Case Presentation Related to Pulmonary Function Tests

This slide provides a concise summary of the case, outlining the patient's symptoms, clinical history, diagnostic findings, final diagnosis, and management plan. The case highlights Interstitial Lung Disease (ILD) linked to an autoimmune disorder, emphasizing the need for early detection and tailored immunosuppressive therapy. Prognostic factors and follow-up strategies are also discussed.

1. Case Presentation
Related to Pulmonary Function Tests
Presenter : Dr. Saran A. K.
Preceptor : Dr. Md. Zabihullah
08 March 2025

2. DEPT. OF PHYSIOLOGY, AIIMS PATNA 2

3. Overview
•Case History
•Physical Examination Findings
•Provisional and Differential Diagnosis
•Investigations – Pulmonary Function Tests
•Diagnosis
•Discussion
DEPT. OF PHYSIOLOGY, AIIMS PATNA 3

4. Case History
Biodata
•Name – Kumari Nandita Sinha
•ID – 109112500105154
•Age – 46 years
•Gender – Female
•Occupation – Homemaker
•Address – Patna
•DOE – 5
th
March 2025
•Department - Pulmonary Medicine
DEPT. OF PHYSIOLOGY, AIIMS PATNA 4

5. Presenting complaints
A 46 year old female, who is a housewife hailing from Patna
presented to Pulmonary Medicine OPD with
•Dry cough - 2 years
•Shortness of breath - 2 month
DEPT. OF PHYSIOLOGY, AIIMS PATNA 5

6. History of Presenting Illness
•According to the patient she was apparently asymptomatic 2 years
ago when she developed dry cough, chronic in onset and
persistent.
•It is gradually progressive, not associated with seasonal/
diurnal variation, also not associated with allergic symptoms.
•It interferes with daily activities like household chores, no relief with
medication, no chest pain or other associative symptoms.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 6

7. DEPT. OF PHYSIOLOGY, AIIMS PATNA 7
MRC Dyspnoea Scale | Primary Care Respiratory Society
https://www.pcrs-uk.org/mrc-dyspnoea-scale

8. •Patient also complains of shortness of breath, gradually
progressive.
•Grade III MMRC, stops for breath after walking about 100 meters
(or after a few minutes) on level ground.
•Insidious onset, not associated with seasonal/diurnal variation.
•Relieves on taking rest.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 8
MMRC- Modified Medical Research Council Dyspnea Scale

9. •Patient also complaints of dryness of mouth and dysphagia.
•There is also on and off joint pain (large joints).
•Also, complains of generalised weakness and muscle pain.
•Patient had complaints of fever lasting for 2 months, low grade,
resolved with symptomatic treatment, one year back.
•History of loud snoring, reported by her son. (STOP BANG – 3 points)
DEPT. OF PHYSIOLOGY, AIIMS PATNA 9

10. DEPT. OF PHYSIOLOGY, AIIMS PATNA 10
STOP-BANG Score for Obstructive Sleep Apnea
https://reference.medscape.com/calculator/531/stop-bang-score-for-obstructive-sleep-apnea

11. •There is no history of small joint pain/malar rash/photosensitivity
•No history of recurrent oral ulcers, nail pitting, Raynaud's phenomenon
•No history of dryness of eyes, watering and itching.
•No c/o rhinitis, sore throat, post nasal drip
•No c/o change in voice or hoarseness of voice
•No h/o voice abuse or trauma
•No c/o palpitation, sweating, dizziness, pedal enema
•No c/o difficulty in swallowing or regurgitation
•No history of weight loss

12. Past History
•K/C/O hypothyroidism x 8 years, on medication Thyroxine 75
•On treatment for heart failure since 2020. Coronary Angiogram(2021)
– Normal study
•Recovered cardiomyopathy EF 20% → 65%
•No history of HTN/T2DM
DEPT. OF PHYSIOLOGY, AIIMS PATNA 12

