Dominance of lung

Dr.T.V.Rajagopal
Fellow,
Dept of Pulmonary Medicine
• A 21 year old female,homeopathic doctor,
presented to Pulmonary medicine OPD
in 2007 with
C/O Breathlessness X 2 years-Progressive
Dry cough X 2 years
-Took homeopathic medicine and improved
symptomatically
-Had come with recurring symptoms for 3 months
No h/o of fever/hemoptysis/chest pain
No h/o of loss of weight or appetite
No h/o of recurrent childhood respiratory illnesses
No h/o skin rashes/joint pain/skin tightening
No h/o exposure to birds

2005-HRCT thorax was done and was diagnosed elsewhere as

ILD
O/E: No pedal edema/JVP elevation
B/l squeaks heard
S1,S2 heard
no murmurs
Radiology
• Xray chest- bilateral lower zones subtle reticular
opacities
• 2D echocardiography- no pulmonary
hypertension
• ANA and RA factor negative
• Spirometry – FVC 1.31L(44% predicted)severe
restriction
• DLCOc 25% pred
• 6MWT showed no desaturation
• Bronchoscopy-TBLB was non diagnostic
 Differential diagnosis
HRCT findings:
Basal predominant reticulations and GGO
Tractional bronchiectasis
No honeycombing

-NSIP pattern
-Idiopathic mixed NSIP
 Standard treatment protocol in 2008
• Azathioprine+Corticosteroids+N-acetyl cysteine
• Tapered off over 3 years and stopped in March
2011
• Disease was Clinically-Physiologically-
Radiologically stable in 2011
• Was continued to be followed up regularly with
Chest xrays/PFT/ANA Stable till 2016
• On every visit she was carefully enquired about
CTD symptoms and examined for the same
• 2012: ANA/RA factor/CENP/ANCA/Anti-
U1RNP/Scl-70/ds-DNA were all negative
• 2016: All above work up along with anti-Jo/anti-
Ro antibody were repeated and found to be
negative
• Every 6 monthly follow-up with lung
function/xray chest and 2 yearly HRCT thorax was
done
• Clinically-Physiologically-Radiologically diasease
was stable from 2012 to 2016
 2016
• Had significant desaturation and was initiated
on long term oxygen therapy
• 2D echo showed no pulmonary hypertension
• No onset of any new symptoms
• In view of increasing fibrosis in previous areas
of ground glass opacities
• Was initiated on pirfenidonecontinued at a
lower dose as she did not tolerate higher
doses
Previously noted areas of
ground glass opacities,
New areas of fibrosis
established fibrosis
involving both upper lobes
predominantly in the lower
lobes
 April 2019
• New onset pulmonary hypertension was detected
• 2D echo RA and RV dilatation with pulmonary
hypertension
• Extended CTD workup was initiated in view of rapid
worsening of pulmonary hypertension(eventhough
symptoms were absent)
Myositis profile was positive
(RO-52 and PL-12)
EMG Motor sensory axonal neuropathy of lower limbs
CPK/LDH negative
• Diagnosis of “lung dominant CTD”-probable
Anti synthetase syndrome was diagnosed and
initiated on MMF + corticosteroids
• Current FVC is 0.7L(24% predicted)
 Lung dominant CTD
• Lung dominant CTD (Proposed criteria) as a
separate entity
-ILD patterns in HRCT(UIP/NSIP/OP/LIP)
-Absent extra-thoracic findings or insufficient to
meet the criteria for any CTD
-Autoantibody positivity
 Literature
points
• Lung dominant CTD(LD-CTD) are being
increasingly reported with the development of
sensitive and specific autoantibody assays
• Median follow up of ILD for about 3 years usually
yielded the diagnosis of underlying CTD
• Had worse survival when compared to CTD
• LD-CTD are common in Idiopathic Inflammatory
myopathy(about 50%) but the latency of CTD
symptoms is unknown
 Specific learning points
• Follow up of 15 years has led to the diagnosis of
CTD(Previously reported upto a maximum of 5 years
before a definite diagnosis of CTD)
• Auto antibodies are positive but the patient still has no
CTD features
• Patients with rapid deterioration in terms of pulmonary
hypertension warrants active search and extended
work-up of CTD even in the absence of symptoms
• Young females with no exposure to known causes of
ILD - CTD features and workup needs to be assessed
frequently
References