Interstitial and Infiltrative ti Lung Diseases
Case 0 65 year male with chronic dyspnea. A was veteran with multiple occupations including construction and shipyard work. Physical exam shows clubbing and bibasal crepitations. CXR shows small lungs and bilateral interstitial opacities. PFTs show FEV1 60%, FVC 60%, FEV1/FVC 90%, TLC 58%, DLCO 56%. All other history, physical examination and lab work is negative.
Case 0 What is your diagnosis? How do you manage this patient?
Case 1 A35 year old ldafi African American female presents to the pulmonary clinic for cough and dyspnea for six weeks. She was seen by her primary care physician on multiple occasions, and was prescribed trimethoprim/sulfamethoxazole, azithromycin and levofloxacin on multiple occasions after she was found to have bilateral infiltrates on her chest X ray
Case 1 List the main differential diagnoses based on the information so far.
Case 1 Further questioning reveals that she has also noted raised red lesions on her skin, a red eye for the last two weeks and has noted multiple painless nodules in her neck and groin region She was recently incarcarated (one month before the onset of these symptoms), but is not sure if anyone she came in contact with had tuberculosis Physical examination confirms posterior uveitis and cervical, axillary and inguinal lymphadenopathy. Pulmonary examination is remarkable for dyspnea and bibasal crepitations
Case 1 List your main differential diagnoses at this point
Case 1 At tuberculin purified protein derivative test t (ppd) is placed and is negative Laboratory examination is remarkable for hypercalcemia and elevated serum ACE levels Chest X ray and CT examination of chest show diffuse interstitial opacities and extensive mediastinal lymphadenopathy
Case 1 What is the next step in evaluating/treating this patient
Case 1 A bronchoscopy and transbronchial biopsy is performed Biopsy shows noncaseating granulomatous lesions Mycobacterial and fungal cultures are all negative
Case 1 What is your final diagnosis? What stage of disease does this patient have? Would you treat this patient? What options do you have with regards to treatment?
Case 2 A45 year female with chronic recurrent urinary tract t infections presents with progressive dyspnea and cough. She is on nitrofurantoin, vitamins and calcium supplements, all of which h she has been taking for years. Physical examination shows bibasal crepitations. CXR and CT shows bilateral interstitial infiltrates. PFTs show a FEV1/FVC of 85%, FVC of 65%, TLC of 65% and DLCO of 45% All other laboratory evaluation is negative.
Case 2 What is your differential diagnosis at this point? How would you treat this patient?
Case 3 A 36 year old female with long standing rheumatoid arthritis which is poorly controlled, presents to the pulmonary clinic i with progressive dyspnea and cough for the past ttwo months. Her medications include prednisone, methotrexate, hydroxychloroquine and ibuprofen.
Case 3 Physical examination shows joint changes consistent with advanced rheumatoid arthritis and bibasal crepitations Chest X ray and CT shows bilateral diffuse interstitial opacities PFTs show FEV1/FVC of 85%, FEV1 of 60%, FVC of 60%, TLC of 60% and DLCO of 40%
Case 3 List your differential diagnoses at this point How would you proceed to diagnose and treat this patient
Case 4 A42 year male with ithhistory of nonresolving sinusitis and nasal septal perforation presents with a cough, constitutional symptoms and hemoptysis for two weeks. He also complains of cloudy urine and swelling of ankles and face. Physical examination shows the nasal septal perforation, right lower lobe crepitations and pedal edema
Case 4 CXR shows a right lower lobe infiltrate with a thick walled cavity His creatinine is elevated, and urinalysis shows hematuria and RBC casts Sputum cultures are all negative for bacterial, mycobacterial and fungal organisms Blood tests are positive for c-anca and p- ANCA (Anti Neurtophilic Cytoplasmic Antibody) Transbronchial biopsy shows granulomatous vasculitis
Case 4 What is your diagnosis? How would you treat this patient?
