GRAND ROUNDS

Mander L. Cambonga, MD
Adult Pumonology Fellow

Objectives
To present a case of a 58-year old female
presenting a chronic cough.
To provide a general approach to diagnosis
along with the basic principles of
management of Interstitial Lung Disease.

History

General Data
F. D.
58-year old female
Married
former dressmaker
Filipino
Roman Catholic
residing at Pampanga

Chief Complaint

cough

History of Present Illness

Six years
prior to
admission

cough
no fever, DOB
consult: Pneumonia
unrecalled antibiotic
slight improvement

History of Present Illness

Five years
prior to
admission

distressing on and
off nonproductive
cough
exertional dysnea
admitted: sputum
AFB, CXR,, Chest CT
scan
t/c:Interstitial Lung
Disease
Given Prednisone

History of Present Illness

Interval
History

distressing on and
off nonproductive
cough
exertional dysnea
admitted: sputum
AFB, CXR,, Chest CT
scan
t/c:Interstitial Lung
Disease; to consider
Idiopathic Pulmonary
Fibrosis

History of Present Illness

One week
prior to
admission

distressing on and
off nonproductive
cough
easy fatigability
shortness of breath
no chest pain, no
hemoptysis

Past Medical History

(+) Hypertension, diagnosed 5 years ago, maintained
on Telmisartan 40 mg/tab OD
(+) Diabetes Mellitus, diagnosed 5 years ago,
maintained on Insulin Glargine
No Bronchial asthma
No history of PTB
No allergy to food or drugs
No previous operation
No Connective tissue disease

Family History
(+) Diabetes Mellitus
No Hypertension
No bronchial asthma
No history of malignancy
No COPD
No Connective tissue disease

Personal and Social History

Non smoker
non- alcoholic beverage drinker
never travelled overseas
had no pets or any exposure to birds
no history of illicit drug use
previous dressmaker
no exposure to metal dusts, wood dust, asbestos

Review of Systems
Constitutional: (-) decrease in appetite, (-) weigh loss
Skin: (-) itchiness, (-) excessive drying and sweating, (-) cyanosis, (-)
jaundice (-) rashes
Head: (-) headache, (-) vertigo, (-) facial swelling
Eyes: (-) pain, (-) photophobia, (-) blurring of vision
Ears: (-) earache, (-) deafness, (-) tinnitus, (-) ear discharge
Nose and sinuses: (-) nose bleeding, (-) nasal obstruction, (-) pain
around paranasal area
Mouth and throat: (-) numbness of the tongue, (-) toothache, (-) gum
bleeding,
(-) disturbance in taste, (-) sore throat, (-) hoarseness

Review of Systems
Neck:(-) pain, (-) limitation in movement
Gastrointestinal: (-) epigastric pain, (-) nausea, (-) dysphagia, (-) diarrhea,
(-) constipation,
(-) hematemesis, (-) melena, (-) hematochezia
Genitourinary: (-) dysuria, (-) frequency, (-) anuria, (-) hematuria, (-)
incontinence,
(-) genital discharge, (-) urethral discharge
Extremities: (-) stiffness, no cyanosis,(-) joint pain/swelling
Hematopoietic System: (-) bleeding tendency, (-) easy bruising
Endocrine System: (-) intolerance to heat and cold, (-) polyuria, (-)
polydipsia, (-) polyphagia
Neurologic: no numbness, no loss of sensation

Physical Examination
General Survey: conscious, coherent, in respiratory
distress
BP= 110/70 mmHg
T= 36.5C
Ht= 1.6 m
BMI= 21.48 kg/m2

CR= 85 bpm

RR= 24 cpm
Wt= 55 kg

sPO2= 94% at room air

Physical Examination
SKIN: Body hair is black, fine, scanty and evenly distributed.
HEENT: Palpebral fissures are normal with no lid edema, no ptosis. Pink
palpebral conjunctiva, anicteric sclera. Lips are pink and dry with no
lesions. Buccal mucosa and gums are pink with no signs of swelling or
lesions.
NECK: No mass. No palpable lymph nodes.
HEART: adynamic precordium, normal rate, regular rhythm. The apex
beat is at the Left mid axillary 5 th ICS, no murmur
CHEST/LUNGS: Symmetrical chest expansion, no retractions, lungs
resonant, (+) crackles bilateral ABDOMEN: flabby, soft, non-tender,
tympanitic, normoactive bowel sounds.
EXTREMITIES: full and equal pulses, no edema. (+) clubbing of fingers

