A 55-year-old Caucasian woman with scleroderma presented to the hospital in February 2009 with severe dyspnea. She felt well until 1 week prior, when she developed fevers, chills, and an intermittent dry cough. She then developed severe dyspnea over 2 days and presented to a local ED. Her temperature was 104°F, and arterial blood gases drawn revealed a pH of 7.46, a Pco2 of 32, a Po2 of 51, and an oxygen saturation of 88% on 100% nonrebreather mask. Chest radiograph showed a left lower lobe infiltrate. The patient was started on azithromycin and ceftriaxone, as well as IV solumedrol. She was intubated 1 day later for progressive hypoxia. Tracheal aspirate showed no organisms on Gram stain, and a culture revealed mixed flora. She was transferred to a tertiary hospital.
The patient has a past history of scleroderma, diagnosed 6 months prior to admission. At that time, she presented with severe edema in her feet, legs, and hands, and also skin changes in her fingers. She has been treated with prednisone and methotrexate for 6 months. She had no history of pulmonary involvement from the scleroderma. Recently, her rheumatologist felt that her skin changes were progressing. The methotrexate was stopped, and she had her first dose of cyclophosphamide, 25 mg, around the time she developed severe dyspnea.
The patient works as an elementary school teacher. She lives in a rural area of Ohio with her husband and has dogs, cats, and goats. She has not traveled recently. She has no history of TB. She had not received prior pneumococcal or influenza vaccinations.
Home medications
Prednisone, 20 mg tid, for 6 months
Methotrexate, 20 mg weekly, for 6 months
Cyclophosphamide, 25 mg daily, started 1 day prior to presentation
Metformin
Valsartan
On examination, her blood pressure was 150/70 mm Hg, pulse was 130/min, and temperature was 101.2°F. Her ventilator settings were the following: PIP of 40, Fio2 of 100%, PEEP of 10. She was sedated and agitated. Her lungs had some soft wheezing. She was tachycardic, with no murmurs. There was severe sclerodactyly below the elbows and in both feet.
Laboratory testing showed a WBC count of 28,000/µL (93% neutrophils) and hemoglobin level of 13.0 g/dL. Complete metabolic panel was unremarkable except for a lactate dehydrogenase level of 695 U/L (normal level is 100 to 220 U/L). Other laboratories included the following: antinuclear antibody test = 1:160, speckled pattern; sedimentation rate = 55 mm/h; C-reactive protein = 13 mg/dL; β-natriuretic peptide = normal level; and lactic acid = normal level. Figures 1 and 2 show her chest radiograph and CT scan, respectively.
Figure 1. Patient chest radiograph.
Figure 2. Patient CT scan.
Within a few hours of transfer, the patient became hypotensive and required multiple boluses of normal saline, and she started to receive pressors.
What is the most likely diagnosis?
Puzzler and case report submitted by:
Sara Mekuria, MD
Resident, Internal Medicine Residency Training Program
Cleveland Clinic
Cleveland OH
Nega Ali Goji, MD
Infectious Disease Specialist
Critical Care fellow, University of Rochester Medical Center
Rochester, NY
Edited by:
Carlos M. Isada, MD, FCCP
Department of Infectious Diseases, Cleveland Clinic
Cleveland OH
Case Puzzlers are a brief clinical vignette on various educational topics. Developed by members of the American College of Chest Physicians' NetWorks, it provides you an opportunity to sharpen your skills.