13. No history of
•Childhood asthma
•Allergic rhinitis, nasal polyps, sinusitis
•Chronic obstructive pulmonary disease
•Pulmonary or Extra Pulmonary TB
•Cerebral Vascular accident/ chronic liver or kidney disease
•Connective tissue disorder/ seizure disorder
•Malignancy
DEPT. OF PHYSIOLOGY, AIIMS PATNA 13

14. Family History
•No similar illness in family
Personal History
•Married, 2 children, Homemaker
•Mixed Diet, denies addiction
•Sleep and appetite normal, bowel and bladder habits unaltered.
•No history of TB, malignancy, chronic lung disease in family.
•Owns buffalo at home.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 14

15. Treatment History
•Diagnosed as cardiomyopathy, and treatment was started, is on
drugs. Regency Hospital, Kanpur in 2021.
•In 2023, diagnosed as Interstitial Lung Disease, was on drugs from
September 2023 to March 2024, then discontinued.
•No history of surgery or hospital admission
Menstrual History
•Menarche at age of 13, Regular cycle with 3-4 flow days
•Attained Menopause 2 yrs ago
DEPT. OF PHYSIOLOGY, AIIMS PATNA 15

16. Socioeconomic History
•Upper lower class as per Modified Kuppuswamy’s Scale (2024)
•Resides in semi pucca house with brick walls and asbestos roofing, one room
and small kitchen
•Moderate ventilation, occasional overcrowding
•Cooking done on LPG stove along with use of firewood.
Occupational and Exposure History
•Homemaker
•No pets at home, no history of any exposure.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 16

17. Summary
A 46-year-old female homemaker from Patna presented with dry cough
for 2 years and progressive shortness of breath for 2 months (MMRC
Grade III). Symptoms were insidious in onset, non-seasonal, and non-
allergic. Additional complaints included dry mouth, dysphagia, joint
pain, generalized weakness, and muscle pain, along with a history of
low-grade fever lasting 2 months a year ago and loud snoring. K/C/O
recovered cardiomyopathy, hypothyroidism, HTN and T2DM on
medication.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 17

18. Physical Examination
General Examination
•Conscious, oriented and cooperative
•Well built and well nourished
•Height - 154cm, Weight - 78kg, BMI- 32.8
•No pallor, icterus, cyanosis
•No clubbing, pedal oedema, lymphadenopathy
DEPT. OF PHYSIOLOGY, AIIMS PATNA 18

19. Head to toe examination
•Facial puffiness +
•Rest of skin, hair, nails – normal (No skin lesions or colour changes,
sclerodactyly or digital ulcers)
•No thyroid swelling, varicosities
•No cervical spine tenderness
DEPT. OF PHYSIOLOGY, AIIMS PATNA 19

20. Vitals
•Pulse Rate - 70/min, regular, normal volume and character, no
radiofemoral delay, no vessel wall thickening.
•Respiratory Rate -20/min, regular, Thoracoabdominal
•Blood Pressure- 110/80, right arm supine position
•Temperature - Afebrile
•SpO2- 92% on NRBM (following TLBC), was maintaining on room air
previously.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 20
TBLC – Transbroncial Lung Cryobiopsy

21. Respiratory System Examination
Upper Respiratory Tract
•Nose – Flaring of nasal alae +, Nasal mucosa normal
•No polyp/ congestion/ discharge/ deviated nasal septum
•No local rise of temperature, tenderness, swelling, erythema over PNS
•Oral cavity - adequate oral hygiene
•Dental carries present
•No tobacco staining
•Gum, palate, tonsil, posterior pharyngeal wall - normal
DEPT. OF PHYSIOLOGY, AIIMS PATNA 21
PNS- Para Nasal Sinuses