Admitting Impression
Intersitial Lung Disease
Type 2 Diabetes Mellitus
Hypertension Stage I

Course in the ward

On admission:

S/O: stable VS, (+) cogh, SOB

ABG pH 7.438 pCO2 38.0 pO2 67.2 HCO3 25.3 CO2 26.4 BE 1.7
O2 sat 93.4
P: 2lpm via nasal cannula
Enoxaparine 0.6 SC q12h
Warfarin 5mg/tab, 1 tab OD
Hydrocortisone 100 mg IV 12
Omeprazole 40mg/cap 1 cap OD
N-acetylcysteine 600mg tab in glass of water BID

Course in the ward

2nd hospital day:
S/O: stable VS; (+) SOB (+) crackles bibasal
P: for chest CT scan with contrast

Course in the ward

3rd hospital day:
S/O: stable vital signs, (+) cough, shortness of breath
and exertional dysnea, (+)hematuria
P: Clexane and warfarin were discontinued.
Hemostan was given

Course in the ward

4th hospital day
S/O: decreased frequency of coughing and shortness of
breath
P: for 6MWT

Course in the ward

5th hospital day:
S/O: stable VS; lesser episode of coughing
P: patient was dischaarged

Discussion
Interstitial lung disease(ILD), also known asdiffuse
parenchymal lung disease(DPLD), is a group oflung
diseases affecting the interstitium (the tissue and space
around theair sacs of the lungs).It concernsalveolar
epithelium, pulmonarycapillaryendothelium,basement
membrane perivascularandperilymphatictissues.

How do we
diagnose patient
s with ILD?

CLINICAL HISTORY

Demographics
The patients age, cigarettesmoking
gender may provide important clues.

status

and

CLINICAL HISTORY
Genetics
13% of patients with IPF
619% of patient with sarcoidosis
Examples of genetic ILDs: HermanskyPudlak
syndrome; Gauchers disease lysosomal storage
disorders; hypocalciuric hypercalcemia and tuberous
sclerosis complex (TSC) (associated with LAM)

CLINICAL HISTORY
Exposures (occupational, hobbies, home environment)
miners (pneumoconiosis)
sandblasters and granite workers (silicosis)
welders, shipyard workers, pipe fitters, electricians,
automobile mechanics (asbestosis)
farm workers, poultry workers, bird fanciers, bird breeders
(HP)
workers in aerospace, nuclear, computer and electronic
industries (berylliosis)

CLINICAL HISTORY
Pulmonary symptoms
A detailed history is the key to diagnose ILD.
Common presenting pulmonary symptoms are
progressive cough (mainly dry), persistent progressive
shortness of breath or dyspnea.

CLINICAL HISTORY
Pulmonary symptoms
disease chronology:
(1) acute, days up to a few weeks
(2) subacute, 4-12 weeks
(3) chronic, longer than 12 weeks

CLINICAL HISTORY
Pulmonary symptoms
Dyspnea
most common symptom
recent change in the degree of dyspnea is linked with
the disease severity and prognosis
insidious onset of symptoms: IPF, PLCH and ILD
associated with connective tissue disease (CTD)

CLINICAL HISTORY
Pulmonary symptoms
Cough
second most common symptom
earliest symptom
dry cough in IPF, sarcoidosis, HP, or COP

CLINICAL HISTORY
productive cough - exacerbation of IPF, pneumonia in
ILD and associated bronchitis or traction bronchiectasis.
chronic irritable cough - lymphangitic carcinomatosis;
mucoid or salty sputum - bronchioloalveolar cell
carcinoma.
wheeze - ChurgStrauss syndrome, HP (pigeon breeders
lung), or airwaystenotic sarcoidosis

CLINICAL HISTORY
Pulmonary symptoms
Hemoptysis
diffuse alveolar hemorrhage (DAH) syndromes such as
Goodpasture syndrome or Wegener disease

CLINICAL HISTORY
Pulmonary symptoms
Pleuritic chest pain pneumothorax LAM, PLCH,
NF and catemenial syndrome
Pleural effusion collagen vascular disease (RA or
SLE) associated, asbestosrelated and drug induced ILD

CLINICAL HISTORY
Extra pulmonary symptoms
dyspepsia or gastroesophageal reflux disorder
scleroderma related ILD
aspiration or dysphagia scleroderma or mixed
CTD
lower gastrointestinal symptoms may suggest
inflammatory bowel disease.

