Interstitial pulmonary syndrome in a patient with bronchial

asthma
Abstract
Sarcoidosis is a multisystemic inflammatory disease. The diagnostic is indicated by symptoms and
imaging, but in order to confirm it, a number of tests is necessary. Sometimes determining the diagnosis is
difficult, especially since the disease evolves during a longer period of time.
We present the case of a 66 year old non-smoking female patient, with professional exposure in the
background, who was diagnosed two years ago with bronchial asthma (dry cough, wheezing, pulmonary sibilant
rale, obstructive syndrome) for which she is under long term corticosteroid inhalation and bronchial dilatation
treatment, having a favorable evolution.
Initially, the chest x-ray appeared to be normal, but under further evaluations, bilateral pulmonary
opacity was present, with a tendency to conflate, which grew in size.
Clinically, the patient presented an aggravated dyspnea, initial dry cough with intervals of purulent
sputum and hemoptysis, under treatment with large spectrum antibitotics.
Biologically, a moderate inflammatory syndrome was observed, and through X-ray, multiple
micronodular pulmonary opacities, with a tendency to conflate were also observed ; an increased level of
angiotensin convertive enzyme, positive rheumatoid factor, IDR to 2 units of anergic PPD , negative sputum
exam for acid and alcohol resistant bacilli.
Functionally, the obstructive syndrome with normal FEV1 value, which , during two weeks evolved
towards aggravation, with a decrease in FEV1 from 1.25l to 0.64L. The CT indicated bilateral matte glass
aspect.
Bronchoscopy done with bronchial alveolar lavage underlined a normal number of cells with a slight
lymphocytosis (16,4 %) and neutrophilia, without tumor cells. Pulmonary biopsy indicated gigantic elliptical
non-necrotic granulomatous nodules, which confirm the sarcoidosis diagnosis, reason for which treatment with
metilprednisolon 0,5mg/kg was initiated, with favorable clinical, functional and imagistic evolution.
Key words : bronchial asthma, sarcoidosis, lung biopsy.

Introduction :
Sarcoidosis is a multisystemic disease of unknown etiology, characterized by a granulomatous nonnecrotic inflammation, with multiple localizations, but predominantly pulmonary and mediastinal. Since the
aetiology of sarcoidosis is unclear, the diagnosis is indicated by a series of clinical and radiologic aspects
together with the histological aspect of non epithelial, non- necrotic granulomas. Sarcoidosis is not specific to
a certain geographical area, although some regions have a higher incidence. The prevalence, varying from 10 to
40 cases in 100, 000 inhabitants can be found in North America, southern Europe and Japan, with an increased
number of cases in Sweden, Denmark and the USA.
The etiology of sarcoidosis remains a highly debated subject. Although numerous clinical and
epidemical studies have suggested as a triggering factor the exposure to a series of microbial agents together
with a genetic component susceptible to the development of this disease, this subject remains open to debate.
Although the implication of an autoimmune component is suggested by an high level of specific markers
(antinuclear acids, rheumatoid factor and hypergammaglobulinemia).

The clinical picture in sarcoidosis has a wide variation. Despite the fact that any organ can be affected,
the lung and the mediastinal ganglions are affected in more than 90% of the cases. Patients can be asymptomatic
or can have an acute, subacute or chronical debut of the symptoms. Systemic manifestations such as fever,
fatigue or weight loss appear in more than 20% of documented cases.
The diagnosis is based on numerous aspects such as a suggestive symptomatology for this disease, the
presence of granulomatous non-necrotic affection in the biopsy exam and the exclusion of other granulomatous
diseases.

Clinical case
We will present the case of a non-smoking , 66 year old female patient, which was professionally exposed for
36 years to chemical substances and paints, with a known , 2 year history of bronchial asthma, under long term
corticosteroid inhalation and bronchodilatation treatment, who complained of dry cough, progressive dyspnea
during effort and fatigue for almost 3 months, with a gradual worsening of the symptoms.
A month before she was hospitalized in our department, the patient underwent a thorax abdomen CT with
contrast medium which indicated multiple infracting condensing processes, with an hilar, matte glass
unsystematic aspect in the upper ventral and dorsal segments; it also indicated multiple cylindrical bronchial
ektases without tumor elements.
During hospitalization, the patient had periods of coughing with purulent sputum and hemoptysis which were
treated with a favorable result with wide spectrum antibiotics.
Biologically : the patient had unspecific inflammatory syndrome, ESR (erythrocyte sedimentation rate)
80mm/h, normal level of leukocytes, increased level of angiotensin convertive enzyme - 45,8 U/ml, increased
rheumatoid factor >8 UI/ml. Sputum samples tested positive for Streptococcus Pneumoniae ++++ and the
microscopic exam was BAAR negativ.
Sputum exam did not identify acid and alcohol resistant bacilli, samples being currently worked on.
IDR to 2U of PPD was anergic after 72 hours.
Chest X-ray : the exam indicated numerous micronodular opacities, with costal intensity, diffusely constrasted
and spread in both pulmonary fields, with bilateral enlarged hilar mass.
Complete functional exams : Initially the patient had a respiratory dysfunction with normal FEV1, FEV1/FVC
ratio : 63.02% and normal TLC (total lung capacity). Throughout hospitalization, the patients functional state
evolved to aggravation with a decrease of FEV1 0,64L (44,7% of what was estimated), TLC : 0,95L (53,6% of
what was estimated), and decreased FEV1/FVC ratio.
Date
16.02. 2015
19.02.2015
23.02.2015

