis. Chemotherapeutic agents Cyclophosphamide Variable onset and course. Vinblastine Synergistic with mitomycin C, Nitrosoureas Dose related delayed onset. Amiodarone Long half life induces ARDS after procedures. Cardiovascular drugs, Hydralazine others Induce SLE. Methotrexate Produces granulomas as found on biopsy. Anti inflammatory drugs Penicillamine Induces Goodpasture like syndrome SLE bronchiolitis obliterans. Gold Injectable only induces BAL lymphocytosis, Nitrofurantoin Induces acute and chronic parenchymal lung disease. Antibiotics, Sulfasalazine BOOP PIE or pulmonary fibrosis.
Methylphenidate I V injection produces talc granulomas. Illicit drugs, Cocaine Induces BOOP alveolar hemorrhage. ARDS acute respiratory distress syndrome BAL bronchoalveolar lavage BOOP bronchiolitis obliterans organizing pneumonia PIE peripheral eosinophilia. 2001 WebMD Inc All rights reserved WebMD Scientific American Medicine. June 2001 Update RESPIRATORY MEDICINE V Chronic Diffuse Infiltrative Lung Disease 2. symmetrical interstitial alveolar or mixed alveolar interstitial ra a. diographic patterns In some cases the lung fields appear com. pletely normal on the chest radiograph despite the presence of. significant clinical and physiologic abnormalities There are cer. tain ancillary radiographic clues that if present may help in mak. ing the differential diagnosis and in narrowing the list of possible. causes see Table 3, high resolution computed tomography. The use of high resolution computed tomography HRCT. represents a significant advance in the evaluation of diffuse pa. renchymal lung disease With the use of HRCT the extent loca. tion and pattern of lung involvement can be determined with. great accuracy see Figure 1 HRCT can often detect abnormalities. in patients who have symptoms of interstitial lung disease but. whose chest radiographs are normal When combined with clini. cal data and chest radiography HRCT of the chest can lead to a. specific diagnosis in 60 to 80 of cases 6 When HRCT indicates. a specific diagnosis e g eosinophilic granuloma the need for a. lung biopsy is eliminated 5, pulmonary function testing. Pulmonary function testing provides diagnostic clues to the. presence of diffuse infiltrative lung disease and is useful during. the course of the disease The hallmarks of the disorder include a. restrictive ventilatory pattern reduced lung volume a normal or. increased ratio of forced expiratory volume in 1 second to forced. vital capacity FEV1 FVC a reduction in the diffusing capacity. of the lung for carbon monoxide Dlco and a reduction in arter. ial oxygen tension Pao2 associated with normal or reduced ar. terial carbon dioxide tension Paco2 In addition there is usually. significant exercise limitation resulting from a fall in Pao2 abnor. malities in respiratory mechanics associated pulmonary vascular. disease or a combination of these factors, bronchoalveolar lavage. In most cases the cause of lung disease remains uncertain de. spite careful clinical radiographic laboratory and physiologic. evaluation The next step is usually to perform bronchoscopy with Figure 1 High resolution computed tomography HRCT in. bronchoalveolar lavage BAL and transbronchial lung biopsy patients with chronic diffuse interstitial lung disease can occa. Certain causes can be diagnosed solely by BAL others only by bi sionally suggest a specific diagnosis a Posteroanterior radiog. raphy demonstrates a diffuse interstitial process but differen. opsy and some by either technique BAL is most useful for diag. tial diagnosis is lengthy b HRCT shows a combined, nosing infectious causes especially PCP The diagnostic sensitiv.