CLINICAL HISTORY
Extra pulmonary symptoms
arthritis CTD or sarcoidosis; combined muscle and
skin symptoms suggest polydermatomyositis
lupus pernio - sarcoidosis
albinism - HermanskyPudlak syndrome

CLINICAL HISTORY
Extra pulmonary symptoms
Neurologic symptoms (cranial nerve involvement,
Bells palsy) vasculitis or sarcoidosis
Polyuria and polydyspsia of diabetes insipidus
sarcoidosis or PLCH.
Hematuria pulmonaryrenal syndromes

PHYSICAL EXAMINATION
Pulmonary signs
Crackles Velcrostrap, basilar, are detected in more
than 80% of patients who have IPF.
Crackles despite a normal chest
granulomatous ILDs (e.g., sarcoidosis)

radiograph

Midinspiratory highpitched squeaks bronchiolitides

and other diseases with airwaycentered pathology
(e.g., HP)

PHYSICAL EXAMINATION
Extra pulmonary signs
Clubbing IPF, asbestosis, chronic HP and DIP

DIAGNOSTIC TESTS
Antinuclear antibody (ANA), rheumatoid factor (RF)
should be obtained in the setting of history or physical
findings that are suggestive of CTD. Low titers of ANA
(1:160) and RF occur in 1020% of patients who have
IPF.

DIAGNOSTIC TESTS
Proximal muscle weakness or tenderness should prompt
measurement of aldolase, creatine kinase, antiJo1
antibody and possibly an electromyogram and muscle
biopsy to rule out polymyositis

DIAGNOSTIC TESTS
Pulmonary function testing
PFTs cannot diagnose a specific ILD and cannot
distinguish between active lung inflammation versus
fibrosis
assessment of respiratory symptoms as well as in
paring the differential diagnosis, grading the severity of
disease and monitoring response to therapy or
progression

DIAGNOSTIC TESTS
Pulmonary function testing
PFT pattern in ILD is a restrictive lung defect
decreased lung volumes (total lung capacity [TLC],
functional residual capacity and residual volume [RV]
80% predicted)
decreased forced expiratory volume in 1 s (FEV1) and
FVC decreased in parallel with a normal or elevated
FEV1/FVC ratio; and decreased DLCO

DIAGNOSTIC TESTS
Chest radiograph: Useful diagnostic patterns
Diffusely abnormal chest radiograph is often the initial
finding that alerts the physician to the possibility of ILD.
Classification of abnormalities based on distribution,
location and overall appearance are useful in narrowing
the differential diagnosis

DIAGNOSTIC TESTS

DIAGNOSTIC TESTS
HRCT
sensitive test in identifying ILD (sensitivity >90%)
the image pattern of parenchymal abnormalities on
HRCT often suggest a particular set of diagnostic
possibilities

DIAGNOSTIC TESTS

DIAGNOSTIC TESTS

DIAGNOSTIC TESTS

DIAGNOSTIC TESTS
Exercise testing
6min walk test guide diagnostic and therapeutic
interventions and direct the use of oxygen therapy.
A cardiopulmonary exercise testing

DIAGNOSTIC TESTS
Bronchoscopy
FOB with BAL or TBLBs may substantiate specific
diagnoses in some patients (e.g., sarcoidosis, LCG, LAM,
CEP, COP)
BAL may be adequate to diagnose specific infections
(e.g., tuberculosis, histoplasmosis, coccidioidomycosis,
endemic fungal infections)

 Surgical Lung Biopsy (SLB)

three purposes:
(1) establishes a precise diagnosis
(2) assesses the extent of inflammation and fibrosis
(3) identifies a histopathological pattern (i.e., IIP)
not warranted in debilitated or elderly patients

Management and treatment of IPF

no therapy proven to improve survival or modify the

clinical course of IPF
High-dose steroid monotherapy (0.51 mg/kg) does not
improve survival or otherwise modify the clinical course
of the disease and is associated with significant
morbidity.

Management and treatment of IPF

Prednisolone with azathioprine and Nacetylcysteine has

better treatment effect than prednisolone and
azathioprine alone
further studies are required

 Pirfenidone
anti-inflammatory, anti-oxidant, and anti-fibrotic
ASCEND study, showed significantly reduced decline in
lung function and IPF disease progression

Management and treatment of IPF

Nintedanib
an investigational orally-administered triple angiokinase
inhibitor that targets receptor tyrosine kinases involved
in the regulation of angiogenesis: fibroblast growth
factor receptor (FGFR), platelet-derived growth factor
receptor (PDGFR), and vascular endothelial growth
factor receptor (VEGFR)

Thank you