Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Year Level 7 [Pulmonary Module] INTERSTITIAL LUNG DISEASE Hilario M. Tamondong Jr. M.D. OUTLINE: I. Objectives II. Interactive Activity III. Pneumoconioses A. Mineral Dust B. Headings C. Bulleting & Indentation D. Others IV. Schedule & Deadlines V. General Rules I. OBJECTIVES To present the clinical manifestations, pathology and radiologic features of patients with Environmental Lung. Disease/s Idiopathic Interstitial Pneumonia ______________________________________________________ II. INTERACTIVE ACTIVITY A. Clinical History General data o 59 year old male o Filipino o Roman Catholic o Retired Seaman o Married o Imus, Cavite City Chief complaint o Dyspnea of >4 months HPI o 4 months PTA recurrent exertional dyspnea (mildmoderate activities) non-productive cough (-) fever (-) failure symptoms, orthopnea, paroxysmal nocturnal dyspnea o 2 months PTA progressive dyspnea whitish productive cough intermittent undocumented fever admitted in a local hosital chest xray: unrecalled/ not available chest ct scan: Non-specific pneumonia; T/C PTB therapeutics unrecalled IV antibiotics anti TB medicines AFB smear not done Discharged on the 3rd day o 1 week PTA progressive dyspnea even at rest persistence of whitish productive cough no fever prompted consult admission Review of systems Team 8 | Ann, Kriska, Francine, Greg, Jet, Kai, Archee July 30, 2010 B. C. o (+) dyspnea at rest o (+) whitish cough o (+) easy fatigability o (+) orthopnea o (+) weight loss o (-) hemoptysis o (-) cyanosis o (-) joint pains o (-) skin rashes Past health history o Unremarkable Family history o HPN on father’s side Social history o 40 pack pear smoker o heavy alcohol drinker Occupational/ environmental history o fisherman o seaman, retired Physical Examination General o Conscious, coherent, in respiratory distress o BP: 100/60 o HR: 120 o RR: 28 o T: 36C o O2sat 87% Room Air o Wt: 60 kg o Ht: 157 cm o BMI: 24 Head/ Neck o anicteric sclerae, pink palpebral conjunctivae, supple neck, (-) CLAD, (-) NVE, (-) bruit Cardiovascular o adynamic precordium, tachycardic, regular rhythm, PMI= AB at 5th ICS LMCL, no murmur Chest/ Lungs o SCE, (+) supraclavicular retractions, decreased breath sounds LLF, increased vocal and tactile fremitus LLF, (+) crackles on both lung fields, no wheezes Abdomen o Flabby, NABS, soft, non-tender, no organomegaly Extremites o Grossly normal extremities, no clubbing, no edema, no bruises, no cyanosis Salient Features 59 year old male, retired seaman chronic/ progressive dyspnea easy fatigability at rest Page 1of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 D. E. F. chronic non-productive -> productive cough weight loss, orthopnea 40 pack year smoker Hospital admission (2months PTA) due to pneumonia PE findings of respiratory distress; (+) supraclavicular retractions, decreased breath sounds LLF, increased vocal and tactile fremitus LLF, (+) crackles on both lung, no wheezes Admitting Diagnosis Primary diagnosis/ initial impression o Chronic Obstructive Pulmonary disease (COPD), Emphysema Differential diagnoses o pneumonia o Pulmonary TB o Lung cancer o Bronchial Asthma o Bronchiectasis o Congestive heart failure o Interstitial lung disease Problems Chronic progressive dyspnea Easy fatigability Chronic cough 1st Hospital Day S> dyspnea at rest Chronic whitish productive cough Intermittent fever Result Hgb 209 Hct 0.63 WBC 9.7 Seg. 0.84 Lymph 0.16 PC 309 G. Team # 127 Na 136 K 3.72 ABG Result pH 7.43 PO2 60.4 PCO2 31.2 HCO3 20.2 BE -2.9 O2sat 92.2 O2 2 lpm O> coherent, in mild respiratory distress BP: 110/60 CR: 125 RR: 26 T: 36 C/L: SCE, supraclavicular retractions, crackles BLF Test Crea CXR: patchy disseminated multilobar consolidative alveolo-interstitial infiltrates, extensive left. ECG: Sinus tachycardia, nsstwc Sputum GS/CS Sputum AFB 3x, BCS x 2, PPD were requested Assessment o Non resolving pneumonia with consolidation o R/O pulmonary mass Therapeutics o Ceftazidime 1 gram IV q8 o Clindamycin 500 mg 1 cap qid o N-Acetylcysteine 600 mg tab od o Salburamol + Ipratropium neb q6 o O2 inhalation 4 lpm vnc o IVF D5NSS 1 L x 8 hours o Phlebotomy 500 cc 2nd- 5th Hospital Day S/O> o less dyspneic o can move with minimal distance o less productive cough C/L: SCE, no retractions, decrease crackles P> cont. antibiotics O2 to 2 LPM Page2of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 Test Result Sputum CS Moraxella Catarrhalis; Alpha