chest imaging ii. interstitial lung diseases -...

Chest imaging II. Interstitial lung diseases

Dávid L. Tárnoki MD, PhD

Ádám D. TárnokiMD, PhD

Department of Radiology

Semmelweis University

Topics

1. Interstitial lung diseases 2. Occupational lung diseases 3. Pleura

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Interstitial lung diseases

3

Anatomy


Radiologyassistant; Webb WR, Radiology 2006;239

Secondary lobule: 1 - 2,5 cm ø Centrilobular artery: 0,5-0,7 mm ø Centrilobular bronchiole (wall: 0,15 mm)

bronchiolus terminalis terminal ducts acini alveoli

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Anatomy


Lynch PJ, Creative Commons, http://keywordsuggest.org/content/282562-lung-interstitium.html

Interstitium: • Pulmonary arteries and veins • Lymph vessels, lymph nodes • Nerves • Fibroblasts • Collagen fibers • Elastic fibers

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Pathogenesis - Overview


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(aerogenous, circulatory) Noxen

Damage of the alveolar /from circulation/

endothelial membrane

Granuloma

Remodeling

Fibrosis

Inflammation cells from

circulation / interstitium

(activation and

recruitment)

Pathogenesis - Overview


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Reduction of the

Diffusion constants (in anemia)

Alveolar block

(real diffusion disorder)

Reduction of the capillary

diffusion area

Reduction of the

alveolar space

GGO

Reticulation

Honeycombing

Definition


• Caused by chronic inflammation of the pulmonary interstitium with a scarred reconstruction of connective tissue proliferation due to different causes

• Over 150 triggers (allergens, Medicines, etc.) • Half of the cases remain unclear

• Mostly affected alveoli and interstitium

• Airways and pulmonary vessels are often secondary affected

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Clinics


• Unproductive cough

• Stress dyspnoea

• Drumstick fingers

• Clubbed (Watch glass) nails

• Basal crackling

• Weakness, weight loss

• Full history required:

- Medicines

- Job

- Hobbies

- "pleasure poisons"

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Diagnostics


Ideally by multidisciplinary ILD board: pneumologists, rheumatologists, radiologists and pathologists • Laboratory: - Kidney functions, electrolytes, CRP - Differential blood counts - Antibodies • Lung function (Restrictive ventilation fault) • Imaging (Rö and HRCT) • Bronchoscopy with transbronchial biopsy (TBB) and bronchoalveolar lavage (BAL) • Open lung biopsy (VATS)

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Clinics


What kind of information the clinician needs from us? – Diagnosis

• UIP (Usual interstitial pneumonia) → biopsy is not necessary • Other DD? → biopsy is necessary

– Prognosis • acute / cronic • Comparison with previuos HRCT: progression / regression?

Therapeutic options - Steroids - Lung transplantation

Prognosis - Median survival after diagnosis 2.5-3.5 years

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Typical patterns of an ILD

Ground glass opacity, consolidation


Ground glass opacities

(Milk glass opacity - increased

parenchyma decreasement)

→ partially filled and / or

collapsed alveoli

e.g. active inflammation

- Consolidation

→ completely filled and / or

collapsed alveoli

(Accumulation of exudate,

transudate, or other tissue in the

alveoli)

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Ground glass opacity (GGO) Consolidation

Ground glass opacity, consolidation


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Consolidation GGO

Idiopathic pulmonary fibrosis (heterogeneous entity)


• AIP (acute interstitial pneumonitis)

• UIP (usual interstitial pneumonitis) 70%

• DIP (desquamative interstitial pneumonia)

• RBILD (respiratory bronchiolitis ILD)

• NSIP (non specific interstial pneumonia)

• BOOP=COP (bronchiolitis obliterans organizing pneumonia = cryptogenic organizing pneumonia)

Why do we need to subtype the idiopathic interstitial Pneumonias? Different prognosis which results different therapeutic approaches

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X-Ray


- Fine-stripping-reticular pattern that is superimposed by a diffuse transparency reduction - Increased reticulation with basal dominance

