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ID Case Conference April 9, 2008. Gretchen Shaughnessy, MD Clinical Fellow Dept of Infectious Diseases. CC: SOB.

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id case conference april 9 2008

ID Case Conference April 9, 2008

Gretchen Shaughnessy, MD

Clinical Fellow

Dept of Infectious Diseases

cc sob
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.
slide3
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.
slide4
PMH
  • HTN
  • DM – type II
  • GERD
  • Hiatial Hernia
  • Hyperlipidemia
  • h/o R shoulder surgery in 2001
  • CRI – baseline Cr 1.6
medications
Medications
  • Allergies – NKDA
  • Atenolol 100mg daily
  • Nexium 40mg daily
  • Glyburide 5mg po BID
antibiotic history
Antibiotic History
  • 11/4-11/14 Biaxin
  • 11/14-11/23 Azithromycin & Ticaricillin/clavulanate
  • 11/21-11/23 Methylpredisolone 60mg IV q8h
social history
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
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.
slide9
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

Physical Exam
osh labs
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 labs13
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
slide27
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
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
“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
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 syndrome31
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
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
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: Thin-section CT findings in 36 patients. Radiology 1999; 211:859.