Hemolytic Streptococci Blood CS NG x 4 days AFB smear (-) x3 PPD Negative H. 8th Hospital Day S> sudden progression of dypsnea at rest O> Coherent, in respiratory distress O2 sat 85% at 2 LPM C/L: supraclavicular retractions, increase crackles L>R No cyanosis CBC Result ABG Result Hgb 189 pH 7.429 Hct 0.68 PO2 63.8 WBC 7.4 PCO2 37.6 Seg 0.58 HCO3 24.3 Lymp 0.32 BE 0.3 PC 194 O2sat 93 O2 2 lpm Team # non resolving pneumonia o t/c bronchogenic carcinoma o interstital kung disease P> Diagnostic s o Sputum cytology o Fiberoptic Bronchoscopy (FOB) Therapeutics o O2 inhalation 4 LPM nasal prong; (O2 sat 90-93%) o Ceftazidime shifted to Ceftriaxone 1 gram IV q8 (culture guided) o Continue nebulization q4 Figure 1. Chest HRCT: Nonspecific multi segmental/ lobar interstitial disease. Mild centrilobular emphysema. Old nodal TB I. Salient Features Persistent hypoxemia Erythrocytosis Radiographic findings of persistent/ progressive interstitial infiltrates. Chest HRCT: Nonspecific multi segmental/ lobar interstitial disease. J. Final Diagnosis Non-resolving pneumonia o t/c interstitial Lung disease Polycythemia Vera Secondary to Chronic Hypoxemia Chronic Obstructive Pulmonary Disease Always start with the duration of symptoms! There is a need to quantify the level of exertion: mild to moderate There is a need to determine whether previous intervention was enough Review of systems: do it from head to toe! Smoking status: compute for the pack years to know its significance ILD is a rare condition, you usually do not consider it as a first impression! Case: o Primary impression is COPD, particularly emphysema because of his 40 pack year smoking history and easy fatigability o Differentials: Pulmonary TB is always a differential for a Filipino with chronic cough Bronchiectasis can cause crackles Asthma has an adult-onset variant Congestive heart failure should be considered Interstitial lung disease Page3of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 o Lab results show markedly increased hemoglobin and hematocrit Polycythemia vera secondary to chronic hypoxemia o Assessment at this point Non-resolving pneumonia with consolidation to rule out pulmonary mass o 8th hospital day Improved infiltrated in the upper left lung field o High-resolution CT scan Non-specific multi-segmental interstitial space, almost like honey combing! Note that honeycombing implies cystic lesions and are associated with poorer prognosis giving the impression that it might be ILD! ______________________________________________________ III. INTERSTITIAL LUNG DISEASE B. Figure 2. Diffuse Parenchymal Lung Disease. A. Team # Estimated Relative Frequency of Interstitial Lung Diseases Major Categories of Alveolar and Interstitial Inflammatory Lung Diseases Lung response o alveolitis, interstitial inflammation and fibrosis o granulomatous Page4of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 C. Pathogenesis of Pulmonary Fibrosis Figure 3. Pathogenesis of Pulmonary Fibrosis. ILDs represent a large number of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between these structures, as well as the perivascular and lymphatic tissues. Team # Alveolar Membrane, barrier to diffusion of oxygen, consists of: o alveolar epithelial cells o fused alveolar and capillary basement membrane o capillary endothelial cells o The alveoli are composed of: type I or type II pneumocytes on one side and the endothelium on the other o We can also find the interstitial space (for gas exchange) where the pathology of Interstitial Lung Disease (ILD) normally takes place o The interstitium includes the space between the epithelial and the endothelial basement membranes and it is the primary site of injury in IIPs. o However, these disorders frequently affect not only the interstium but also the airspaces, peripheral airways, and the vessels along with their respective epithelial and endothelial linings. Page5of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 o o o o Team # Cellular bases for the pathogenesis of interstitial lung disease o Multiple microinjuries damage and activate alveolar epithelial cells (top left), which in turn induce an antifibrinolytic environment in the alveolar spaces, enhancing wound clot formation o Alveolar epithelial cells secrete growth factors and induce migration and proliferation of fibroblasts and differentiation into myofibroblasts (bottom left) o Subepithelial myofibroblasts and alveolar epithelial