Disclosure Statement Interstitial Lung Disease: What the Generalist Needs to Know A Case Based Approach Talmadge E. King, Jr., M.D. Julius R. Krevans Distinguished Professorship in Internal Medicine Chair, Department of Medicine University of California San Francisco (UCSF) San Francisco, CA Dr. King has served on a Scientific Advisory Board for the following companies: InterMune ImmuneWorks Boehringer Ingelheim Daiichi Sankyo Pharma Case 1 60 year old college professor dyspnea with exertion dry cough, throat clearing Occupational/Environmental Exposures: shipyard worker (in his 20 s) home remodeled (within last 5 yrs.) 1
(continued) PAST MEDICAL HISTORY: former smoker (20 pack yrs; quit 10 yrs) coronary artery disease systemic arterial hypertension chronic nasal congestion GERD FAMILY & SOCIAL HISTORY Negative for lung disease MEDICATIONS: lisinopril (ACE inhibitor) lortadine (antihistamine) omeprazole (proton pump inhibitor) albuterol/ atrovent (continued) PHYSICAL EXAMINATION RR 14 Bibasilar crackles O 2 sat 98% at rest 90% after walking 200 meters (continued) HRCT Scan of Lungs Baseline 1 yr 2 yr FEV1 2.8 (87) 2.7 (86) 2.5 (80) FVC 3.2 (72) 3.2 (72) 3.0 (68) FEV1/FVC 87% 85% 84% TLC 4.0 (59) 4.1 (61) 3.4 (50) DLCO 16.1 (60) 16.6 (62) 13.5 (51) 2
Final Diagnosis Case 2 53 year old woman 5 month history of shortness of breath and non productive cough Past Medical History: Hypertension Treated for TB exposure in 1980 Arthritis GERD Medications: Diltiazem 3
Social History Former smoker (18 pack yrs, quit 1 year ago) Occupation: office worker Family History No history of lung disease BP 135/80 HR 90 RR 16 SpO 2 94% RA @ rest & 90% walking Dry inspiratory crackles at both bases Normal cardiac examination No clubbing, joint deformities, rashes FVC 3.10 (63%) FEV 1 2.67 (70%) FEV 1 /FVC 0.86 TLC 5.40 (72%) DL CO 16.41 (48%) ANA 1:80 speckled RF 1:40 4
Clinical Case 1 Pathology Diagnosis: Nonspecific Interstitial Pneumonia 5
RNP = negative Scl70 = negative SSA, SSB =negative Jo 1 = negative CPK = 104 Anti cyclic citrullinated peptide (CCP) >100 Final Diagnosis Case 3 26 year old woman was well until approximately 18 months ago. At that time she developed a couple of "pimplelike" lesions on the right side of her face. These lesions became larger and spread to her legs, arms, and trunk. The soles of her feet and her hands have been spared. The lesions were painful, especially when any pressure was applied over a lesion. 6
Skin Lesions S.J.: Leg S.J.: Lower Back 4 x 3 cm erythematous macular confluent patch of erythema on the right mandible at the angle of the mandible. Similar 1 x 1 lesion on the mandible on the left side of the face. There are a couple of subcentimeter similar erythematous macules on the forehead. Diffusely over the body with the greatest distribution on the posterior trunk and bilateral lower extremities, specifically the calves, there are multiple subcentimeter to centimeter raised red, somewhat plaquelike lesions, which are palpable. She saw a dermatologist. The initial treatment was a topical ointment (unclear what this was). It was ineffective. She was started on minocycline and had injections of the lesions (unclear what was injected). Approximately the same time that the skin rash developed, she developed night sweats. However, these sweats stopped after she discontinued a supplemental detoxification regimen (that she bought at a nutrition store). She acknowledges at least a 4 pound weight loss; despite a voracious appetite. She has had a cough, occasionally productive of white or yellow sputum. She complains of extreme fatigue (as well as the pain that she experiences from her rash). Because of this, she notes that she has been essentially bed bound for the last eight She complains of occasionally blurred vision, although she had normal evaluation by an ophthalmologist two months ago. She denies any burning or gritty sensation in her eyes. She occasionally has headaches, which she describes as diffuse pressure in her head. 7
