This document describes an unusual case of a 25-year-old male with ulcerative colitis who developed bronchiolitis obliterans organizing pneumonia (BOOP) and air leak syndrome. He was initially treated with steroids and azathioprine for his ulcerative colitis. He later developed respiratory symptoms and imaging showed diffuse lung involvement. A lung biopsy confirmed BOOP. He required prolonged ventilation and developed multiple complications including pneumothoraces requiring chest tubes. Despite treatment with various antibiotics, antivirals and immunosuppressants, his condition deteriorated and he ultimately passed away. The case report reviews BOOP, mesalamine-induced pneumonitis, and discusses the patient's complex clinical course.
Difference Between Skeletal Smooth and Cardiac Muscles
Interesting case of Ulcerative Colitis with BOOP
1. An Unusal case of Ulcerative colitis complicated
with bronchiolitis obliterans organizing
pneumonia (BOOP) and air leak syndrome
Dr. Nagu Penakacherla ,MBBS,MEd(USA),
DNB (Family Medicine)
2.
3. • Mr X 25 years old who
is K/C/O Idiopathic
Ulcerative colitis since
august 2014 on irregular
treatment with steroids
& since january 2015 he
is on regular treatment
with tapering dose of
steroids .
• And later he was
initiated on staggered
dose of AZA
4.
5. In April 16th 2015 he
came to OPD and
Chest x ray was done
which was not
significant and CT
thorax showed
underlying left lower
lobe bronchiectatic
features and was
treated with
Augmentin.
6. • In May he came with c/o
breathlessness from 10
days , o/e he was
tachypneic, hypotensive
and unstable , so he was
admitted.
• He was maintaining
oxygen saturation with 5-
6 litres O2/min.
• Blood and Urine cultures ,
H1N1 swab were sent and
was empirically initiated
on Tamiflu and broad
spectrum antibiotics.
7. • Total count and Renal functions were with in normal
limits, LFT showed elevated GGT & Alkaline
phosphatase. 2D Echo Revealed normal. USG
abdomen showed mild hepatosplenomegaly .
8. 1.05.2015
• HRCT (1.05.2015)showed left lower lobe collapse
consolidation, showed increase in extent compared to
previous CT done on 16.04.2015, it also showed
diffuse ground glass opacities involving both the
lungs.
9.
10. • Bone marrow – Revealed Cytopenia .
• There were Super added infections So
Consideration for PCP made us to withdraw
AZA(50mg).
• Then Mesacol was added .
11. • 5/5/2015
• Blood and urine cultures which were done came out
as negative .
• Bronchoscopy and lavage done and sample analysis
was unremarkable.
12.
13. 9/5/2015
• Chest x ray showed fluid overload state and was
managed with IV diuretics. Repeat 2D echo was
normal.
• Antibiotics were escalated to Inj Meropenem,
Fluconazole was added. Repeat blood and urine
cultures were negative.He was persistently
tachypneic, hypoxemia requiring intermittent NIV
and later managed with high flow oxygen with
humidifier.
14. 12/05/2015
• Repeat CT thorax
12/05/2015 showed
extensive diffuse ground
glass opacities, alveolar
opacification invloving
bilateral upper lobes-
features suggestive of
diffuse alveolitis/interstital
pneumonia /?viral /?PCP.
• PCP stain and X-pert MTB
were negative.
• So He was initiated on Tab
Bactrim DS and continued
on steroid replacement.
ANA [IF] was tested
negative.
17. 14/5/2015
• Bone marrow analysis was done on 14/5/2015.
Focal erythroid aggregates are noted with occasional
giant erythroblasts. No viral inclusions are seen. No
granuloma or malignant cells are seen.
• Bone marrow aspiration showed normocellular
marrow with mild megaloblastic erythroid maturation
pattern and occasional giant erythroblasts.
18.
19.
20. • Blood for cytomegalovirus DNA came as positive.
• So Inj gancyclovir was also added. Mesacol was withdrawn, as
patient developed fibrosis .Inj Vit B12 added and steroids
continued.
