Chinese Medical Journal 2012;125(17):3195-3197 3195
Clinical practice
Organizing pneumonia associated with common variable
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immunodeficiency
CAO Meng-shu, CAI Hou-rong, ZHANG Ying-wei, MENG Fan-qing and SUN Ling-yun
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Keywords: organizing pneumonia; common variable immunodeficiency; surgical lung biopsy
C ommon variable immunodeficiency (CVID) is a rare
disease characterized by recurrent pulmonary
infections, hypogammaglobulinemia, and diminished
specific antibody response to immunization. The
estimated prevalence rate of CVID ranged from 1/50 000
to 1/200 000.1 The common clinical manifestations of
CVID are repeated and prolonged respiratory infections.
Thus, bronchitis, bronchiectasis, and pneumonia are all
well recognized, whereas diffuse interstitial lung disease
(ILD) has not been comprehensively investigated in
CVID. Organizing pneumonia (OP), one of the
phenotypes in ILD, is rarely associated with CVID. There
are only two cases reported in English literature searched
in Pubmed.2,3 We herein present the first case of OP
related to CVID in China, who was successfully treated
with corticosteroid therapy.
An 18-year-old girl went to local hospital for evaluation
of abnormal opacities on chest radiography detected by a
health screening for college entrance examination in
March 2011 in China. Chest CT showed nodular and
patchy consolidations of the bilateral lungs, ground glass
opacities (GGOs) of the lower lobes, and bronchiectasis
of the right lower lobe. She had 3-year history of
recurrent respiratory tract infections, and was allergic to
“blattodea and cat hair”. Initially she was diagnosed as
hypersensitivity pneumonitis and treated with
anti-allergic drugs (no glucocorticoids) for one month.
Chest CT revealed patchy consolidations of the bilateral
lower lobes decreased, with the nodular shadows
increased. So she was admitted to another hospital in
Nanjing for further diagnosis and treatment.
On admission, there were no abnormal findings on
physical examination. Laboratory findings demonstrated
normal white cell counts. Biochemistry tests showed total
protein of 52.2 g/L (normal range 60–85 g/L), albumin of
38.3 g/L (normal range 35–55 g/L) and globin of 13.9 g/L
(normal range 25–40 g/L). Serum levels of IgG, IgA and
IgM were 1.6 g/L, 0.26 g/L and 0.38 g/L (normal range
7.0–16.0 g/L, 0.7–4 g/L and 0.4–2.3 g/L) respectively.
Rheumatoid factor (RF), antistreptolysin O (ASO),
antinuclear antibody (ANA) and antineutrophil
cytoplasmic antibody (ANCA) were all negative.
C-reactive protein and erythrocyte sedimentation rate
levels were normal. The serum level of γ-interferon was
normal. The sputum cultures for fungi and bacteria and
specific staining for mycobacteria were all negative.
Pulmonary function tests (PFTs) showed a forced vital
capacity (FVC) of 2.25 L (72.3% predicted), a forced
expiratory volume in one second (FEV1) of 1.85 L
(70.2% predicted), and an FEV1/FVC ratio of 82.45% and
a total lung capacity (TLC) of 3.34 L (71.9% predicted),
which was a mild restrictive pattern. Diffusing capacity of
carbon monoxide of the lung (DLCO) was 4.11
mmol·min-1·kPa-1 (45.9% predicted) (Table 1). A
bronchoscopy was performed. Bronchial alveolar lavage
fluid (BALF) cultures and specific stainings for fungi and
bacteria were all negative. Polymerase chain reaction for
DNA to tubercle bacillus of the BALF was normal. The
histopathology of transbronchial lung biopsy samples
showed chronic inflammation in mucous membrane. The
patient was initially diagnosed of pulmonary infection
and injected with piperacillin/tazobactam sodium for 10
days. She was discharged from hospital because of
asymptomatic. However, new nodular and patchy
consolidations of the bilateral lungs were revealed on
chest CT (Figure 1A and 1B) one month later. PFTs
demonstrated a progressive decrease of the pulmonary
ventilation function and diffusion capacity. Since there
was still some uncertainty of the pulmonary diagnosis,
the patient was readmitted to the former hospital for
video-assisted thoracoscopic surgery biopsy (VATS).
After surgical lung biopsy, she was referred to the
Affiliated Drum Tower Hospital of Nanjing University
Medical School for further diagnosis.
On admission the laboratory examination showed the
percentage of B lymphocytes in peripheral blood was
only 1.2% (normal range 6.4%–22.6%), and the
percentages of CD3+CD4+ T cells, CD3+CD8+ T cells and
DOI: 10.3760/cma.j.issn.0366-6999.2012.17.045
Department of Respiratory Medicine, Affiliated Drum Tower
Hospital of Integration of Traditional and Western Medicine,
Nanjing University of Chinese Medicine, Nanjing, Jiangsu 210008,
China (Cao MS)
Department of Respiratory Medicine (Cai HR and Zhang YW),
Department of Pathology (Meng FQ), Department of
Rheumatology and Immunology (Sun LY), Affiliated Drum Tower
Hospital of Nanjing University Medical School, Nanjing, Jiangsu
210008, China
Correspondence to: Dr. CAI Hou-rong, Department of Respiratory
Medicine, Affiliated Drum Tower Hospital of Nanjing University
Medical School, Nanjing, Jiangsu 210008, China (Tel:
86-25-83105207. Fax: 86-25-83105207. Email: caihourong@
yahoo.com.cn)
The authors declare that they have no competing interests.
 3196 Chin Med J 2012;125(17):3195-3197