MEDICATIONS: No medications. ALLERGIES: No known drug allergies. SOCIAL HISTORY: She was born in Alameda and has lived most of her life in northern California, having briefly lived in Hawaii as well as in Indiana. Her childhood medical history is only notable for having had "dizzy spells," which required that she stay in bed for days up to a week. She has never had any jobs outside the home. EXPOSURES: Significant exposures include the fact that her home has excessive dust as well as mildew. The carpeting has been the same for the last 17 years. The only pets in the home were cats; however, there are no animals in the home at this time. She feels worse when she is in the house; specifically, that she notes increased nausea at that time. HABITS: She previously drank alcohol approximately 3 times a week (two drinks). She smoked cigarettes, marijuana, and cocaine powder in the past but quit >6 months ago. FAMILY HISTORY: Her biological mother has hypertension and had a stroke in her 40s (had crack cocaine addiction). All of the patient's siblings are healthy. Her grandfather had coronary artery disease with his first bypass surgery in his 50s. Diabetes has been found in multiple more remote members of her father's and mother's families 8
PHYSICAL EXAMINATION: VITAL SIGNS: Weight 126 pounds, BP: 107/67, Pulse: 117, O 2 saturation of 99% at rest on room air. With ambulation on level ground, heart rate rose to 140 with desaturation to 94%. GENERAL: Comfortable at rest, though she is quite concerned and intermittently tearful. HEENT: 1 cm left supraclavicular fossa lymph node, mobile and nontender. LUNGS: Symmetric expansion. No dullness to percussion. Clear to auscultation CARDIAC/ABDOMEN/NEUROLOGIC/EXTREMITIES: no abnormalities noted SKIN: (as previously described) Laboratory Studies: White blood cell count 8,400, hematocrit 30.2 (MCV 78), platelets 348,000. (also microcytic with 0 to 1 schistocytes seen per high powered field). Differential included 5.9 neutrophils, 1.47 lymphocytes, and 0.81 monocytes. Serum chemistries: sodium of 133, potassium 3.9, chloride 97, bicarbonate 24, BUN 15, creatinine 1.4. Glucose was 112, calcium 10.8, AST 31, ALT 14, total bilirubin 0.7, and lipase 21. PA Chest x ray 9
PULMONARY FUNCTION TESTS FEV1 3.23 (103) FVC 4.24 (108) FEV1/FVC 76% TLC 5.69 (110) DLCO 20.5 (91) OTHER LABS: ACE = 480 May 2002: hepatitis B and C antibodies negative. April 2002: ANA and RPR = negative Sedimentation rate = 38 CK 45, AST 60, total bilirubin 0.6, and alkaline phosphatase 178. Creatinine = 1.2 SKIN BIOPSY Right leg: sarcoidal granulomatous dermatitis with overlying postinflammatory pigment alterations. Left arm: sarcoidal granulomatous dermatitis. Final Diagnosis 10
Case 4 42 y.o. woman, previously healthy except for mild systemic arterial hypertension. Chief Complaint Fatigue & SOB with minimal exertion Missing days from work Occupation: social worker for school district 42 y.o. woman CXR and CT Scan Physical examination Crackles at bases CXR and HRCT scan Pulmonary Function Testing 11
Pulmonary Function Tests Predicted Baseline % of Predicted Lung Volumes TLC 5.38 3.35 62% TGV 3.00 1.90 63% RV 1.31 1.14 87% Spirometry FVC 4.06 2.21 54% FEV 1 3.35 1.88 56% FEV 1 /FVC 82 85 Diffusing Capacity DL CO 31.29 14.7 47% DL CO /VA 6.03 4.96 82% Flow Lung Restriction & Increased Elastic Recoil: Stiff Lungs Flow Volume Curve Volume % Pred. TLC Pressure Volume Curve Pressure Review of Previous History 8 months ago (June) Developed a cold Saw M.D., given antibiotics for walking pneumonia 7 months ago (July) Developed a cold Saw M.D. Chest x ray and CT scan obtained Given antibiotics for walking pneumonia Initial CXR and CT Scan 12
Review of Previous History 7 months ago (July) Developed a cold Saw M.D. Chest x ray and CT scan obtained Given antibiotics for walking pneumonia Went on vacation for 2 weeks, returned feeling much better B.G. Review of Previous History 4 months ago (October) Feeling more fatigue, weight loss, dyspnea with exertion Saw M.D. Chest x ray and CT scan obtained B.G.: Follow up CXR and CT Scan B.G. Review of Previous History 2 months ago (December) Fatigue worse Dyspnea with exertion worse Developed a bad cold 13
New history Bronchiolocentric Inflammation & Loosely Formed Granulomas Feb. Daughter has bird in her bedroom It s her bird, I have little to do with it. Final Diagnosis Thank you for your attention. 14