Jurnal

A 56 -Year-Old-Man With Common Variable

Immunidefiency and Worsening Dyspneu

Oleh :
Sarah Nadia (18174052)

Pembimbing :
dr. Nurfitriani Sp.P

Bagian/SMF Ilmu Kesehatan Paru

RSUD Meuraxa
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Chok limsuwat, MD; Philip J. Daroca, MD; and Joseph

A. Lasky, MD

CHEST 2018
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CASE PRESENTATION

A 56-year-old man with a history of common variable

immunodeficiency (CVID), in addition to recurrent bronchitis and
pneumonia, presented with progressively worsening shortness of
breath over a period of a few months. The patient also developed
a frequent dry cough and wheezing.
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CASE PRESENTATION

He denied fever, chest pain, weight loss, and hemoptysis. He had

been diagnosed with CVID in 1968, at 7 years of age, after
recurrent episodes of bronchitis and pneumonia, and was treated
with IV immunoglobulin monthly for decades. The patient was a
lifelong nonsmoker and had no significant environmental or
occupational exposures. He was referred to our hospital for
further management.
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Physical Examination Findings

✖ Temperature, 98.2 F
✖ Heart rate regular at 95 beats/min
✖ Respirations, 20 breaths/min
✖ Blood pressure, 106/70 mmHg
✖ He was mildly obese with a BMI of 31
kg/m2 .
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The patient appeared comfortable at rest on

room air, and his oxygen saturation was 95%.
His lung examination revealed diminished
breath sounds throughout with a prolonged
expiratory phase. Mild rhonchi were present
bilaterally.
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Diagnostic Studies
Result of CBC and differential are as follows
✖ WBC, 10.3/mL (73.8% polymorphonuclear
neutrophils, 13.9% lymphocytes, 7.3%
monocytes, 1.2% eosinophils) and hematocrit,
39.9%.
✖ Basic metabolic panel : Normal
✖ HIV serologic : Negative
✖ Pulmonary function tests (PFTs) revealed
severe obstruction
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Diagnostic Studies

CT scan
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Diagnostic Studies

A diagnostic video-assisted
thoracoscopic surgery (VATS)
✖ Revealed centrilobular peribronchiolar
collagenous fibrosis with bronchiolarization of
centrilobular airspaces.
✖ Small centrilobular noncaseating epithelioid
granulomas and patchy lymphohistiocytic
interstitial inflammation
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Diagnostic Studies

Histologic section
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What is the diagnosis ?

Common variable immunodeficiency with
granulomatous and lymphocytic interstitial lung
disease
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discussion

CVID is a disorder with impaired B-cell

differentiation and immunoglobulin production.
- Clinical manifestations of CVID include recurrent
infections, most commonly involving the
sinuses, lung, and skin.
- Chronic pulmonary disease is a common problem
among patients with CVID.
- CVID is also associated with the occurrence of
granulomatous lymphocytic interstitial lung
disease (GLILD)
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29%
patients with CVID have manifestations of chronic lung
diseases

10% to 18%
patients with CVID develop interstitial lung disease, such as
GLILD
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GLILD
GLILD is characterized by histologic changes that include
lymphocytic interstitial pneumonitis, follicular
bronchiolitis, and nonnecrotizing granulomas. The
pathobiology of GLILD is unclear.
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GLILD
GLILD is associated A recent retrospective
with increased study reported on the
mortality and is not treatment of GLILD with
clearly responsive to a combination of weekly
treatment with rituximab for 4 weeks
corticosteroids. used at 4- to 6-month
intervals for three to four
cycles in total and
azathioprine therapy for
18 months in seven
patients.
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CLINICAL COURSE
The patient had CT imaging and histologic findings typical of
GLILD. He was treated with rituximab weekly (375 mg/m2 per
infusion) for 4 weeks, followed by treatment with
azathioprine 200 mg/d.
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CLINICAL PEARLS

Interstitial lung There is no

diseases occur in current
w10% to 18% of standard
patients with CVID treatment
and may manifest regimen for
as GLILD. GLILD

High-resolution CT scans
in patients with GLILD
usually demonstrate
diffuse ground-glass or
reticular patterns, and
VATS biopsy is generally
required to make a
definitive diagnosis.
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GLILD is associated with increased mortality and is not

clearly responsive to treatment with corticosteroids

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between green and spectrum. danger and courage.
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