22. Lower Respiratory Tract Examination
Inspection
•Shape of chest - Normal
•Chest wall - Bilaterally symmetrical
•Trachea appears central
•No supraclavicular fullness/ hollowing
•No drooping of shoulder, rib crowding
•Apical impulse not visible
DEPT. OF PHYSIOLOGY, AIIMS PATNA 22

23. •Respiratory Rate- 20/min, regular rhythm, thoracoabdominal type
•Chest movements equal on both sides
•Use of any accessory muscles +
•No internal coastal indrawing
•No kyphosis/ scoliosis
•No dilated veins/ scars, sinus, visible pulsations
•No evidence of wasting
DEPT. OF PHYSIOLOGY, AIIMS PATNA 23

24. Palpation
•No local rise of temperature
•No chest wall or intercoastal tenderness
•Trachea ? Slight deviation to left
•Apex beat - Left 4th ICS, 1.3cm medial to MCL
•B/L chest movements equal
•Vocal fremitus equally felt bilaterally in all areas
•No e/o rib crowding
ICS- Intercoastal space, MCL- Mid Clavicular Line

25. Measurements
•Chest circumference- 96cm
•Right hemithorax- 48cm
•Left hemithorax- 48 cm
•AP diameter- 24cm
•Transverse diameter-34cm
•Ratio 0.68
•Expansion- 3cm
•Expansion 1.5cm
•Expansion 1.5 cm

26. Percussion
•Kronig’s isthmus - Resonant
•Direct percussion over clavicles resonant
•Resonant note appreciated in all areas of lungs bilaterally
•Liver dullness percussed in Rt 5th ICS in MCL, 7th ICS in MAL and 9th ICS posteriorly
•Tidal percussion- Positive
•Cardiac border
•Right corresponds to Right sternal border
•Left corresponds to Apex beat
Rt- Right , ICS- Intercoastal space, MCL- Mid Clavicular Line, MAL – Mid Axillary Line

27. Auscultation
•Normal Vesicular Breath Sound with equal intensity heard over all areas
•B/L basal fine crepitations +
•Vocal Resonance equal bilaterally in all areas
•No added sounds

28. DEPT. OF PHYSIOLOGY, AIIMS PATNA 28
Oxford Handbook in Clinical Medicine

29. Cardiovascular System Examination
•JVP not elevated
•Pulse Rate- 70/min
•BP- 110/80mmHg
•Apex beat - Lt 4th ICS 1.3cm medial to MCL
•No palpable S2/ left parasternal heave
•S1, S2 heard normally
•No murmur or loud P2

30. CNS Examination
•Normal higher mental functions
•Motor System
•Bulk – Normal
•Tone – Normal
•Power – 4/5 in bilateral upper and lower limbs (proximal muscles),
5/5 in distal muscles.
•Reflexes – Superficial and Deep intact
DEPT. OF PHYSIOLOGY, AIIMS PATNA 30

31. •No cerebellar signs
•Gait could not be assessed
•No involuntary movements
•Sensory System – No deficits
•Cranial Nerves – Normal
DEPT. OF PHYSIOLOGY, AIIMS PATNA 31

32. Previous Reports
•Pulmonary Function Tests (16/10/23) - Restrictive pattern
•FVC = 57%
•FEV1 = 56%
•FEV1/FVC = 82.66%
DEPT. OF PHYSIOLOGY, AIIMS PATNA 32

33. •Provisional Diagnosis : ?Fibrotic ILD under evaluation,
Hypertensive, Recovered cardiomyopathy, Hypothyroidism.
•Differential Diagnosis :
1.ILD-CTD
2.ILD
3.Chronic bronchitis
4.Bronchiectasis
5.Tuberculosis
DEPT. OF PHYSIOLOGY, AIIMS PATNA 33

34. Investigations
Blood Routine Examination
•Hb - 12.1 gm/dL
•TLC- 7960 thousand/micro litre
•DC - N 77%, L 20.6%, E 0%, M 1.4%, B 0.4%
•RBC – 4.40 thousand/micro litre
•PCV – 37.9
Prothrombin Time (PT) and INR
•PPT-13.8 (Reference Range 11-16)
•T-INR -1.02