Table 1. Serial pulmonary function tests

Before steroid therapy After steroid therapy
Variables May 13, 2011 July 15, 2011 August 18, 2011 October 20, 2011
Act % Pred Act % Pred Act % Pred Act %Pred
FVC (L) 2.25 72.3 1.58 52.7 2.09 70.1 2.19 72.8
FEV1 (L) 1.85 70.2 1.31 47.7 1.66 60.9 1.91 69.6
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FEV1/FVC (%) – 82.45 – 83.31 – 79.59 – 86.87

TLC (L) 3.34 71.9 2.49 58.2 2.86 68.0 2.81 66.7
RV (L) 1.2 95.5 1.13 114.7 0.81 85.6 0.68 72.7
DLCO (mmol · min-1 · kPa-1) 4.11 45.9 5.09 34.5 6.27 43.0 5.15 35.5
Pred: predicted values; Act: actual values; FVC: forced vital capacity; FEV1: forced expiratory volume in one second; TLC: total lung capacity; RV: residual volume;
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DLCO: diffusing capacity of carbon monoxide of the lung.

Figure 1. The chest CT and lung histopathology. A, B: Chest CT

scan (June 29, 2011) showed multiple nodular opacities and patchy
consolidations of the bilateral lungs, and ground glass opacities
(GGOs) of the two lower lobes. C, D: Plug of granulation tissue
obliterated the alveolar lumen, alveolar ducts and small airways.
The adjacent pulmonary parenchyma was infiltrated by chronic
inflammatory cells. In local areas, there were also nodular
lymphoid hyperplasia with germinal centers and fibrosis (HE,
original magnification ×200). E, F: HRCT scan (September 21,
2011) showed sparing strip opacities and GGOs at the bilateral middle and lower of the lungs, bronchiectasis of right middle lobe, and
mild reticular shadows at the bilateral basilar segments. The extent of nodularity and consolidations was reduced apparently.