cells produce gelatinases that may increase basement membrane disruption and allow fibroblast–myofibroblast migration (bottom right) o Angiogenic factors induce neovascularization o Both intraalveolar and interstitial myofibroblasts secrete extracellular matrix proteins, mainly collagens o An imbalance between interstitial collagenases and tissue inhibitors of metalloproteinases provokes the progressive deposit of extracellular matrix (top right) o Signals responsible for myofibroblast apoptosis seem to be absent or delayed in usual interstitial pneumonia, increasing cell survival o Myofibroblasts produce angiotensinogen that as angiotensin II provokes alveolar epithelial cell death, further impairing reepithelialization Inflammation and Fibrosis o The initial insult is an injury to the epithelial surface causing inflammation in the air spaces and alveolar walls o If the disease becomes chronic, inflammation spreads to adjacent portions of the interstitium and vasculature and eventually causes interstitial fibrosis o The development of irreversible scarring (fibrosis) of alveolar walls, airways, or vasculature is the most feared outcome in all of these conditions because it is often progressive and leads to significant derangement of ventilatory function and gas exchange lungs are small ,heavy, variegated appearance as parenchyma replaced by pale fibrosis more marked in the lower lobes, subpleural regions and interlobular septa honeycombed spaces up to 1.5 cm in diameter, although most are less than 0.5 cm arteries have thick walls pleura is thin Granulomatous Lung Disease o This process is characterized by an accumulation of T lymphocytes, macrophages, and epithelioid cells organized into discrete structures (granulomas) in the lung parenchyma o The granulomatous lesions can progress to fibrosis o Many patients with granulomatous lung disease remain free of severe impairment of lung function, or, when symptomatic, they improve after treatment o The main differential diagnosis is between sarcoidosis and hypersensitivity pneumonitis D. Diagnostic Process in DPLD E. History: Duration of Illness Acute o acute interstitial pneumonia (AIP) o eosinophilic pneumonia o hypersensitivity pneumonitis Subacute o sarcoidosis o drug induced ILDs o Cryptogenic Organizing pneumonia (COP) Page6of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 o F. Team # Acute Immunologic Pneumonia, SLE or Polymyositis Chronic o idiopathic Pulmonary Fibrosis (IPF) o Sarcoidosis o Pulmonary langerhans cell histiocytosis (PCLH) o Pneumoconiosis o Connective Tissue Disease Past History: Who are at Risk? Age o 20-40 y/o sarcoidosis ILD associaited with CTD Lymphanioleiomyomatosis Pulmonary Langerhans Histiocytosis (PLCH) Inherited forms of ILD Familial IPF Gaucher’s disease HermanskyPudlak syndrome o 50 y/o Gender o Female LAM Pulmonary involvement in tuberous sclerosis ILD in Hermansky-Pudlak syndrome CTDs o Male ILD in rheumatoid arthritis (RA) pneumoconioses Smoking History o PLCH o Desquamative interstitial pneumonia (DIP) o Goodpasture’s syndrome o Repiratory bronchiolitis o Pulmonary alveolar proteinosis Family history o Autosomal dominant Tuberous sclerosis Neurofibromatosis o Autosomal Recessive Niemann-Pick disease Gaucher's disease Hermansky-Pudlak syndrome Medications Agent Clinical Syndrome Bleomycin, Methotrexate Pulmonary fibrosis and HP Cyclophosphamide, Carmustine (BCNU), Azathioprine Pulmonary fibrosis Nitrofurantoin Acute Infiltrates with Eos – 1mo Chronic – 6 mos. to 2 y Amiodarone Pulmonary fibrosis Table 1. Drugs Associated with ILD Occupational history Environmental exposure Disease Farmer’s lung Antigen Faeni rectivirgula Humidifier lung Thermoactinomyces Bagassosis Thermoactinomyces vulgaris Avian droppings, feathers, serum Alternaria sp., wood dust Pigeon breeder’s lung Woodworkers lung Source Moldy hay, grain, silage Contaminated water reservoirs Moldy sugarcane Parakeets, pigeons, chickens, turkeys Oak, cedar, mahogany dust, pine and spruce pulp Table 2. Environmental/ Occupational Exposures G. General Pulmonary Symptoms progressive dyspnea- over months to years cough- nonproductive fatigue and weight loss Pleuritic chest pain – rare Wheezing- hypersensitivity pneumonitis Hemoptysis – rare Other sign and symptoms: clubbing, joint swelling H. General Pulmonary Findings bibasilar end-inspiratory dry crackles (Velcro rales) resting tachypnea and tachycardia increase in pulmonic component