35. ESR – 61 ↑
URE – Normal
Liver Function Tests
•Bilirubin T/D – 0.52/0.37
•ALT/SGPT - 76.03 ↑
•AST/SGOT – 62.99 ↑
•Globulin – 3.59 ↑
•A/G Ratio 1.16 ↑
Kidney Function Tests
•Urea/Creatinine 36.9/0.79
•Uric Acid 7.92 ↑
•Sodium 130.02 ↓
DEPT. OF PHYSIOLOGY, AIIMS PATNA 35

36. HbA1C – 6.4 ↑
CRP Quantitative – 2.63
HBSAg, HCV and HIV – Non Reactive
Sputum studies
•AFB - negative
•CBNAAT - neg
•Culture & sensitivity - Normal pharyngeal flora
DEPT. OF PHYSIOLOGY, AIIMS PATNA 36

37. Pulmonary Function Tests
•Spirometry (Forced Vital and Slow Vital Capacity)
•DLCO/TLCO
•Body Plethysmography for TLC and RV
•Forced Oscillometry Technique (FOT) for Rrs and Xrs
DEPT. OF PHYSIOLOGY, AIIMS PATNA 37
TLCO – Transfer factor of lung for CO, TLC – Total Lung Capacity, RV- Residual Volume, Rrs-
Resistance of airways, Xrs – Reactance of airways

38. Parameter Predicted Observed (Pre)% of PredictedInterpretation
FVC (L) 2.47 1.35 54.52%
FEV1 (L) 2.06 1.08 52.26%
FEV3 (L) 1.88 1.24 65.77%
FEV1/FVC (%) 83.18 79.90 96.06%
PEF (L/s) 5.21 4.59 88.12%
MEF25 (L/s) 5.21 4.59 88.12%
MEF50 (L/s) 3.44 1.35 39.36%
MEF75 (L/s) 2.49 1.09 49.80%
DEPT. OF PHYSIOLOGY, AIIMS PATNA 38
↓
↓
↓
↓
↓
Normal
Near Normal
Near Normal
Spirometry- Forced Vital Capacity

39. DEPT. OF PHYSIOLOGY, AIIMS PATNA 39

40. Parameter Predicted Observed % of PredictedInterpretation
Tidal Vol (L) 0.67 -
VC (L) 2.04 1.24 60.83%
IRV (L) 0.45 -
ERV (L) 0.91 0.12 13.31%
IC (L) 1.13 1.12 98.93%
EC (L) 0.79 -
Ti/Ttot (sec) 0.62 -
RF (#/min) 26.32 -
DEPT. OF PHYSIOLOGY, AIIMS PATNA 40
↓↓↓
↓
↑
Near Normal
↑
Slow Vital Capacity

41. Diffusion Single Breath (TLCO-sb)
Parameter Predicted Observed % of Predicted Interpretation
TLCO (mL/min/mmHg) 19.27 4.61 23.95%
Va (L) (Alveolar Volume) 3.45 1.65 47.91%
KCO (TLCO/Va) 5.58 2.79 49.99%
VI (L) 2.14 1.18 55.21%
Breath Hold Time (s) 10+/-2 11.78 -
RV/Va (%) 0.00 28.59 - -
TLCO (Hb corrected) 19.27 4.61 23.95%
KCO (Hb) (TLCO(Hb)/Va) 5.58 2.79 49.99%
DEPT. OF PHYSIOLOGY, AIIMS PATNA 41
↓↓↓
↓
↓
↓
↓↓↓
↓
Acceptable ATS/ERS
TLCO – Transfer factor of Lung for CO, KCO – Carbon monoxide transfer coefficient

