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Interstitial Lung Disease and Vasculitis186
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• Nasal or oral inflammation (e.g. oral ulcers or bloody/inflammatory
nasal discharge).
• Abnormal CXR identifying pulmonary nodules, infiltrates or cavities.
• Microscopic haematuria.
• Biopsy confirmation of granulomatous inflammation of an artery or
perivascular region.
>80% of patients with GPA are associated with ANCA +ve serology, usually
•
PR3-ANCA, 20% patients have alternative ANCA and 10% of patients are
ANCA negative.
• Utilisation of ANCA serology can be used to replace histological
diagnosis if appropriate clinical history and radiology are supportive.
11.9 MICROSCOPIC POLYANGIITIS (MPA)
A poorly defined autoimmune disease characterised by necrotising small ves-
•
sel vasculitis without evidence of granulomatous inflammation.
Previously termed polyarteritis nodosa.
•
Usually the disease is p-ANCA positive.
•
Clinically and pathologically there is much overlap with GPA and MPA. e
•
presentation is usually insidious, with constitutional symptoms such as fever,
weight loss and fatigue.
It can develop into a rapidly progressive vasculitis, which can affect the lungs;
•
with breathlessness, wheeze and haemoptysis. Necrotising crescentic glomerulonephritis is characteristic of MPA.
11.10 GOODPASTURE’S SYNDROME
1. DEFINITION AND EPIDEMIOLOGY
Goodpasture’s syndrome (GS) is characterised by anti-glomerular basement
•
membrane antibodies (anti-GBM) deposition within affected organs.
• Although vasculitis is present in some diagnoses of GS, it is not primar-
ily a vasculitis: it is an autoimmune disease.
• It is included in this section as it is an important differential in lung
disease with a vasculitic picture.
• It presents with a rapidly progressive glomerulonephritis with 30–40%
of patients having pulmonary involvement (mainly haemorrhage).
GS is rare, with an estimated incidence of 2 cases per million.
•
of cases occur in Caucasians, and there is a male predominance.
80%
•
ere are 2 peaks of onset:
•
• 20- 30 years
• 60- 70 years.
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11.12 Presentation, Investigation and Management of Vasculitis
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2. AETIOLOGY AND PATHOPHYSIOLOGY
Patients with GS have an antibody (anti-GBM) to the alpha-3 chain of type
•
IV collagen which is found in the lung and glomeruli of the kidney.
• Identification of the antibody alongside the clinical picture is
diagnostic.
ere may be a genetic association and many patients will have a history of
•
smoking or hydrocarbon exposure.
11.11 CONNECTIVE TISSUE DISEASE AND
LUNG VASCULITIS
1. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
SLE can affect almost any organ system, it is an auto-immune disorder with
•
anti-phospholipid and anti-nuclear antibodies (ANA).
is can lead to underlying vasculitis within the lung and diffuse pulmonary
•
haemorrhages creating a picture similar to primary vasculitis.
SLE can present as a pulmonary renal syndrome.
•
It is more common in females, Afro-Caribbeans and younger patients.
•
Skin and joint manifestations usually predominate.
•
2. OTHER CONNECTIVE TISSUE DISORDERS
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Other systemic disorders can affect the lung such as:
•
• Rheumatoid arthritis
• Systemic sclerosis
• Ankylosing spondylitis.
All of these disorders classically cause lung fibrosis, rather than a vasculitic
•
picture and are therefore not discussed here (see Chapter 12: Interstitial lung
disease).
11.12 PRESENTATION, INVESTIGATION AND
MANAGEMENT OF VASCULITIS
1. PRESENTATION
Vasculitis involving the lung can have a variety of signs and symptoms
•
involving both the upper and lower respiratory tract, as well as systemic
manifestations (See Figure 11.6).
Specific symptoms are more typical of certain causes, as well as the speed
•
of onset.
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Figure 11.6 Clinical features of vasculitic diseases that affect the lung.
2. INVESTIGATIONS
Investigation findings in vasculitis are listed in Table 11.2.
•
Table 11.2 Investigations in suspected vasculitis of the lung.
INVESTIGATIONS FINDINGS
Full blood count (FBC) Eosinophilia in EGPA, leukopenia in SLE.
Urea and electrolytes U&E may be deranged with a raised creatinine if the
(U&E) kidney is involved in any of the vasculitides.
Erythrocyte sedimenta- Will be raised in active disease with any of the
tion rate (ESR) and vasculitides.
C-reactive protein (CRP)
Urinalysis and Haematuria and casts will be seen in EGPA, GS and
microscopy SLE.
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MPA: proteinuria and haematuria.
(Continued)
11.12 Presentation, Investigation and Management of Vasculitis
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Table 11.2 (continued) Investigations in suspected vasculitis of the lung.
INVESTIGATIONS FINDINGS
ANCA, ANA, anti- EGPA: pANCA +ve.
GBM antibodies GPA: cANCA +ve.
GS: anti-GBM +ve, ANCA: may be +ve.
SLE: ANA +ve (anti-dsDNA and anti-Smith more
specific).
MPA: pANCA +ve.
Complement Low levels of complement in SLE.