of 2nd heart sound, tricuspid insufficiency peripheral edema- cor pulmonale digital clubbing cyanosis extra pulmonary findings Page7of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 2. High Resolution CT Scan (HRCT) Figure 4. Clinical Profile of LCP Patients Diagnosed with ILD 19982007 (Flaviano & Diaz). I. What are the Ways to Diagnose ILD 1. Chest Radiography reticular pattern nodular pattern o sarcoidosis o PLCH o Chronic hypersensitivity pneumonitis o Silicosis o Beryliosis o RA (necrobiotic nodular form) o Ankylosing spondylitis mixed pattern – most common pattern honeycombing- poor prognosis Figure 6. High Resolution CT Scan (HRCT) of ILD. HRCT has become an integral part of the evaluation of the patient with idiopathic interstitial pneumonia High-resolution computed tomography (HRCT) is superior to the plain chest x-ray for early detection and confirmation of suspected ILD Also, HRCT allows better assessment of the extent and distribution of disease, and it is especially useful in the investigation of patients with a normal chest radiograph. Coexisting disease is often best recognized on HRCT scanning, e.g., mediastinal adenopathy, carcinoma, or emphysema. In the appropriate clinical setting HRCT may be sufficiently characteristic to preclude the need for lung biopsy in IPF, sarcoidosis, hypersensitivity pneumonitis, asbestosis, lymphangitic carcinoma, and PLCH. When a lung biopsy is required, HRCT scanning is useful for determining the most appropriate area from which biopsy samples should be taken. Figure 5. Radiographic finding of honeycombing Team # Page8of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 5. Sarcoid Exercise induced hypoxemia common in ILD Serology ACE, Lysozyme Wegener’s Goodpasture’s c-ANCA, p-ANCA Anti-GBM Rheumatoid Rheumatoid factor Scleroderma SCL-70, anti-centromere Sjogren’s SS-A, SS-B Lupus ANA, DS-DNA, anti-histone Polymyositis Anti Jo-1 ILD SP-A, SP-B, MCP-1, KL-6 6. 3. 4. Team # Spirometry and Lung Volumes asses lung involvement severity obstructive vs. restrictive pattern most ILD, have a restrictive defect Reductions in TLC, FRC, RV Decrease in flow rates (FEV1 & FVC) DLCO reduction common but nonspecific ABG normal or may reveal hypoxemia respiratory alkalosis Co2 retention in end stage ILD Normal Po2 does not exclude ILD Lung Biopsy a. Surgical Lung Biopsy establish a specific diagnosis o with atypical signs/ symptoms o normal radiographic features exclude neoplastic/ infectious processes assess disease activity identify a more treatable process definitive diagnosis/ predict prognosis prior to initiation of therapy to establish firm clinicopathologic diagnosis, and allows patient and clinician to make more informed decisions about treatment option almost all of the current treatments for the IIPs have potentially serious risks and side effects, and it is not reasonable to expose patients to these risks in the presence of diagnostic uncertainty detection of fibrotic processes related to specific exposures can have important compensation implications for the patient, and important public health consequences for the community; for example, asbestosis to establish etiology and stage of ILD high diagnostic yield (>92%) Open thoracotomy b. VATS same specimen adequacy & diagnostic accuracy Page9of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 c. Neutrophils preferred method in part because several retrospective studies have found that morbidity is improved with VATS compared with open thoracotomy. FOB with Transbronchial Lung Biosy/ Bronchioalveolar Lavage (BAL) initial procedure of choice BAL for malignancy or opportunistic infection Installation of saline into pathologic areas and retrieval of fluid for analysis Interpretation in context of clinical/HRCT findings BAL cellular counts with no prognostic significance Cellular profiles specific ILDs Eosinophils Table 4. Lung conditions and their respective bronchoalveolar lavage findings. Lymphocytes Infection Eosinophilic PNA Sarcoidosis ARDS Drug-induced ILD Hypersensitivity pneumonitis AIP Churg-Strauss COP Hypereosinophilic Radiation syndrome pneumonitis DIP Tropical Eosinophilia Drug-induced ILD Rheumatologic lung disease Figure 8. ATS/ ERS Criteria for Diagnosis of Idiopathic Pulmonary Fbrosis in Absence of Surgical Lung Biopsy Table 3. BAL WBC Differential Profiles in ILD J. Figure 7. Probability of Diagnosis Diffuse Diseases Team # Treatment Remove offending agent Supportive