FVC
1,98 L (101,5%)
1,87 L (102.6%)
0.95 L (53,6%)

FEV1
1,25 L (78,1%)
1,20 L (80,9%)
0,64 L (44,7%)

Tiffneau index
63.02%
64,11%
67,59%

Electrocardiogram: sinus rhythm, AV=76b/min, QRS:86ms, minor RDB , suggestive ischemia aspect
in DI, of VL, V5, V6.
Echocardiography: right cavities and pulmonary artery wirh no dilatation, without segmental cinetic
abnormalities at interventricular septum level , PAPs 45mmHg, acceleration time AP 56ms. Left cavities of
normal size, left ventricle preserved global systolic function, left diastolic dysfunction delayed relaxation.
The 6 minute walking test : the patient walked a distance of 425 m (90% of estimated), without desaturation
(98% initial 98% final), dyspnea (Borg scale) : 0,5 initial 2 final

Bronchoscopy with bronchial alveolar lavage: dynamic present in the larynx, bilateral sequelar bronchitic
aspect, sequelar bronchitic deformations of primitive calcidied extrabronchial adenopathies distal left,
intermediate proximal, medium lobary is deformed by antracotic scar (possibly tuberculosis). Bronchial alveolar
lavage indicated alveolar machrophages - 76,4%, - lymphocytes -16,4%, granulocytes 7,2%, neutrophilia
6,6%, eosinophilia 0,6%. Bronchial aspiration negative BAAR.
6
Citology total nr of cells 4,2x 10 Result (%)

Normal value (%)

Macrophages
Lymphocytes
Granulocytes
Neutrophiles
Eosinophiles
Epithelian cells

>84
<13
<3
<3
<0,5
-

76,4
16,4
7,2
6,6
0,6
21

In this moment of the diagnosis stagem the diferential diagnosis that can be taken into consideration are:
Infectious causes : pneumonia, tuberculosis
Uninfectious causes : BOOP, sarcoidosis , adenocarcinoma bronchial-alveolar carcinoma.
Because the bronchoscopy with bronchial alveolar lavage did not point out to a certain diagnosis, it was
decided to carry out a pulmonary biopsy, which indicated:
40/20/15 mm lung fragment, on the section there is a relatively well defined nodule, grayish white in
color, with an 11mm diameter and increased consistency.
Lateral limit L1 lung parenchyma fragment containing bronchioles with a slight thickened lining by
the hyperplasia of smooth musculature, slight luminal narrowing, relatively low peribronchial chronical
inflammatory infiltrate, content of mucus and macrophages endoluminal conclusive to chronic unspecific
bronchiolitis
Nodule fragment with pleura lung parenchyma fragment containing gigantic elliptical non-necrotic
granulomatous nodules, with a tendency to peripheral collagenization. Multinuclear gigantic cells contain
occasionally crystal inclusions. In the center of the lesion there is a anthracotic fibrohyaline scleral nodule.
Lateral limit L1 lung parenchyma fragment with unspecific chronic bronchiolitis lesions, parcelar
septal thickening, locally, thick interstitial chronic inflammatory infiltrate, arteries with thickened walls due to
media hypertrophy and intima fibrosis. The Ziehl Neelson colorations did not indicate acid and alcohol
resistant bacilli.
The suspicion of sarcoidosis having been confirmed by the histopathological exam, it was decided to start
corticosteroid therapy with Metilprednisolon 32mg/day. The patient reacted very well to the treatment, the
symptoms were significantly attenuated and the X-ray exam shows the diminution of interstitial
modifications. Currently the dose was reduced to 24mg/day Metilprednisolon until further reevaluation.
-

Discussion
Sarcoidosis is a systemic disease of unknown etiology. Environmental factors are incriminated in
triggering the disease. Sarcoidosis appears in individuals who have a genetical predisposition; a so-called
sarcoid constitution is described in scholarly literature.

As in the case which was described, asthma and sarcoidosis share many basic characteristics , therefore
diagnosing a patient with a known history of asthma with sarcoidosis is difficult, as the symptoms of the two
diseases can be identical . Obstructive ventilatory dysfunction appears in asthma as well as sarcoidosis,
therefore ventilatory function tests are inconclusive to a diagnostic.
There are no specific tests to diagnose sarcoidosis, various tests can only back it up, sarcoidosis remaining an
exclusion diagnostic.
Bronchoscopy has a decisive role in reaching a diagnosis and determining treatment in respiratory tract
sarcoidosis.
It can also exclude diseases which immitate sarcoidosis, thus bronchoscopy more maneuvers can be performed
to positively diagnose sarcoidosis:
Bronchial membrane biopsy
Transbronchial ganglion needle aspiration (lymphatic nodules)
Transbronchial pulmonary biopsy
Bronchioalveolar lavage
With a few exceptions, such as the presented case, in which bronchoscopy with bronchioalveolar lavage
is inconclusive, it is mandatory that the sarcoidosis diagnosis rely on the biopsy results.
The particularity of this case is how sarcoidosis was masked by the presence of asthma through similar
symptoms, ventilatory function tests (which showed respiratory tract obstruction, which is present in both
diseases, reason for which it cannot be exclusively attributed to only one of them), as well as bronchoscopy
with lavage, which did not underline a suggestive cytology, therefore the only conclusive investigation was the
pulmonary biopsy.

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