A 67-year old man was admitted to the hospital with
a chronic cough, weight loss, muscle weakness, and cholestasis.
The patient had been well until 1 year prior to
when he began to manifest weakness and weight loss.
He developed a mild exertional dyspnea 10 days before
admission. The patient’s history was significant for 10
pack-years of cigarette smoking, but he had recently quit.
However, it was known that the patient was a professional
furniture maker who had a history of exposure to chemicals,
which was used in the wood industry. On admission,
his heart and respiratory rates and body temperature were
82/minute, 20/minute, and 37.8°C, respectively. Patient’s
skin was pale and weakness in the proximal muscles of
the extremities, particularly lower, suggested the occurrence
of slight myalgia. Dry inspiratory crackles were
heard bilaterally in the bases of the lungs, without signs
of consolidation, and with occasional bilateral wheezes.
On laboratory examination the following results were
found; hemoglobin: 9.2 g/dL (14.00-18.00), Serum gamma
glutamyl transpeptidase: 196 IU/L (7.00-60.00), alkaline
phosphatase: 281 IU/L (25.00-100.00), unconjugated
bilirubin: 1.2 mg/dL (0.10-0.50), conjugated bilirubin: 3.5
mg/dL (0.2-0.8), serum aspartate transaminase: 102 IU/
L (12.00-50.00), alanine transaminase: 58 IU/L (10.00-
70.00), alpha 2 globulin: 5.2% (8.0-12.0), C-reactive
protein: 96 mg/dL (0.00-6.00), erythrocyte sedimentation
rate: 110 mm/h (0.00-20.00), ferritin: 1244 ng/mL (18.
00-250.00), creatinine kinase: 504 mg/dL (22.00-200.00),
lactic dehydrogenase: 414 mg/dL (100.00-240.00), serum
albumin: 3.2 g/dL (3.40-5.00), and globulin: 3.6 g/dL
(1.50-3.50). Direct tests with standard and Ziehl-Neelsen
stains; cultures of blood, sputum, and urine; and antibodies
against various infectious agents including viruses
(hepatitis A, hepatitis B, hepatitis C, human immunodeficiency
virus, cytomegalovirus, and Epstein-Barr virus),
and chlamydia were negative, as were specific tests for
autoimmune disorders (Table
1). Cancer antigens CA 15-
3 and neuron specific enolase were found to be elevated in
the patient’s serum (Table
1). Chest radiograph revealed
occasional coarse reticular opacities in the lower lobes of
both lungs. Serially obtained noncontrast- and contrastenhanced
spiral computed tomographic examination of
the chest revealed a soft tissue mass (1x1.5 cm) near the
main pulmonary artery on the right hilus and bilateral
mild fibrotic changes with ground-glass appearance in the
bases (Figure
1). Single-breath carbon monoxide diffusing
capacity (mL/min/mm Hg) was 8.7 (59% of predicted
value), and the carbon monoxide diffusing constant was
1.3 (61% of predicted value). In electrophysiological
examination, small compound action potentials which underwent
a significant increase to normal limit after brief
exercise were found. Repetetive stimulation revealed a
decrement of compound action potentials at low rates of
stimulation and an increment (over 100%) at high rates of
stimulation. On the other hand, motor and sensory nerve
conduction velocities were within normal limits, F-waves
had borderline latencies. Needle electromyographic examination
demonstrated myogenic involvement in the
proximal muscle, suggesting a Myasthenic syndrome
(Lambert-Eaton type). An anthracotic lymph node was
found in a percutaneous transthoracic tru-cut biopsy of
soft tissue mass. Open lung biopsy revealed interstitial
lymphocyte, and plasma cell infiltration and accumulation
in the intraalveolar spaces, together with relatively
few numbers of polymorphonuclear leukocytes and eosinophils
with mild interstitial fibrosis. The alveolar space
was observed to be filled with alveolar macrophages,
and type 2 pneumocyte hyperplasia, suggesting the occurrence
of desquamative interstitial pneumonitis in the
subacute phase (Figure
2). A biopsy specimen of the liver
revealed mononuclear cell infiltration in the portal area,
severe centrilobular cholestasis with spotty necrosis, and
mild fatty degeneration (Figure
3). A biopsy specimen of
the muscle demonstrated immunoglobulin G, A, and M
deposition in the immunohistochemical examination of
the arterial walls.
Table 1: Patient characteristics of the study group
Fig 1: Contrast-enhanced spiral computed tomography of the thorax: The highresolution image of the lung bases reveals peripheral reticular opacities and peripheral ground-glass opacities in the lower lobes. Small round lucencies in the lower lobes suggest early honeycomb changes
Fig 2: Desquamative interstitial pneumonia (DIP): Interstitial lymphocyte and plasma cell infiltration and accumulation in the intraalveolar spaces, together with fewer numbers of polymorphonuclear leukocytes and eosinophils with mild interstitial fibrosis. The alveolar space is filled with alveolar macrophages, and type 2 pneumocyte hyperplasia, suggesting desquamative interstitial pneumonitis in the subacute phase (H&E;, x200)
Fig 3: A biopsy specimen of the liver: Mononuclear infiltration in the portal area, centrilobular severe cholestasis with spotty necrosis, and mild fatty degeneration (H&E;, x200)
The patient was given empirical antibiotic treatment
initially (ceftriaxone 1 g/12 hours for 7 days). Shortly
after, he was administered oral levofloxacin 500 mg/24
hours, and sulbactam-ampicillin 3 g i.v., b.i.d., for 7 days
but to no effect. High-dose methyl prednisolone (initial
dose 30 mg/kg/day) and cyclophosphamide (1000 mg
once a month) therapy were started six weeks after admission,
and symptomatic and clinical improvement were
subsequently seen.
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