management Drug therapy o Glucocorticoids mainstay of therapy for suppression of alveolitis in organic dust disease starting dose of prednisone 0.5–1 mg/kg in a once-daily oral dose (based on the patient's lean body weight) x 4-12 weeks Maintenance dose 0.250.5 mg/kg x 4-12 weeks o Cyclophosphamide and Azathioprine (1–2 mg/kg lean body weight per day), with or without glucocorticoids o Methotrexate o Colchicine o Penicillamine Page10of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 o K. Cyclosporine ILD Associated with Connective Tissue Disorders 1. Progressive Systemic Sclerosis (PSS) Clinical evidence of ILD is present in about one-half of patients with PSS, and pathologic evidence in three-quarters Pulmonary function tests show a restrictive pattern and impaired diffusing capacity Pulmonary vascular disease alone or in association with pulmonary fibrosis, pleuritis, or recurrent aspiration pneumonitis is strikingly resistant to current modes of therapy. 2. Rheumatoid Arthritis (RA) more common in men Pulmonary manifestations include pleurisy with or without effusion ILD in up to 20% of cases o necrobiotic nodules (nonpneumoconiotic intrapulmonary rheumatoid nodules) with or without cavities o Caplan's syndrome (rheumatoid pneumoconiosis) o pulmonary hypertension secondary to rheumatoid pulmonary vasculitis o BOOP o upper airway obstruction due to cricoarytenoid arthritis. 3. Systemic Lupus Erythematosus (SLE) Pleuritis with or without effusion is the most common pulmonary manifestation Other lung manifestations include the following: atelectasis, diaphragmatic dysfunction with loss of lung volumes, pulmonary vascular disease, pulmonary Team # hemorrhage, uremic pulmonary edema, infectious pneumonia, and BOOP Acute lupus pneumonitis characterized by pulmonary capillaritis leading to alveolar hemorrhage is uncommon Chronic, progressive ILD is uncommon. It is important to exclude pulmonary infection. Although pleuropulmonary involvement may not be evident clinically, pulmonary function testing, particularly DLCO, reveals abnormalities in many patients with SLE. 4. Polymyositis and Dermatomyositis (PM/DM) ILD occurs in ~10% of patients with PM/DM. Diffuse reticular or nodular opacities with or without an alveolar component occur radiographically, with a predilection for the lung bases. Weakness of respiratory muscles contributing to aspiration pneumonia may be present. A rapidly progressive illness characterized by diffuse alveolar damage may cause respiratory failure. 5. Sjogren’s Syndrome General dryness and lack of airways secretion cause the major problems of hoarseness, cough, and bronchitis. Lymphocytic interstitial pneumonitis, lymphoma, pseudolymphoma, bronchiolitis, and bronchiolitis obliterans are associated with this condition. Lung biopsy is frequently required to establish a precise pulmonary diagnosis. Glucocorticoids have been used in the management of ILD associated with Sjögren's syndrome with some degree of clinical success ILD is a histopathologic diagnosis! Diffuse parenchymal diseases manifest with same symptoms, differentiated only by histopathology. ILD classification is difficult to make Page11of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 Note that idiopathic interstitial pneumonias pose a 40% risk. ILD involves lung parenchyma and not airways. Recall that Type II epithelium produced surfactant. ILD involves an autoimmune process resulting to fibrosis, causing stiffness of airways that lead to problems with respiration leading to hypoxemia. Note the drugs associated with ILD. Note that lymphangioleiomyomatosis (LAM) is only seen in women, usually of the young reproductive age, while most ILDs manifest in > 50 y.o. In a study conducted at the lung center, it was found that the most common presentation among Filipino patients is cough (96.30%) followed by difficulty of breathing (59.30%). Measurement of ILD presents as restrictive: Normal FEV1/FVC ratio, decreased FEV1, decreased FVC Serology is important with cases of probable known causes. Connective tissue diseases (CTD) are difficult to rule out, therefore consider them first. Most common form of CTD is SLE, which presents with pleuritis. Most common with granulomatous lung response is sarcoidosis. Most common with UNKNOWN etiology and vague presentation in Idiopathic Interstitial Lung Disease is Idiopathic Pulmonary Fibrosis (IPF) or Unspecified Idiopathic Pneumonia (UIP), which present