Case 5 A42 year old nonsmoking female presents with cough and dyspnea for the last four months. She admits to couging small quantities of blood intermittently. Physical examination shows bilateral wheezing, and a scar on the side of his chest, which according to her was for a tube that was placed for a collapsed lung three month ago. CBC is unremarkable. FEV1/FVC is 60%, FEV1 is 55%, FVC is 80%, TLC is 140%, DLCO is 55%. CXR shows hyperinflation and some reticular prominence, and a right sided small pleural effusion. HRCT chest shows diffuse thin walled round cysts without any ground glass opacities, airspace consolidation or honeycombing.
Case 5 This presentation ti is consistent t with: (More than one may be correct) Bronchial asthma COPD Chronic eosinophilic pneumonia Idiopathic pulmonary fibrosis (Usual interstitial pneumonitis) Acute interstitial pneumonitis Acute eosinophilic pneumonia Cryptogenic organizing pneumonia Lymphangioleiomyomatosis Pulmonary langerhans cell histiocytosis
Case 6 A35 year old male smoker presents with cough and dyspnea for the last two months. Physical examination shows bilateral wheezing, and a scar on the side of his chest, which according to him was for a tube that was placed for a collapsed lung one month ago. CBC is unremarkable. FEV1/FVC is 60%, FEV1 is 55%, FVC is 70%, TLC is 70%, DLCO is 55%. CXR and CT show bilateral cysts and nodules more prominent in the upper zones, without t any volume loss, and clear costophrenic sulci.
Case 6 This presentation is consistent with: (More than one may be correct) Bronchial asthma COPD Chronic eosinophilic pneumonia Idiopathic pulmonary fibrosis (Usual interstitial pneumonitis) Acute interstitial pneumonitis Acute eosinophilic pneumonia Cryptogenic organizing pneumonia Lymphangioleiomyomatosis Pulmonary langerhans cell histiocytosis
Case 7 A 75 year old male with no significant ifi history presents to his PCP for evaluation of progressive dyspnea for the past 4 months. No significant drug or exposure history is present. Physical examination shows a comfortable patient with bibasal coarse velcro crepitations and clubbing. Remaining history and physical is negative. CXR shows bilateral interstitial markings in the bases. HRCT shows bilateral subpleural reticular abnormalities with honeycombing and without any ground glass abnormalities. PFTs show a FEV1/FVC of 85%, FEV1 of 65%, FVC of 65%, TLC of 65% and DLCO of 45%. BAL shows neutrophilia but is negative otherwise.
Case 7 This presentation is consistent with: (More than one may be correct) Acute eosinophilic pneumonia Pulmonary langerhans cell histiocytosis Chronic eosinophilic pneumonia Idiopathic pulmonary fibrosis i (Usual interstitial titi pneumonitis) Acute interstitial pneumonitis Cryptogenic organizing pneumonia Lymphangioleiomyomatosis
Case 7 What are the treatment options for this patient?
Case 8 A45 year male with ithaids presents to the pulmonary clinic for five weeks of dry cough and constitutional symptoms. Physical exam shows occational basal crepitations. CXR shows bilateral diffuse interstitial opacities. Differential diagnoses? Diagnostic/therapeutic plan?
ILDs: History Acute/Subacute/Chronic Dyspnea Cough Other symptoms, depending on associated conditions Exposure, drugs, connective tissue disorders, Exposure, drugs, connective tissue disorders, other system involvement etc.
ILDs: Physical Examination Dyspnea/tachypnea Crackles Clubbing Hypoxemia Other findings depending on the cause Eye, joint, skin findings etc.
ILDs: Diagnostic tests Cell counts Eosiniphilia etc. Chemistry Renal function, calcium etc. Specific tests Serum precipitins, connective tissue serology, ACE levels etc. PFTs Restrictive Lung biopsy
ILDs: Treatment Removing offending agent Immunosppressants Prednisone Others Oxygen Lung transplantation