42. Body Plethysmography- TLC
Parameter Predicted Observed % Predicted Interpretation
TGV (Thoracic Gas Volume) (L) 2.18 1.31 59.86%
TLC (Total Lung Capacity) (L) 3.45 2.29 66.33%
PL-FRC (L) 1.23 - -
RV (Residual Volume) (L) 1.27 1.19 93.64%
RV/TLC (%) 36.88 52.07 141.17%
PL-ERV (Expiratory Reserve Volume) (L) 0.91 0.04 4.75%
PL-IRV (Inspiratory Reserve Volume) (L) 0.63 - -
42
↓
↓
Normal
↓↓↓

43. Resistance
Parameter Predicted Observed % Predicted Interpretation
SGAW (Specific Airway
Conductance) (1/s/cmH₂O)
0.14 0.32 225.77%
GAW (Airway Conductance)
(L/s/cmH₂O)
0.41 0.41 100.43%
SRAW (Specific Airway
Resistance) (cmH₂O*s)
7.04 3.12 44.29%
RAW (Airway Resistance)
(cmH₂O/L/s)
2.44 2.43 99.57%
TGV (raw/L) 2.18 1.28 58.82%
DEPT. OF PHYSIOLOGY, AIIMS PATNA 43
↑
↓
↓
Normal
Normal

44. Forced Oscillation Technique
44
Rrs- Resistance of airways, Xrs – Reactance of airways, ULN – Upper Limit of Normal, LLN- Lower Limit of Normal, Pred- predicted

45. DEPT. OF PHYSIOLOGY, AIIMS PATNA 45
R- Resistance of airways, X – Reactance of airways, ULN – Upper Limit of Normal, LLN- Lower Limit of Normal

46. •Elevated Rrs at 5Hz (compared to predicted) suggests increased airway
resistance, which may be due to airway narrowing, fibrosis, or
inflammation.
•A flat or minimally decreasing pattern as frequency increases suggests
increased homogenous airway resistance, seen in restrictive diseases.
•More negative Xrs (below predicted and near LLN) suggests reduced
lung compliance and increased lung stiffness.
•Both inspiratory and expiratory Xrs are abnormal, with Xrs more negative
at low frequencies consistent with restrictive lung disease.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 46

48. Imaging Studies
•Chest X Ray
•CT Chest
•HRCT Thorax
DEPT. OF PHYSIOLOGY, AIIMS PATNA 48

49. Chest X-Ray
DEPT. OF PHYSIOLOGY, AIIMS PATNA 49
Ill defined irregular shape patchy
radiopacities seen in the left lower
zone and at the left hilar aspect –
possibility of consolidation- likely
atypical pneumonitis changes.

50. CT Scan (08/03/24)
•Focal elevation of R hemidiaphragm
•Few fibro actelectic bands in bilateral lung fields in basal
segments of lower lobe.
HRCT Thorax (16/10/23)
•Patchy areas of consolidation with bronchial wall thickening is
seen in bilateral lower lobes. Few fibrotic bands in apical
portions of bilateral upper lobes.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 50

51. HRCT (Thorax) 27/01/2025
•Left lung lower lobe has a fairly large area of opacity with some
interlobular and intralobular spatial thickening causing
parenchyma distortion/volume loss.
•Right lung upper lobe central and apical patchy opacities
•Enlarged pretrachial, pre carnial, subcarinal, b/l hilar regions
14x6 mm.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 51

52. HRCT Thorax
DEPT. OF PHYSIOLOGY, AIIMS PATNA 52

53. DEPT. OF PHYSIOLOGY, AIIMS PATNA 53
UIP – Usual Interstitial Pneumonia, NSIP – Nonspecific Interstitial Pneumonia, COP – Cryptogenic Organizing
Pneumonia, RB-ILD – Respiratory Bronchiolitis-Associated Interstitial Lung Disease, DIP – Desquamative Interstitial
Pneumonia, AIP – Acute Interstitial Pneumonia, LIP – Lymphoid Interstitial PneumoniA