with honeycombing and has a 50% 5-year mortality rate. ______________________________________________________ IV. IDIOPATHIC PULMONARY FIBROSIS INTERSTITIAL PNEUMONIA (UIP) (IPF)/ Figure 9. IPF/ UIP. Chest radiograph shows typical peripheral reticular opacity, most marked at the bases, with honeycombing. Lower lobe volume loss USUAL A. Clinical Manifestations exertional dyspnea Nonproductive cough Inspiratory crackles with or without digital clubbing may be present on physical examination. Figure 10. IPF/ UIP CT images show basal predominant peripheral predominant reticular abnormality with traction bronchiectasis and honeycombing. Team # Page12of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 Often mechanical ventilation is required but is usually not successful, with a hospital mortality rate of up to three-fourths of the patients. In those that survive, a recurrence of acute exacerbation is common and usually results in death at those times. Lung transplantation should be considered for those patients who experience progressive deterioration despite optimal medical management and who meet the established criteria ______________________________________________________ V. DESQUAMATIVE INTERSTITIAL PNEUMONIA (DIP) DIP is a rare but distinct clinical and pathologic entity found exclusively in cigarette smokers The peak incidence is in the fourth and fifth decades Most patients present with dyspnea and cough Figure 11. IPF/ UIP Pattern. Marked fibrosis consisting of dense collagenous scarring with remodeling of the lung architecture and cystic changes Figure 12. DIP HRCT shows peripheral predominant ground glass abnormality, with some associated cystic changes B. Clinical Course median length of survival from time of diagnosis varies between 2.5 and 3.5 yr (50% in 5 yr) periods of rapid decline that represents accelerated disease, intercurrent viral infection with the development of organizing pneumonia, or diffuse alveolar damage Improvement in lung physiology and radiologic abnormalities is rare C. Treatment no therapy has been found to be effective in the management of acute exacerbations of IPF Team # Page13of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 Figure 13. DIP Pattern. The alveolar spaces are diffusely involved by marked alveolar macrophage accumulation and there is mild interstitial thickening. DIP course o prognosis of DIP is generally good o most patients improve with smoking cessation and corticosteroids o The overall survival is about 70% after 10 yr ______________________________________________________ VI. RESPIRATORY BRONCHIOLITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE (RB-ILD) Figure 15. RB-ILD Pattern. Faintly pigmented alveolar macrophages fill the lumen of this respiratory bronchiole and the surrounding airspaces. There is mild thickening of the wall of the respiratory bronchiole. Many patients improve after cessation of smoking. Progression to dense pulmonary fibrosis has not been reported. As the number of patients studied to date is small, definitive reports of its natural history are not available. Survival: 5% in 5 yr ______________________________________________________ VII. ACUTE INTERSTITIAL PNEUMONIA (AIP, HAMMAN-RICH SYNDROME) Rare, fulminant form of lung injury Most patients are older than 40 years AIP is similar in presentation to the acute respiratory distress syndrome (ARDS) and probably corresponds to the subset of cases of idiopathic ARDS. The onset is usually abrupt in a previously healthy individual A prodromal illness, usually lasting 7–14 days before presentation, is common Fever, cough, and dyspnea are frequent manifestations at presentation. HRCT shows a geographic distribution of ground glass abnormality with consolidation Figure 14. RB- ILD HRCT lung scanning shows bronchial wall thickening, centrilobular nodules, ground-glass opacity, and emphysema with air trapping. Team # Page14of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 Figure 16. AIP Pattern: The lung shows diffuse alveolar wall thickening by proliferating connective tissue and inflammation There is no proven treatment mortality rates are high (>50-60%), most deaths occurring between 1 and 2 mo of illness onset. Survivors of AIP may experience recurrences and chronic, progressive interstitial lung disease ______________________________________________________ Figure 17. COP. Chest radiograph shows multifocal consolidation (arrows). VIII. CRYPTOGENIC