NORMAL FIBROSIS 15

HRCT


Acut Alveolitis: GGO → the location

of optimal bronchoscopic tissue collection

Chronic: Nodules, lines and bands, pleural thickening Final stadium: Honeycombing

„Beehive pattern"

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Plays a central role in detection, diagnosis and differential diagnosis Sufficiency of assessability with a layer thickness of 1.25 mm or less

Basic pattern in HRCT


linear reticular pattern

nodular pattern

Structures with density increase (Lesions with „more air")

Structures with density reduction (Lesions with "less air")

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ILD classification according to ATS / ERS


18 http://err.ersjournals.com/content/22/128/158

1. AIP (acute interstitial pneumonia)


Acute alveolitis: milk opacity (density increase with still recognizable bronchovascular structures)

"idiopathic form of ARDS" / ARDS of unclear cause

Acute and short prognosis

HRCT image: Acute: - homogeneous GGO (alveolitis) - diffuse consolidation Chronic: honeycombing, fibrosis

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2. NSIP (Non-specific interstitial pneumonia)


Chronic form Manifestation: 4. Life decade Not tobacco-associated HRCT Image:

- GGO

- Peripheral, basal, subpleural, symmetrical

fine reticular condensation, traction bronchiolectasis

- no honeycomb pattern (no honeycombing)

Usually occurs as a pulmonary involvement in the context of collagenosis Importantly, the NSIP. can be treated with systemic glucocorticosteroids → prognosis more favorable than with UIP

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3. UIP (Usual Interstitial Pneumonia)


Simultaneity of inflammation, proliferation and fibrosis HRCT is very specific: - GGO (signs of active inflammation and fibroblast proliferation) - Honeycomb patterns (honeycombing), bronchiectasis as signs of fibrosis (not present in NSIP!) - subpleural and basally stressed localization - Reticular pattern - Lack of changes that are not associated with UIP are compatible.

Prognosis: poor

manju-imagingxpert.blogspot.com

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Probability

a correct diagnosis

of a UIP at nearly

100% with HRCT

(http://www.szote.u-szeged.hu/radio/mellk1/mellk7a.htm)

Severe pulmonary parenchymal changes


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Normal or pathologic image?

Fibrosis

http://www.szote.u-szeged.hu/radio/mellk1/mellk7a.htm




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Honeycombing: subpleural localized, multi-rowed, small cystic changes

Upper lobes: signs of activity (GGO, alveolitis)

Lower lobes: honeycombing

Regardless of the cause

End stage: honeycomb lung


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Lung fibrosis: honeycombing pattern


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Patients with a typical UIP pattern in HRCT: does not need lung biopsy

DD: Fibrosis after radiation (due to breast cancer) 43 year old woman


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HRCT

3D reconstructions Lung volumen Pattern softwares


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UIP (Usual Interstitial Pneumonia)


Diaphragm movement disorders - ultrasound M-mode

Complication:

shrinking lung

http://journal.publications.chestnet.org/article.aspx?articleid=1089629 28

4. Collagenoses and vasculitides


Pulmonary manifestations: - Rheumatoid arthritis (RA) - Systemic Lupus Erythematosus (SLE) - Sjögren syndrome - Systemic sclerosis / scleroderma - Dermatopolymyositis - Wegener granulomatosis X-ray: less sensitive In pronounced cases: Basal-dominant reticulonodular pattern HRCT: Patological findings in 30% pleural thickening Late stage: fibrosis What will the clinician ask from the radiologist? Disease characterization and expansion. UIP or NSIP patterns?

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Occupational lung diseases

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ILD classification according to ATS / ERS


31 http://err.ersjournals.com/content/22/128/158


• New diseases due to occupational regulations • Chronic inhalation of inorganic dusts (e.g., silicate) • Long-time exposure (e.g., mineral workers) • Alveolar phagocytosis of inhaled particles and interstitial deposition

→ Interstitial reticulo-granuloma formation, sometimes massive

fibrosis • Therapy: Exposure stop

I. DISEASES OF IMMUNOLOGICAL / UNCLEAR AETIOLOGY II. PNEUMOCONIOSIS (Inhaled Particles)

• X-Ray:

• Nodular herd often with calcifications • Hilary / mediastinal lymph nodes with calcifications ("laryngeal calcification")

HRCT: • X-ray patterns + • Micronodular lesions • pulmonary fibrosis 32

1. Organising pneumonia (OP)


Idiopathic form: Cryptogen-organizing pneumonia (COP) Non-infectious inflammation that may occur in collagenous diseases, chronic eosinophilic pneumonia, exogenous allergic alveolitis, infections or even as a medication reaction • Symptoms: chronic subfebrile temperatures, dyspnoea and cough

• HRCT: Band-shaped, subpleural and peribronchial stressed focal consolidations with basal predominance - migrating consolidations

• Atoll sign: by a ring-like consolidation with central milk glass

33 Prosch H. Journal für Pneumologie 2015; 3 (1), 24-27

I. DISEASES OF IMMUNOLOGICAL / UNCLEAR AETIOLOGY

2. Exogen Allergic Alveolitis, EAA („Farmer

lung”, Hypersensitivity pneumonitis, HP)


• Immune reaction of the alveoli and bronchioles • Inhalation of organic dusts (actinomycetes,

aspergilli, excrements, flour), chemotherapeutic agents

• Anamnesis!

• X-ray: normal in the acute / subacute stage

• HRCT: • Acute / subacute stage:

• Milk glass infiltrates (centrilobular) • reticular pattern • bronchial wall thickening

• Chronic stage: • Fibrosis (X-Ray)

I. DISEASES OF IMMUNOLOGICAL / UNCLEAR AETIOLOGY

34 Prosch H. Journal für Pneumologie 2015; 3 (1), 24-27

3. Silikosis


• Inhalation of mineral dusts (e.g., SiO2, coal) • CT: - Nodules - Hiliary and mediastinal lymphadenopathy Eggshell calcifications - Complicated pneumoconiosis: fibrosis

II. PNEUMOCONIOSIS (Inhaled Particles)

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4. Asbestosis


• Inhalation of asbestos fibers

• Manifestation 20-40 years after exposure

CT:

- interlobular septal thickening

- Honeycomb pattern (honeycombing)

- Pleural plaques

• Malignisation: pleural mesothelioma,

bronchial carcinoma

II. PNEUMOCONIOSIS (Inhaled Particles)

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Asbestosis


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Community Houses

Pleura

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Pleura

Ultrasound

Pleura lines

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Artefacts:

B Lines

In ILD patients pleural effusion is not typical. Pleura irregularities!

Pleura

Mesothelioma - 5-10% of all asbestosis cases develop with a latency of 20-30 years - High-risk groups: construction workers, carpenters, electricians, vehicle workshops - Clinical: chest pain, dyspnoea, weight loss, sometimes fever - Infiltrations of the thorax wall and the ribs come before survival is an average of 18 to 28 months - X-Ray, CT: elongated or glandular pleural thickening, thickness over 10 mm pleural calcifications

X-Ray CT US

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Summary

• For the characterization of interstitial changes, the Computed tomography (HRCT) is the

method of choice • None of the samples presented is specific for a

disease (UIP only) • The suspicious diagnosis results from the

morphology, the distribution of findings, the dynamics and the clinic

→ discussion with the clinicans (!)

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Summary

1. What kind of information needed for the clinicians?

- Diagnosis of the ILDs, biopsy, alveolitis or fibrosis patterns 2. X-Ray: Fine-striated reticular pattern, basal dominance 3. HRCT-Signs: Acute inflammation (alveolitis): GGO Chronic: Honeycombing 4. Difference between NSIP and UIP: NSIP: no honeycombing UIP is very specific: honeycombing

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Summary

5. Consequences of asbestosis: pleural mesothelioma, bronchial carcinoma 6. X-ray and CT signs of sarcoidosis: Bilateral hilar and mediastinal lymphnodes, intrapulmonary changes.

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Thank you!

E-mail: [email protected]

Thank you for the images: Dr.

Monostori und Dr. Judit Pápay

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„You only see what you know“ (Johann Wolfgang von Goethe)

chest imaging ii. interstitial lung diseases -...

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