54. HRCT : Supporting Evidences to PFT
PFT Finding HRCT Finding
Reduced TLC (66%) and FVC (54%) →
Restrictive lung disease.
Severely reduced TLCO (23.95%) → Poor
gas exchange.
No obstruction (FEV1/FVC normal).
DEPT. OF PHYSIOLOGY, AIIMS PATNA 54
Confirms restrictive lung disease, likely interstitial lung disease (ILD).
Parenchymal distortion & volume loss in
lower lobes suggest lung restriction due to
fibrosis or chronic inflammation.
Thickened interlobular septa & opacities
impair oxygen transfer, matching ILD.
HRCT doesn’t show emphysema or airway
thickening, ruling out COPD.

55. DEPT. OF PHYSIOLOGY, AIIMS PATNA 55
Test Use Result
ANA (IFA/ELISA) Autoimmune screening
ENA Profile
Identifies specific
autoantibodies
CCP Antibody RA-specific marker
RF (Rheumatoid Factor)Supports RA diagnosis
HP Panel
Hypersensitivity
Pneumonitis
Serum ACE Sarcoidosis marker
CTD Workup and other Tests
CTD – Connective tissue disorder, ANA – Anti nuclear Antibody, ENA- Extractable Nuclear Antigen,
CCP Ab – Cyclic Citrullinated Peptide, ACE- Angiotensin Converting Enzyme
Speckled 2+ 1:320
Antibody Ku 2+
Negative
Negative
Negative
Negative

56. •Myositis Panel
56

57. Myositis Panel Results
•Myositis Specific Antibody Mi-2 Borderline Positive –
Dermatomyositis associated with neoplasia
•Myositis Associated Antibody –
•Ku Positive – SLE, SSc, Myositis
•PMScl 100 Border line positive – Overlap Syndrome
DEPT. OF PHYSIOLOGY, AIIMS PATNA 57

58. Test Result Reference/Interpretation
ANA (Antinuclear Antibody) ProfilePositive (Speckled, 1:320)
Suggests autoimmune disease (SLE,
SSc, MCTD, Myositis-Overlap).
Ku Antibody Positive
Seen in Systemic Sclerosis (SSc),
Myositis, ILD.
CRP (C-Reactive Protein) 130 mg/L (Elevated)
High inflammation, possibly active ILD
or systemic inflammation.
Rheumatoid Factor (RF) & Anti-CCPNegative
Rules out Rheumatoid Arthritis (RA-
related ILD).
Hypersensitivity Pneumonitis (HP)
Panel
Negative
Not HP-related ILD (supports
autoimmune ILD instead).
Serum ACE (Angiotensin-Converting
Enzyme)
109 U/L (High)
Mildly elevated, seen in sarcoidosis but
not diagnostic alone.
2D Echocardiography Distal IVS & LV Apex hypokinesiaMild cardiac dysfunction
LVEF (Left Ventricular Ejection
Fraction)
50-55%
Borderline normal, needs follow-up for
heart involvement.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 58

59. Electrodiagnostic Tests
DEPT. OF PHYSIOLOGY, AIIMS PATNA 59
•Nerve Conduction Study –
Normal

60. DEPT. OF PHYSIOLOGY, AIIMS PATNA 60
•Electromyography
EMG showed, polymyositis
pattern predominantly seen
in proximal muscles of upper
and lower limb.
CPKMB – Normal

61. 6MWT (6 Minute Walk Test)
•Pre spO2 - 98% RA --> Post spO2 - 95%
•PR - 88/min --> 92/min
•Distance 409.5 metre
DEPT. OF PHYSIOLOGY, AIIMS PATNA 61

62. Reports awaited
•Bronchoalveolar Lavage (CBNAAT, Acid fast Bacilli and Culture and
Sensitivity, Cytology)
•Trans Bronchial Lung Cryobiopsy (TBLC)– Histopathology