ORGANIZING PNEUMONIA (COP) Clinicopathologic syndrome of unknown etiology The onset is usually in the fifth and sixth decades The presentation may be of a flulike illness with cough, fever, malaise, fatigue, and weight loss Inspiratory crackles are frequently present on examination Pulmonary function is usually impaired, with a restrictive defect and arterial hypoxemia being most common. Non-specific reaction to lung injury found adjacent to other pathologic processes or a component of other primary pulmonary disorders: o Cryptococcosis o Wegener's granulomatosis o Lymphoma o Hypersensitivity pneumonitis o Eosinophilic pneumonia Figure 18. COP. HRCT shows areas of air-space consolidation, ground-glass opacities, small nodular opacities, and bronchial wall thickening and dilation. These changes occur more frequently in the periphery of the lung and in the lower lung zone. Team # Page15of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 Figure 19. COP Pattern. Consists of polypoid plugs of loose organizing connective tissue within an alveolar duct and the adjacent alveolar spaces. F Figure 20. NSIP. HRCT shows bilateral, subpleural ground-glass opacities, often associated with lower lobe volume loss. Patchy areas of airspace consolidation and reticular abnormalities may be present, but honeycombing is unusual. Majority of patients recover completely on administration of oral corticosteroids A significant number relapse within 1 to 3 months when the corticosteroids are reduced or stopped. Prolonged treatment for 6 months or longer is advised. A small proportion of patients recovers spontaneously Death is rare ______________________________________________________ IX. NON-SPECIFIC INTERSTITIAL PNEUMONIA (NSIP) Idiopathic NSIP is a subacute restrictive process with a presentation similar to IPF but usually at a younger age, most commonly in women who have never smoked It is often associated with a febrile illness. Team # Figure 21. NSIP Pattern. The key histopathologic features of NSIP are the uniformity of interstitial involvement across the biopsy section, and this may be predominantly cellular or fibrosis. There is less temporal and spatial heterogeneity than in UIP and little or no honeycombing is found. Page16of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 The prognosis of NSIP is more variable than in IPF and appears to depend on the extent of fibrosis Some patients experience almost complete recovery, and most of the remainder stabilize or improve on treatment Relapse may occur. A minority of patients progress and die (10% in 5 yr) ______________________________________________________ X. LYMPHOCYTIC INTERSTITIAL PNEUMONIA (LIP) Figure 23. LIP Pattern. There is diffuse thickening of alveolar walls by a moderately severe infiltrate of lymphocytes and plasma cells. F Figure 22. LIP. HRCT shows diffuse ground glass attenuation with multiple lung cysts Team # Corticosteroids are the most widely used treatment, and are thought to arrest or improve symptoms in a large proportion of patients. More than one-third progress to diffuse fibrosis, and it is unclear whether treatment influences the course of the disease or has a significant effect on lung physiology. Occasional cases resolve or improve substantially. Note those that respond to therapy: o LIP Corticosteroids relieve symptoms o COP Unknown etiology Responds to corticosteroids Lung transplantation has not been done in the country as of date. DIP o Peak incidence is 4th to 5th decade o Prognosis is generally good IPF and DIP are associated with smoking! Role of pulmonologists in ILD is the provision of a high index of suspicion. AIP (Hamman-Rich Syndrome) presents like ARDS and with unspecific symptoms such as fever, cough and dyspnea NSIP o Subacute restrictive o Younger women who are never smokers o 10% mortality rate in 5 years o No honeycombing! Better prognosis! Page17of 18 INTERSTITIAL LUNG DISEASE Year Level 7 [Pulmonary Module]|July 30, 2010 XI. DPLD (OTHER FORMS) Pulmonary Langerhans Cell Histiocytosis (PLCH) Pulmonary Alveolar Proteinosis (PAP) Pulmonary Lymphangioleiomyomatosis Syndromes of ILD with Diffuse Alveolar Hemorrhage Goodpasture's Syndrome FOR THE EXAM, note the following: o Those that respond to therapy o Differences in histopathology o The most common ones mentioned o Those that are common with not the usual patients (old/ smokers) o Honeycombing= worse prognosis Team # Page18of 18