5. Interstitial lung disease, pulmonary vasculitis

General findings of all interstitial lung diseases (ILD)

- Progressive exertion dyspnea

- Chronic dry cough

- Insidious onset

General PE findings on all interstitial lung diseases (ILD) (+ advanced findings)

- Bibasilar dry rales

- Decreased air movement

- Tachypnea

Advanced: RHF, digital clubbing

General CXR findings on all interstitial lung diseases (ILD)

- Low lung volume

- Diffuse "ground glass" opacities

Are ILD obstructive or restrictive?

Restrictive

Pathophysiology of ILD

Persistent alveolitis → irreversible fibrosis resulting in distal/small airway disease

What part of the airway is spared in ILD?

Airways proximal to bronchioles

What is important about the diagnosis of ILDs?

History

Additional causes of ILD other than primary pulmonary disorders & systemic disorders

Med: Amiodarone

Env: Dusts - asbestos, silica (inorganic), aspergillus (organic)

Lung infections: Fungal

MCC of ILD

Primary pulm disorder: Diffuse interstitial pneumonias

Systemic disorders: AI, sarcoidosis

MC ILD diagnoses

Idiopathic pulmonary fibrosis

Demographic of idiopathic pulmonary fibrosis

5-6th decade, smoking history

How to diagnose idiopathic pulmonary fibrosis?

- Diagnosis of exclusion with THOROUGH history

- CXR/CT

CXR/CT findings of idiopathic pulmonary fibrosis

CT: Diffuse, patchy, progressive fibrosis with pleural-based honeycombing

Prognosis of idiopathic pulmonary fibrosis

Medications can reduce rate of decline, BUT no definitive treatment

Pharmacological management of idiopathic pulmonary fibrosis

Can trial 2 months of oral steroids

How to evaluate idiopathic pulmonary fibrosis suspicion further?

Lung tissue sample

Sarcoidosis

Systemic granulomatous inflammation MC of lung

S/S of sarcoidosis

Pulm: Dyspnea, cough

Skin: Granulomas, erythema nodosum

Eye: Vision changes, dry eyes

PE findings with sarcoidosis

NORMAL lung exam, anterior uveitis, retinitis, lympadenopathy

Diagnostic workup & findings of sarcoidosis

CXR: Hilar adenopathy / parenchymal involvement

Labs: Elevated ESR, calcium, ACE

Biopsy: NONcaseating granulomas

What are parenchymal involvement findings on CXR in sarcoidosis?

Diffuse reticular / focal infiltrates, nodules

What causes an increase in calcium with sarcoidosis?

Granulomas produce excess vitamin D

What is required in all patients diagnosed with sarcoidosis?

Ophthalmologic exam

First-line treatment of sarcoidosis

Long-term high dose oral steroids, tapered for maintenance

Prognosis of sarcoidosis with ILD

Disease can resolve within 2-5 years (generally treatable form of ILD)

What causes aspergillosis?

Aspergillus fumigatus - mold/fungus

Regions where aspergillus is found

Soil, decaying vegetation, HVAC leak

What demographic is most susceptible to allergic bronchopulmonary aspergillosis?

Underlying asthma or CF

What demographic is most susceptible to chronic aspergillosis?

Pre-existing lung damage BUT immunocompetent

What demographic is most susceptible to invasive aspergillosis?

Immunocompromise or acute critical pulmonary

Presentation of allergic bronchopulmonary aspergillosis

Worsening bronchospasm, pulmonary infiltrates

Presentation of chronic aspergillosis

- Aspergilloma mass or chronic fibrosis aspergillosis

- Weight loss, malaise

- Symptoms >3 months

Presentation of invasive aspergillosis

- Severe necrotizing pneumonia, invasive sinus disease, tissue infarction

- Can involved CNS, skin, organ

Diagnostic workup of allergic bronchopulmonary aspergillosis

Eosinophilia and IgE and IgA aspergillus antibody serology

Diagnostic workup & findings of chronic aspergillosis

CXR: Nodules/cavitation

** Must exclude other causes (ie: TB)

Diagnostic workup & findings of invasive aspergillosis

Serum assay for B-D-glucan and serum/BAL for galactomannan

Treatment of allergic bronchopulmonary aspergillosis

Corticosteroid taper + itraconazole

Treatment of chronic aspergillosis

Resection of apergeilloma + very long-term itraconazole

Treatment of invasive aspergillosis

Azole

Pneumoconioses

Chronic fibrotic lung diseases caused by inhalation of inorganic dusts

Prognosis for all pneumoconioses

Supportive

Types of pneumoconioses (3)

- Coal dust

- Silicosis

- Asbestosis

Demographic susceptible to silicosis pneumoconioses

Rock mining, quarry, stone cut, sandblast

Diagnostic findings of CXR with silicosis pneumoconioses

Eggshell appearance of hilar lymph nodes (calcifications)

What should be included in the workup/diagnosis of silicosis pneumoconioses?

TB workup

S/S of asbestosis pneumoconioses

Progressive dyspnea, inspiratory crackles

Diagnostic findings of CXR with asbestosis pneumoconioses

Honeycomb changes at lung bases

Granulomatosis with polyangiitis

AI pulmonary-renal syndrome - small vessel vasculitis

Pulmonary-renal syndrome

Affects BV in lungs & glomeruli

Pathophysiology of granulomatosis with polyangiitis

Anti-neutrophil cytoplasmic antibody medicated

S/S of granulomatosis with polyangiitis

Decreased GFR, purpura, migratory arthritis, ocular

Diagnostic workup & findings of granulomatosis with polyangiitis

- Biopsy of symptomatic tissue

- ANCA serology

- UA

- CXR

- CBC: anemia, mild leukocytosis

General treatment of ALL vasculitis conditions

Initiate steroid treatment ASAP

Treatment of granulomatosis with polyangiitis

High-dose steroids + rituxumab/cyclophosphamide

Rituxumab/cyclophosphamide: PJP prophylaxis due to immunosuppression

Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss)

AI multisysem vasculitis

S/S with eosinophilic granulomatosis with polyangiitis

Pulmonary and skin findings MC: Hemoptysis, purpura, skin ulcerations

Other: Sinusitis with polyps

Diagnostic workup & findings of eosinophilic granulomatosis with polyangiitis

- Positive ANCA

- Eosinophilia

- Tissue sample of eosinophilic infiltrates

Treatment of eosinophilic granulomatosis with polyangiitis

High-dose steroids

Which disease is more concerning for rapid worsening of pulmonary/renal function: gnulomatosis with polyangiitis OR anti-GBM?

Anti-GBM

Anti-GBM (good pasture syndrome)

AI destruction of basement membrane in glomeruli and alveoli

Demographic of anti-GBM? Which has greater lung involvement?

More lung: 30s, M>F

Less lung: 60-70s, M=F

S/S & exam findings of anti-GBM

Hemoptysis, rapid-progressive renal failure

Diagnostic workup & findings of anti-GBM

- CT/CXR: Diffuse bilaterally alveolar infiltrates/pulmonary hemorrhage

- UA: Hematuria, proteinuria

- Serology: Anti-GBM antibodies

Treatment of anti-GBM

IV → PO corticosteroids + cyclophosphamide + plasmaphoresis

What is the purpose of plasmaphoresis for anti-GBM?

Removes circulating autoantibodies

Hemoptysis and pulmonary hemorrhage on imaging is associated with...

Anti-GBM