63. Final Diagnosis
Interstitial Lung Disease
DEPT. OF PHYSIOLOGY, AIIMS PATNA 63
- Connective Tissue Disorder
probably Overlap Syndrome between Inflammatory
Idiopathic Myositis (IIM) and Systemic Sclerosis (SSc)
“IIM-ILD Overlap Syndrome”

64. ILD in CTD
•Interstitial lung disease (ILD) is a common manifestation of
connective tissue diseases (CTDs)
•ILD can be seen in all CTDs but most commonly in
•Rheumatoid arthritis (RA),
•Systemic sclerosis (SSc), and
•Idiopathic inflammatory myopathy (IIM).
DEPT. OF PHYSIOLOGY, AIIMS PATNA 64

65. Idiopathic inflammatory myopathies (IIM)
•The IIMs encompass a group of autoimmune disorders characterized by a
combination of muscle weakness, rash, and autoantibodies
(designated myositis-specific antibodies).
•Inflammation is central to the three main forms of myositis that are
associated with ILD.
•Dermatomyositis (DM).
•Polymyositis (PM)
•Antisynthetase syndrome (ASS)
DEPT. OF PHYSIOLOGY, AIIMS PATNA 65

66. DEPT. OF PHYSIOLOGY, AIIMS PATNA 66
Pathophysiology of IIM- ILD (MDA5 and Anti Synthetase Syndrome)
Moda M, Yanagihara T, Nakashima R, Sumikawa H, Shimizu S, Arai T, Inoue Y. Idiopathic Inflammatory Myopathies-
Associated Interstitial Lung Disease in Adults. Tuberc Respir Dis (Seoul). 2025 Jan;88(1):26-44.

67. •The prevalence of interstitial lung disease (ILD) varies widely varies from 20
to 80 percent in patients with idiopathic myositis.
•In IIM-ILD, breathing issues resemble idiopathic interstitial pneumonias,
but muscle weakness can worsen dyspnea and increase aspiration risk.
•Pulmonary manifestations can range from subclinical ILD to rapidly
progressive respiratory failure.
•Chest imaging and pathology most commonly show non-specific interstitial
pneumonia and organizing pneumonia patterns.
•Detection of myositis-specific and myositis-associated antibodies can aid in
diagnosis and disease characterization.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 67

68. DEPT. OF PHYSIOLOGY, AIIMS PATNA 68

69. DEPT. OF PHYSIOLOGY, AIIMS PATNA 69
Chaudhry S, Christopher-Stine L. Myositis interstitial lung disease and autoantibodies. Front Med (Lausanne). 2023 Jun
13;10:1117071. doi: 10.3389/fmed.2023.1117071. PMID: 37384043; PMCID: PMC10296774.

70. Overlap syndromes
•Patients with anti-PM-Scl and anti-Ku antibodies may have features of
both PM/DM and SSc, including sclerodactyly, Raynaud phenomenon,
and interstitial lung disease
•DM and PM features + features of systemic rheumatic diseases,
including systemic sclerosis (SSc), systemic lupus erythematosus
(SLE), mixed connective tissue disease, rheumatoid arthritis, and
Sjögren’s syndrome
DEPT. OF PHYSIOLOGY, AIIMS PATNA 70

71. Monitoring for disease progression
Initial Evaluation
•PFT: Within 3 months to assess lung function.
•HRCT: Within 6 months to determine disease extent.
Follow-Up:
•Mild CTD-ILD: PFT every 6 months for the first 1–2 years.
•Moderate-Severe or Progressive ILD: PFT every 3–6 months.
•HRCT: Yearly for the first 3 years to track progression.
•Echocardiogram: Every 1–2 years to assess RVSP & cardiac function.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 71

