Interstitial Lung Disease

-
Differential Diagnosis
Primary (idiopathic) usual interstitial pneumonia (UIP), respiratory bronchiolitis associated inter stitial lung disease (RBILD), desquamative intersti tial pneumonia (DIP), acute interstitial pneumonia (AIP), non specific interstitial pneumonia (NSIP), lymphoid interstitial pneumonia (LIP), cryptogenic organizing pneumonia (COP)
Secondary
Drugs chemotherapy (bleomycin), sulfa, penicillin, sulfonylurea, gold, penicillamine, phenytoin, amiodarone, nitrofurantoin
Infiltrative lymphangitic carcinomatosis, sarcoidosis
Infections TB, histoplasmosis,coccidioidomycosis
Inflammatory rheumatoid arthritis, SLE, sclero derma, ankylosing spondylitis, myositis
Congestive Heart Failure
Environment organic dust (hypersensitivity pneumonitis), inorganic dust(asbestos, silica, beryllium, coal worker’s pneumoconiosis)
Eosinophilia-associated  Pulmonary infiltrates allergic bronchopulmonary aspergillosis (ABPA), parasitic, drugs
ETC pulmonary histiocytosis X, idiopathic pulmonary hemosiderosis, lymphangioleiomyomatosis, radiation
Clinical Features
History dyspnea (duration, progression), cough, hemoptysis, wheezes, chest pain, impaired exercise tolerance, occupational history (details of all previous jobs, exposure to gases or chemicals particularly important), environmental exposure (home setting, air con ditioning, pets, hobbies), rash, joint swelling, past medical history (smoking), medications, family history
Physical vitals (tachypnea, hypoxemia), cyanosis, clubbing (idiopathic pulmonary fibrosis, asbestosis, rheumatoid lung, fibrosing NSIP), decreased chest expansion, crackles (fine), wheezes, cor pulmonale. Note that sarcoidosis and silicosis may have a normal lung examination
Investigations
Basic
Labs CBCD, ANA, RF, anti CCP antibody, anti SCL antibody, anticentromere antibody, anti Jo antibody
Imaging CXR, CT chest (high resolution), echocardiogram (if suspect pulmonary hypertension)
ABG
PFT
Special
Biopsy bronchoscopy (transbronchial biopsy), open lung biopsy

Diagnostic Issues
Characteristic Chest X-ray Patterns for Interstitial Lung diseases
Upper lobe Predominance sarcoidosis, hypersensitivity pneumonitis, pneumoconiosis, silicosis, histiocytosis X, PJP, ankylosing spondylitis, ABPA, TB
Lower lobe Predominance idiopathic pulmonary fibrosis, asbestosis, rheumatoid arthritis, scleroderma, drugs
Bilateral Hilar/Mediastinal Adenopathy with Interstitial Infiltrates sarcoidosis, berylliosis, lymphangitic carcinomatosis, TB, fungal, lymphoma
Eggshell Calcification of Hilar/Medistinal Lymph nodes, fungal
Calcified Pleural Plaques asbestos
Pleural Effusion with Interstitial infiltrates HF, lymphangitic carcinomatosis, rheumatoid arthritis, SLE
Management
Treatment steroids in most cases. Idiopathic pulmonary fibrosis (steroids plus either azathioprine or cyclophosphamide)
Lung Transplant
Specific Entities
Idiopathic Pulmonary Fibrosis (IPF), also knows as usual interstitial Pneumonia (UIP)
Pathophysiology unknown, Fibrotic rather than inflammatory process
Diagnosis CT chest (honeycombing, interlobular septal thickening, traction bronchiectasis, periph eral, sub pleural, lack of ground glass pattern), bronchoscopy (to rule out other causes, mostly infectious); consider open lung biopsy if CT is not consistent with above
Treatments steroid monotherapy usually ineffective. For patients
Hypersentivity Pneumonitis
Pathophysiology inhaled organic antigens ͢  immune response ͢  acute, subacute, or chronic granulomatous pneumonia
Diagnopsis major criteria (compatible symptoms, antigen exposure, imaging findings, lavagelymphocytosis, histologic findings (poorly formed granulomas), reexposure triggers symptoms); minor criteria (bilateral crackles decreased DLCO, hypox emia). Combination of major and minor criteria will help raise suspicion of hypersensitivity pneumoni tis. Serology may be helpful
Treatments cessation of exposure, steroids
Cryptogenic organizing Pneumonia (COP)
Previously known as bronchiolitis obliterans organizing pneumonia (BOOP)
Causes idiopathic (80%), post infectious (CMV, influenza, adenovirus, Chlamydia), drugs (amiodarone, bleomycin, gold, sulfasalazine, cephalosporin, cocaine), connective tissue dis ease (RA, SLE, scleroderma, Sjogren’s, dermato myositis), immunologic (essential mixed cryoglobulinemia), transplantation (bone marrow, lung, kidney), malignancy (MDS, lymphoproliferative diseases, radiation)
Clinical Features about 50% of cases preceded by viral like respiratory infection. Symptoms
include dyspnea on exertion, persistent non productive cough, and weight loss
Diagnosis characteristic findings on CXR and CT chest include bilateral, diffuse, ill defined alveolar opacities distributed peripherally. PFT shows mainly restrictive lung disease pattern