• Patient had a spike of fever , pan cultures were sent
(19/5/2015) which revealed MDR klebsiella oxytoca in
sputum; Inj colistin was added . On 23rd may , lung biopsy was
planned, he underwent bronchoscopy guided lung biopsy
under aseptic precautions.
• Post biopsy patient developed left sided pneumothorax ; under
aseptic precautions left sided ICD was placed on 24/05/2015
and connected to closed suction. Subsequently patient
developed upper thorax subcutaneous emphysema.
21.
22.
23. • Lung biopsy report revealed
bronchiolitis obliterans organizing
pneumonia.
• C-ANCA , P-ANCA were
negative, lung biopsy was
negative for AFB. Sputum gram
stain on 27/05/2015 revealed
MDR Kleibsiella pneumonia so
Inj meropenem and inj colistin are
being continued.
• Patient was continued to be on
ventilatory support with out any
improvement in P/F ratio ,
antibiotics were continued (Inj
Meropenem , colistin ,
Azithromycin , Tab Bactrim DS).
Adequate nutritional support was
maintained through RT feeds,
significant leak was still persisting
through left ICD.
Presence of granulation tissue in the
bronchiolar lumen, alveolar ducts and
some alveoli, associated with a variable
degree of interstitial and airspace
infiltration by mononuclear cells and
foamy macrophages.
24. • Two trials of HFOV (High frequency oscillation
ventilation ) were given but not tolerated. Shock liver
(raised AST and ALT) were on downward trend.
Patient had a spike of fever on 4/6/2015 for which
repeat pan cultures were sent.
• On 7/6/2015 patient had an episode of sudden
desaturation and hypotension, clinical examination
revealed spontaneous tension pneumothorax on right
side. Right sided ICD was inserted immediately ;
patients hemodynamics stabilized following ICD
insertion. Significant leak was present on Right ICD
suggestive of Bronchopleural fistula.
25.
26. • Patient required minimal inotropic support
(Noradrenaline) to maintain blood pressure .On ABG
P/F ratio continued to decrease (<100) and PCO2
were on higher side(70-80). Lung protective
measures were continued.
• Patients blood pressure was continued to decrease and
requiring high doses of inotropes. Bilateral ICD’s are
insitu and was working adequately. His chest x ray
suggestive of severe ARDS with multiple
pneumatoceles with right partially collapse lung
27.
28. • Due to persistent pyrexia >101 pan cultures were
repeated.
• ET culture grew MDR Klebsiella (CFU-6,00,000),
Urine and blood cultures were negative , but Inj
Meropenem and Inj colistin were continued.
• On 10/6/2015 he was persistently hypoxic (P/F ratio
<100) , hypotensive requiring high dose of
vasopressors. Increased leak on right ICD, lungs were
not expanding with persistent pneumothorax.
Requiring 100% of FiO2 , saturating 82-85%.
Gradually his condition deteriorated and patient was
declared dead on 11/6/2015.
37. BOOP
• Bronchiolitis obliterans with organizing pneumonia (BOOP) is a
rare lung condition in which the small airways (bronchioles) and air
exchange sac (alveoli) and the walls of small bronchi become
inflammed and plugged with connective tissue.
• 6 out of 100, 000 hospitalizations.
• It usually starts with a flu-like will be persistent and weight loss
occurs in about half of patients.
• This condition is a form of interstitial pneumonia of unknown
origin. Very similar interstitial pneumonias can be seen in
association with connective tissue diseases such as lupus
erythematosis, several drug exposures and malignancies.
38. • Lung biopsy is recommended for confirmation.
Pulmonary function tests are nonspecific.
• The course of the disease is variable however it tends to
be persistent and not self limited.
• Current therapy involves relatively high doses of
corticosteroids [e.g. prednisone] for several months
depending upon the response. Other
immunosuppressive drugs [e.g. cyclophosphamide]
may also be used.
• Treatment usually but not always results in significant
improvement. However recurrences are common and
patients should be periodically monitored with chest
radiography, especially in the first year after treatment.