NK cells were normal. The histopathology of lung biopsy
samples obtained by VATS showed that plug of
granulation tissue obliterated the alveolar lumen, alveolar
ducts, and small airways (Figure 1C and 1D), and the
adjacent pulmonary parenchyma was infiltrated with
chronic inflammatory cells. In local areas, there were also
nodular lymphoid hyperplasia with germinal centers and
fibrosis. Finally, she was diagnosed of OP associated
CVID and treated with oral prednisone 40 mg/d. She did
not accept immunoglobulin replacement because of
expensive medicine expenditures.
In the following month, pulmonary functions were greatly
improved (Table 1). Chest CT (Figure 1E and 1F) showed
sparing strip opacities and GGOs at the middle and lower
of the bilateral lungs, bronchiectasis of the right middle
lobe, and mild reticular shadows at the bilateral basilar
segments two months later. The extent of nodular
opacities and consolidations was markedly reduced. Then
the dose of prednisone was tapered to 20 mg/d. Six
months after corticosteroid therapy, the white cell and
lymphocyte counts were normal, the percentage of B
lymphocytes in peripheral blood was still 1.2%. Serum
levels of IgG, IgA and IgM decreased to 0.1 g/L, 0.01 g/L
and 0.05 g/L, respectively. The patient refused
immunoglobulins replacement treatment again.
CVID is a rare disorder characterized by the defection of
B lymphocytes function. Usually, the number of B cells
in patients is lower or even not changed compared with
that in normal individuals. However, these B cells failed
to mature into plasma cells in patients with CVID. The
current patient had a lower peripheral blood B cell
number with normal levels of other immune cell subsets,
which may result in lower serum levels of
immunoglobulin. So far, the diagnosis of CVID remains
exclusive, and the subjects must meet the criteria
proposed by Chapel and Cunningham-Rundles4 in 2009.
Our patient was an 18-year-old girl, the serum levels of
IgG, IgA and IgM were all below normal. The diagnosis
of CVID was based upon a medical history of recurrent
respiratory tract infections associated with a dramatic
decrease in serum levels of immunoglobulins.
The common clinical manifestations of antibody
deficiency syndromes are repeated and prolonged
respiratory infections. However, the frequency and type
of ILD in patients with CVID are incompletely
appreciated. Popa et al5 described that ILD was frequent
(19.6%) in a consecutive series, much more prevalent
than expected from general population surveys. Several
histologic patterns of ILDs have been reported in patients
with CVID, including lymphocytic interstitial
pneumonitis (LIP),6,7 granulomatous interstitial
8
pneumonitis, bronchiolitis obliterans organizing
pneumonia (BOOP) or OP,2,3,9 and usual interstitial
pneumonitis.10 The most common pattern of ILDs in
patients with CVID is LIP, actually OP is quite rare. Up to
now, only two cases of OP with CVID have been reported
in English2,3 and one case reported in Spanish.9 It noted
 Chinese Medical Journal 2012;125(17):3195-3197
that the patients with CVID who exhibit diffuse lung
disease were easily misdiagnosed as pulmonary infection
as our patient.
OP is a pathological feature of various diseases, such as
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infectious disease, autoimmune disorder, drugs,
environmental and occupational factors. Infectious
disease was firstly eliminated in our patient through
cultures and specific staining of the sputum and BALF.
Indeed, there was no evidence of collagen vascular
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disease because RF, ASO, ANA and ANCA were all
negative; nor exposure to fumes, toxins or drugs known
to induce OP. After extensive evaluation of causative
factors, we speculated on the relationship between OP
and CVID in view of two cases reported before.2,3
Although our patient was absent of typical clinical
symptoms of OP, her serial PFTs were consistent with a
severe restrictive pattern and reduced gas diffusing
capacity. Chest CT analysis showed migratory and patchy
consolidations and nodules of the bilateral lungs. Finally,
OP was definitively demonstrated by the pathological
findings.
There is no established guideline for the treatment of
ILDs in CVID. Most commonly, corticosteroids and
immunoglobulins replacement monthly have been used to
treat these patients.1-3,6 There is no established guideline
on dosage and duration of treatment with corticosteroid.
Corticosteroids combined with immunoglobulin
replacement had been used in the two patients of OP with
CVID reported, which resulted in the improvement of
clinical symptoms and radiographic abnormalities.2,3 Of
note, Wislez et al2 reported the patient’s conditions
resolved after nearly 2 years of treatment. Interestingly,
imaging abnormalities of the current case were markedly
improved only after oral prednisone without adding
intravenous immunoglobulins. Thus, the standard therapy
for OP in patients with CVID remains to be evaluated.
In summary, OP associated with CVID is very rare. To
our knowledge, the clinicopathologic spectrum of ILD
with CVID has not been reported in China. Surgical lung
biopsy should be considered in order to exclude
noninfectious diseases, especially in patients with CVID
who exhibit diffuse lung disease.
3197
Acknowledgements: We thank Dr. LIANG Jun and Dr. WANG
Dan-dan for reviewing this manuscript.
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(Received February 16, 2012)
Edited by WANG Mou-yue and LIU Huan