Treatments prednisone 1 mg/kg PO daily

Comments

Post a Comment

Popular posts from this blog

Pleural Effusion

-
Image
Pleural effusion , sometimes referred to as “water on the lungs,” is the build-up of excess  fluid  between the layers of the pleura  outside the lungs. The  pleura  are thin membranes that line the lungs and the inside of the chest cavity and act to lubricate and facilitate breathing. Differential Diagnosis Exudative malignancy, infections, connective tissue disease, pulmonary embolism, hemothorax, pancreatitis, chylothorax Trabsydatuve HF, hypoalbuminemia (GI losing enteropathy, cirrhosis, nephrotic syndrome, malnutrition), SVC obstruction, hepatohydrothorax, urinothorax, atelectasis, trapped lung, peritoneal dialysis, hypothyroidism, pulmonary embolism Note: pulmonary embolism, malignancy, and sar coidosis can present as either exudative or transudative effusions. HF following diuresis may become ‘‘pseudo exudative’’ (check albumin gradient) Clinical Features History dyspnea, cough, hemoptysis, chest pain, weight loss, fever, trauma, occup...
Read more

FNAC of Salivary Gland Tumors

-
Image
Fine Needle Aspiration Cytology (FNAC) of Salivary Gland Tumors Introduction: Salivary gland neoplasms though relatively uncommon are by no means rare, accounting for 3 % of all head and neck tumors. Distribution wise 80% of the tumors occur in parotid gland, 15% in submandibular gland and less than 1% in sublingual glands. The World Health Organization (WHO) has classified the tumors of salivary gland as primary or secondary, malignant or benign and by the tissue of origin. The five broad – categories of salivary gland tumors are: Malignant tumors: e.g. Mucoepidermoid Carcinoma, Acinic cell Carcinoma and Adenoid Cystic Carcinoma, Benign tumors: e.g. Pleomorphic Adenoma, Warthin’s tumor, Myoepithelioma, Soft Tissue tumors: e.g. Hemangioma, Hematolymphoid tumors: e.g. Hodgkin’s Lymphoma & Secondary tumors. Of the malignant tumors Mucoepidermoid Carcinoma is the most common neoplasm occurring most frequently in the parotid gland. Exposure to radiation has been thought to play or...
Read more

Crohn's Disease

-
Image
Crohn's disease causes inflammation of the small intestine. Although it may involve any part of the digestive tract from the mouth to the anus, it most commonly affects the last part of the small intestine, called the ileum, and the large intestine, including the colon and rectum. This is considered a chronic condition because it may recur at various times throughout your lifetime, with periods of remission in which you are free of symptoms. There is no consistently accurate way to predict when a remission may occur or when symptoms will return. The disease may occur in people of all ages, although most are diagnosed before the age of 30. However, it also can affect young children and older people in their seventies or beyond. Crohn's disease occurs most commonly in people living in northern climates and affects men and women equally. The condition can be inherited and is common in some families. About 20 percent to 25 percent of those with Crohn's disease have a close r...
Read more