Affiliate NetWork Online Puzzler - November 2007
Online Puzzler
Clinical presentation
The patient is a 60-year-old Japanese man who presented with a 2-week history of fevers, myalgias, fatigue, and a sore throat. He was treated as an outpatient with amantadine and clarithromycin for 5 days prior to admission without improvement. During this period, he also noted increasing right knee pain without any swelling, as well as poor appetite and a 10-lb weight loss. He did not report any history of rashes, cough, shortness of breath, chest pain, abdominal pain, nausea, vomiting, or diarrhea. There was no history of recent travel, contacts with people who are sick, or animal exposures. After an initial workup, he was admitted to the general medical ward and placed on broad-spectrum antibiotics. On the second hospital day, he was transferred to the medical ICU for severe tachycardia and tachypnea.
Past medical history:
Diabetes, hypertension, and dyslipidemia
Outpatient medications:
Metformin, rosuvastatin, and ramipril
Social history:
The patient denied tobacco use, illicit drug use, and heavy alcohol use. He is a restaurant owner in Los Angeles.
Physical examination (upon transferring to the ICU):
- Vital signs: temperature = 104.5°F; heart rate = 140 bpm; BP = 150/70 mm Hg; respiratory rate = 40 breaths/min; Sao2 = 100%, with supplemental oxygen via a nonrebreather face mask
- General: Febrile; tachypneic; alert and oriented to time, place, and person; could only speak in short sentences
- HEENT: Mild swelling and erythema of the oropharynx and soft palate. No lymph nodes were palpable.
- CV: Regular rhythm, tachycardic, no murmurs or gallops; there was a pericardial friction rub; no pulsus paradoxus
- Chest: Bibasilar crackles with slightly decreased breath sounds
- ABD: Active bowel sound, nontender, no hepatosplenomegaly
- Musculoskeletal: His right knee was tender to palpation and erythematous but no effusion was noted
- Neuro: No neurologic deficits or neck stiffness
- Skin: No rash
Laboratory data:
- WBC = 37 x 103 cells/µL, with 88% neutrophils and 7% bands
- Hemoglobin, hematocrit, and platelet levels were normal
- Renal and liver function test results were normal
- Urinalysis results were normal
- All smear and culture results from blood, urine, and sputum were negative
- Arthrocentesis of right knee revealed 3 mL of normal synovial fluid
- C-reactive protein (CRP) = 26.20 mg/dL; erythrocyte sedimentation rate (ESR) = 65 mm/h
- Antinuclear antibody (ANA) profiles, antineutrophil cytoplasmic antibody (ANCA) levels, and rheumatoid factor (RF) test result were negative
- Angiotensin-converting enzyme (ACE) level was normal
- Mycoplasma titer, Coxsackie titer, Legionella urine antigen, Lyme titer, Coccidioides titer, Histoplasma urine antigen, Epstein-Barr virus titer, cytomegalovirus polymerase chain reaction (CMV-PCR), and HIV test result were negative
- QuantiFERON® TB Gold (Cellestis Limited, Carnegie, Victoria, Australia) test result was negative
- Ferritin level = 8,000 ng/mL
- Serum troponin I and creatine kinase (CK) levels were normal
EKG:
Sinus tachycardia with diffuse ST elevation, concave upward
Transthoracic echocardiogram:
Mild- to moderate-sized pericardial effusion
Imaging studies:
- Admission chest radiograph was normal
- Chest CT on day 3 showed two peripheral nodular densities in the posterior left apical region (Fig 1A), calcified subcarinal and left hilar lymph nodes, a small focal area of parenchymal consolidation in the posterior upper lobe (Fig 1B), and a small pericardial effusion (Fig 1C)
- Chest CT day 4 (Fig 2) showed interval development of bilateral pleural effusion with lower lobes atelectasis and enlarging pericardial effusion
| 1A |  |
| 1B |  |
| 1C |  |
Figure 1. A, Chest CT on day 3 showing two peripheral nodular densities in the posterior left apical region. B, Chest CT on day 3 showing a small focal area of parenchymal consolidation in the posterior upper lobe. C, Chest CT on day 3 showing a small pericardial effusion.
Figure 2. Chest CT on day 4 showing interval development of bilateral pleural effusion with lower lobes atelectasis and enlarging pericardial effusion.
Hospital course:
After transferring to the ICU, the patient was intubated and placed on mechanical ventilation support. Antibiotic coverage was broadened, and the patient was empirically started on pulse-dose corticosteroids for a presumed fulminant connective tissue disease or vasculitis. A video-assisted thoracoscopic surgery right lung biopsy demonstrated acute focal capillaritis (Fig 3) and hemosiderosis. His condition significantly improved after the initiation of corticosteroids, and he was extubated. However, the patient deteriorated and required reintubation shortly after the corticosteroid dosage was reduced.
Figure 3. A video-assisted thoracoscopic surgery right lung biopsy demonstrating acute focal capillaritis (hematoxylin-eosin, original x 400).
At this point, what is the most likely diagnosis?
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