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ID Case Conference April 9, 2008 Gretchen Shaughnessy, MD Clinical Fellow Dept of Infectious Diseases CC: SOB 46M presented to PCP with SOB and cough productive of white sputum. No hemoptysis, cough had been progressive over the past 3 weeks. He had been on Biaxin x 10 days without improvement. He’d also noticed some orthopnea, but denied any post-nocturnal dyspnea. Over the past 4-6 weeks he reported a 20lb wt loss and intermittent night sweats. Admitted to Outside Hospital for further workup. HPI During admission to Outside Hospital the patient had a normal echo (no pulm HTN), chest CT with moderate R pleural effusion and LLL interstitial process. Sputum cultures and pleural fluid analysis and cultures were done. Bronchoscopy with biopsy done day 6 of hospitalization, complicated by tension pneumothorax requiring chest tube placement. Despite broad specturm antibiotics and chest tube placement his respiratory status declined from 11/14-11/23. Transferred to UNC MICU. PMH HTN DM – type II GERD Hiatial Hernia Hyperlipidemia h/o R shoulder surgery in 2001 CRI – baseline Cr 1.6 Medications Allergies – NKDA Atenolol 100mg daily Nexium 40mg daily Glyburide 5mg po BID Antibiotic History 11/4-11/14 Biaxin 11/14-11/23 Azithromycin & Ticaricillin/clavulanate 11/21-11/23 Methylpredisolone 60mg IV q8h Social History No tob, ETOH, or drugs. Currently works in construction, former truck driver. A few weeks ago was sweeping parking lots and exposed to a lot of dust. No recent sick contacts. No recent travel. Frequently rides in a friend’s van that is used to transport chickens and rabbits (patient has never encountered the animals in person) History of travel to the southwest as a truck driver, but none in the past 2 years. Denies HIV risk factors, has never been tested. Family History Mother - DM type II Father – ETOH induced liver disease No family history of autoimmune disease Patient’s mother had Mycobacterial Tuberculosis 15 years ago. He reports 6 months of treatment for his mother and the whole family had to get PPDs placed. The patient’s PPD was positive but he does not remember getting LTBI treatment. ROS Night sweats and weight loss for 6 weeks No chest pain, no N/V/D, no BRBPR, no hematuria or dysuria. No joint pain or swelling. No rashes or skin lesions. Otherwise negative ROS. Physical Exam T 37.7 - HR 110 - RR 36 BP 95/65 – 99% on 100% NRB Tachypnec, on 100% NRB a&ox3, pleasant and cooperative mild errythema seen on the ventral surface of the elbow on the RUE soft NT nabs, no HSM no c/c/e nl tone, full ROM present no focal defecits EOMI, PERRLA, nonicteric no e/e on OP no JVD no LAD appreciated in cervical, supraclavicular, or inguinal regions II/VI systolic murmur decreased breath sounds at the bases. L chest wall is higher than R, crepitus present. chest tube present OSH Labs Pleural fluid - exudative process, pH was 8 and the glucose was reportedly elevated Micro from bronch- gram stain many WBCs, yeast, rare GPCs. aerobic cultures candida albicans only anaerobic cultures no growth fungal cultures growing candida albicans only AFB smear and culture pending at the state lab. Bronch biopsy – diffuse fibrosis and inflammation, ?UIP OSH Labs Urine microscopy: granular casts, monomorphic red cells, and yeast with pseudohyphae urine microalbumin 100 (11/24), UP/C 4.069 UA 1.010/5.0/1+ protein/3+ blood/7 WBC/148 RBC/4 granular casts/occ bacteria HgbA1C 8.2 ESR 68 SPEP and UPEP negative ANA, ENA panel negative Radiology Discussion Labs Serum crypto, urine histo, urine legionella negative PCP DFA negative from bronch AFB smears and cultures from bronch, pleural fluid, all negative Further Hospital Course Patient had slides from lung biopsy sent to UNC Pathology showed findings concerning for acute interstitial fibrosis. AFB, bacterial, viral, and fungal cultures all negative. Hypoxia progressed, ARDS, unable to oxygenate Patient expired on post-transfer day #10 “A Diagnostic test was performed…” Unfortunately, autopsy. (“Pathologists always get the diagnosis… just a little late.”) Late exudative stage of early organizing stage idiopathic diffuse alveolar damage (acute interstitial pneumonia). – Severe bilateral acute interstitial pneumonia Nephrotic range proteinuria 2/2 minimal change disease All cultures and microscopic analysis negative Diagnosis – Hamman Rich Syndrome Hamman-Rich Syndrome Acute interstitial pneumonia Described by Hamman and Rich in 1934 Rare and fulminant form of rapidly fibrosing lung disease (idiopathic DAD) Occurs in previously healthy individuals without a history of lung disease, presents within days to weeks of onset of symptoms Unknown mechanism of the damage to the pulmonary endothelium and epithelium Hamman-Rich Syndrome Onset usually abrupt, prodromal illness lasts 7 to 14 days Most common clinical signs and symptoms are fever, cough, and shortness of breath Not associated with cigarette smoking Most patients are over the age of 40 years, with a mean age of 50 to 55 years Treatment Prognosis is poor, mortality rate >60% at initial presentation. Most patients die within 6 months Treatment is supportive, attempt to identify any possible cause References Mandell’s Principles and Practices of Infectious Disease, 6th Ed. Hamman, L, Rich, AR. Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Climatol Assoc 1935; 51:154. Vourlekis, JS. Acute interstitial pneumonia. Clin Chest Med 2004; 25:739. Katzenstein, ALA, Myers, JL, Mazur, MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell kinetic study. Am J Surg Pathol 1986; 10:256. Bouros, D, Nicholson, AC, Polychronopoulos, V, du Bois, RM. Acute interstitial pneumonia. Eur Respir J 2000; 15:412.6.Vourlekis, JS, Brown, KK, Cool, CD, et al. Acute interstitial pneumonitis. Case series and review of the literature. Medicine (Baltimore) 2000; 79:369. Fulmer, JD, Katzenstein, ALA. The interstitial lung diseases. In: Pulmonary and Critical Care Medicine, Bone, RC (Ed), Mosby Year Book, St. Louis; 1993, M1.8.Olson, J, Colby, TV, Elliott, CG. Hamman-Rich syndrome revisited. Mayo Clin Proc 1990; 65:1538. Primack, SL, Hartman, TE, Ikezoe, J, et al. Acute interstitial pneumonia: Radiographic and CT findings in nine patients. Radiology 1993; 188:817. Johkoh, T, Muller, NL, Taniguchi, H, et al. Acute interstitial pneumonia: Thinsection CT findings in 36 patients. Radiology 1999; 211:859.