72. Treatment Strategies
•Medication therapy involves either
•Immunosuppressive and/ or
•Anti-fibrotic therapy
•Depends on the subtype of ILD (i.e. the more inflammatory NSIP and
OP or the more fibrotic UIP).
DEPT. OF PHYSIOLOGY, AIIMS PATNA 72
ILD- Interstitial Lung Disease, NSIP – Non specific Interstitial Pneumonia, OP- Organizing Pneumonia,
UIP – Usual Interstitial Pneumonia

73. Primary Treatment:
•Immunosuppression is the mainstay.
•Aggressive combination therapy for rapidly progressive ILD.
•Stepwise escalation for chronic ILD.
Additional Therapies:
•Antifibrotic agents (Pirfenidone/Nintedanib) for progressive
fibrotic ILD.
•Lung transplantation in end-stage disease.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 73

74. DEPT. OF PHYSIOLOGY, AIIMS PATNA 74

75. Prognosis
•Highly Variable Course – Prognosis depends on the pattern of ILD,
presence of autoantibodies, and response to immunosuppression.
•Rapidly Progressive ILD Has Poor Outcomes – More common in
Anti-MDA5-positive dermatomyositis
•Non-Specific Interstitial Pneumonia (NSIP) Pattern Responds Better
to Treatment than Usual Interstitial Pneumonitis.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 75

76. •Overlap Syndrome ILD Prognosis Varies – Systemic Sclerosis-
Myositis Overlap (SSc-Myositis-ILD) has a worse outcome due to a
high risk of progressive fibrosis and pulmonary hypertension.
•Hypoxia on 6MWT Predicts Worse Outcomes – Desaturation
below 90% during exertion indicates more severe ILD and worse
prognosis.
•Early Immunosuppression and Antifibrotics Improves Survival
DEPT. OF PHYSIOLOGY, AIIMS PATNA 76

77. Summary
77
•46-year-old female homemaker from Patna with a 2-year history of dry
cough and progressive shortness of breath for 2 months (MMRC Grade III).
•Additional symptoms include dry mouth, dysphagia, joint pain, weakness,
muscle pain, and past fever episodes.
•Known case of hypothyroidism, recovered cardiomyopathy, and possible
ILD.
•Physical exam: Bilateral basal crepitations, restrictive lung pattern, no
signs of obstructive lung disease.

78. Diagnostic Findings
•Pulmonary Function Tests: Restrictive pattern with decreased FVC (54%)
and TLCO (23.95%).
•HRCT findings: Lung fibrosis, parenchymal distortion, and volume loss
suggestive of ILD.
•Positive autoimmune markers (ANA, Ku Antibody, Myositis Panel)
indicating connective tissue disorder.
•Myopathic pattern in Electromyography\
Final Diagnosis
Interstitial Lung Disease (ILD) associated with a connective tissue
disorder, likely overlap syndrome between inflammatory myositis and
systemic sclerosis.
DEPT. OF PHYSIOLOGY, AIIMS PATNA 78

79. References
79
1.American Thoracic Society – Connective Tissue Related interstitial Disease Primer
2.Myositis associated Interstitial Lung Disease – UpToDate
3.Murray and Nadel Textbook of Respiratory Medicine
4.Fishman’s Pulmonary Diseases and Disorders
5.Moda M, Yanagihara T, Nakashima R, Sumikawa H, Shimizu S, Arai T, Inoue Y. Idiopathic
Inflammatory Myopathies-Associated Interstitial Lung Disease in Adults. Tuberc Respir Dis
(Seoul). 2025 Jan;88(1):26-44. doi: 10.4046/trd.2024.0072. Epub 2024 Sep 2. PMID:
39219439; PMCID: PMC11704735.
6.Chaudhry S, Christopher-Stine L. Myositis interstitial lung disease and autoantibodies. Front
Med (Lausanne). 2023 Jun 13;10:1117071. doi: 10.3389/fmed.2023.1117071. PMID:
37384043; PMCID: PMC10296774.