interstitial and cystic process that is virtually diagnostic of. ity of this procedure for AIDS related PCP is approximately 90 eosinophilic granuloma of the lung. to 95 Thus it is the procedure of choice for diagnosing the 20. to 50 of cases of AIDS related PCP that cannot be diagnosed. by induced sputum examination Other opportunistic infections. such as cytomegalovirus pneumonia and disseminated fungal or. Table 3 Radiographic Clues to Diagnosis tuberculous infection can also be diagnosed by BAL Noninfec. of Diffuse Infiltrative Lung Disease tious causes that can be diagnosed by this technique include alve. olar proteinosis lymphangitic carcinomatosis and alveolar cell. Associated carcinoma In addition BAL may provide helpful information by. Primary Diagnostic Considerations, Radiographic Finding. revealing one of the following changes 1 increased numbers of. Hilar adenopathy Sarcoidosis lymphoma carcinoma granulo eosinophils in chronic eosinophilic pneumonia 2 asbestos bod. matous infection ies in asbestosis 3 so called foamy cells with lamellar inclusions. Pleural effusion Collagen vascular disease asbestosis lymphan in amiodarone induced disease 4 hyperplastic and atypical type. giomyomatosis chylous tuberculosis, Pneumothorax Eosinophilic granuloma Pneumocystis carinii. II pneumocytes in cytotoxic drug induced lung injury 5 Langer. pneumonia lymphangiomyomatosis hans cells in eosinophilic granuloma and 6 a bloody effluent. Upper lung zone Silicosis eosinophilic granuloma sarcoidosis with abundant hemosiderin in alveolar macrophages in diffuse. predominance alveolar hemorrhage Quantitating the number and distribution. Peripheral Eosinophilic pneumonia bronchiolitis obliterans. predominance organizing pneumonia drug induced injury. of inflammatory cells e g macrophages lymphocytes and neu. trophils may suggest a specific diagnosis 7 The alveolar lavage. 2001 WebMD Inc All rights reserved WebMD Scientific American Medicine. June 2001 Update RESPIRATORY MEDICINE V Chronic Diffuse Infiltrative Lung Disease 3. case the impact that a more precise diagnosis would have on ther. Table 4 Diagnostic Efficacy of apy and an assessment of the risk of open lung biopsy in a given. case5 see Figure 2 The frequency with which open lung biopsy is. Transbronchial Lung Biopsy performed to better define the histopathologic features of nonin. Diseases Frequently Diagnosed by TLB Findings fectious forms of the disease varies greatly among different cen. Infectious diffuse infiltrative lung disease ters Use of video assisted thoracoscopy rather than open lung bi. Sarcoidosis opsy has not resulted in the anticipated decrease in morbidity. Lymphangitic carcinoma and cost 8, Alveolar cell carcinoma. Talc induced diffuse infiltrative lung disease treatment. Diseases Suggested by TLB Findings The treatment recommendations in this subsection are based. Cytotoxic induced disease on small nonrandomized series often without control groups. Bronchiolitis obliterans organizing pneumonia occasionally case reports and clinical experience There are very few random. Eosinophilic pneumonia, ized controlled trials of treatment for these diseases. Hypersensitivity pneumonitis, Even before a specific diagnosis is known preventive mea.
Not Diagnosable by TLB Findings sures can be initiated Immunization against pneumococcal anti. Idiopathic pulmonary fibrosis, gens every 5 years and yearly immunizations against influenza. Diseases commonly misdiagnosed as idiopathic pulmonary. fibrosis are indicated Patients who have a reversible obstructive defect. Eosinophilic granuloma may benefit from bronchodilators Supplemental oxygen given at. Lymphangiomyomatosis rest and with exercise will often improve patients tolerance of ac. Bronchiolitis obliterans organizing pneumonia tivities of daily living 9 Pulmonary hypertension and cor pul. Hypersensitivity pneumonitis monale occurring in patients with far advanced disease can be. Sarcoidosis occasional cases improved with appropriate treatment see 14 XI Pulmonary Hyper. Lymphangitic carcinoma occasional cases tension Cor Pulmonale and Primary Pulmonary Vascular Diseases. TLB transbronchial lung biopsy, Chronic Diffuse Infiltrative Lung Disease of Known. liquid of healthy nonsmokers typically contains 84 to 99 mac. drug induced disease, rophages 1 to 14 lymphocytes and 0 to 1 neutrophils. The common causes of nongranulomatous diffuse infiltrative Many different drugs have been reported to cause diffuse infil. lung disease e g IPF some of the collagen vascular disorders trative lung disease see Table 2 10 Estimates are that drug induced. and asbestosis are often characterized by a neutrophilic alveoli lung disease affects several hundred thousand patients each year. tis whereas sarcoidosis and hypersensitivity pneumonitis are as To minimize the morbidity and mortality of drug induced dis. sociated with increases in lymphocytes However overlap exists ease early recognition is critical Discontinuance of the offending. e g IPF with an increased number of lymphocytes or sarcoido agent is often followed by spontaneous improvement whereas. sis with a normal number of lymphocytes failure to appreciate the causal relation between the drug and the. pulmonary disease can lead to irreversible lung injury Unfortu. lung biopsy nately certain aspects of drug induced disease can hinder the. In many cases the diagnosis remains unknown until a lung recognition of this cause and effect relation First the drugs that. biopsy is obtained Pathologically these disorders may be charac cause diffuse infiltrative lung disease are numerous and drug in. terized by variable degrees of involvement of alveolar septa or duced lung disease usually occurs in only a small fraction of pa. alveoli by inflammatory cells mesenchymal cells fibrosis granu tients who receive such drugs Second the onset of the pulmo. loma or neoplastic cells In rare instances the abnormal substance nary disease may occur weeks to months after the drug is begun. that accumulates in the lung parenchyma is blood proteinaceous In the case of cytotoxic drug induced disease the on. symmetrical interstitial alveolar or mixed alveolar interstitial ra diographic patterns In some cases the lung fields appear com pletely normal on the chest

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