Clotting screen Needed in suspected GS before performing renal
biopsy.
Prothrombin time (PT) will be prolonged in SLE.
Chest X-ray or Will show transient interstitial infiltrates or nodules
computed tomography in EGPA.
(CT) GPA may show cavitating lung nodules and infiltrate.
In GS there may be lung shadowing due to pulmo-
nary haemorrhage.
Pulmonary function Will show reversible airway obstruction in EGPA.
tests Will show elevated diffusion capacity in pulmonary
haemorrhage.
Renal biopsy Will show crescentic glomerulonephritis, and characteris-
tic linear IgG staining on immunofluorescence in GS.
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3. MANAGEMENT
Rapid diagnosis and treatment is critical to prevent death and long-term end
•
organ damage.
Management is usually done in conjunction with renal specialists and/or
•
rheumatologists.
Corticosteroids are the mainstay of treatment in all causes of lung vasculitis.
•
Steroids are combined with other immunosuppressive agents used either to
•
induce remission or to maintain disease control.
• Examples include cyclophosphamide, azathioprine, mycophenolate and
rituximab.
Plasmapheresis is sometimes used in severe renal or pulmonary disease.
•
Patients on long-term immunosuppression are usually offered Pneumocystis
•
jirovecii prophylaxis:
• Co-trimoxazole 150 mg once a day (OD).
4. PROGNOSIS
e prognosis of vasculitic disease in the lung is dependent on the cause as
•
well as the speed of treatment initiation.
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EGPA prognosis is highly dependent on extent and severity of organ involve-
•
ment, but with appropriate immunosuppression survival is similar to age
matched controls.
GPA has a poor prognosis untreated with median survival of 5 months, but
•
aggressive immunosuppression regimens achieve remission for most patients.
• Morbidity in these patients is common as most will suffer long-term
complications.
• Almost half of patients will suffer a relapse in their lifetime.
GS has a good prognosis with early aggressive treatment.
•
• Over 90% of patients will recover and not require dialysis.
• If dialysis is required on admission to hospital, the prognosis is much
poorer as end-stage renal failure usually develops.
MICRO-case
A 30-year-old man presents with a 3-month history of nasal stuffiness,
haemoptysis and epistaxis, associated with shortness of breath, weight
loss and night sweats. Examination revealed crusting of his nares. Initial
blood tests revealed elevated white cell count (neutrophilia) and anaemia.
There is evidence of acute kidney injury on his U&E, with a urine dipstick
positive for blood. CT chest demonstrates multiple infiltrative lesions
and bronchoscopy shows evidence of recent haemorrhage with cytology
demonstrating eosinophilia. As vasculitis is suspected, an ANCA screen is
sent. The patient is positive for cANCA and anti-PR3, confirming granulomatosis with polyangiitis. He deteriorates rapidly, necessitating critical care
input for type 1 respiratory failure and renal replacement therapy for acute
kidney injury with oliguria. He is treated with high-dose glucocorticoids in
combination with rituximab. Due to the severity of renal disease, he also
undergoes plasma exchange. Following recovery from the acute episode,
he continues on a reducing course of prednisolone, alongside rituximab.
Co-trimoxazole is added for P. jirovecii prophylaxis and alendronic acid
with calcium and vitamin D supplements for bone protection.
Key Points
Granulomatosis with polyangiitis requires rapid diagnosis and treat-
•
ment to prevent severe morbidity and mortality.
Glucocorticoids in combination with other immunosuppressive
•
agents (usually cyclophosphamide or rituximab) are the mainstay of
treatment.
An MDT approach is often necessary as patients may have extra-
•
pulmonary manifestations (e.g. renal disease).
Plasma exchange may be required in severe cases.
•
Patients should receive prophylaxis against P. jirovecii when on high
•
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dose steroids.
Vitamin D, calcium and bisphosphonates are given to protect against
•
osteoporosis due to long-term steroid use.
12
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Pulmonary
Hypertension
12.1 PULMONARY HYPERTENSION
1. DEFINITION
e 2022 European Society of Cardiology/European Respiratory Society
•
(ESC/ERS) guidelines now define pulmonary hypertension (PH) by a mean
pulmonary arterial pressure >20 mmHg at rest.
e definition of pulmonary arterial hypertension (PAH) also implies a
•
pulmonary vascular resistance (PVR) >2 Wood units and pulmonary arterial
wedge pressure ≤15 mmHg.
2. CLASSIFICATION OF PH
Patients are classified into World Health Organization (WHO) PH
•
categories 1–5 (see Table 12.1).
• Group 1 PH disorders are known as pulmonary arterial hypertension
(PAH).
• Groups 2–5 are referred to as PH but can also be used to refer to all five
groups collectively.
Table 12.1 WHO classification of PH.
WHO PH GROUPS WHO PH SUBGROUPS
Group 1: Pulmonary 1. Idiopathic pulmonary arterial hypertension:
arterial hyperten- 2. Heritable:
sion (PAH) • BMRP2
• ALK 1, endolin
• Unknown
3. Drug and toxin induced (e.g. dasatinib,
dexfenfluramine, methamphetamines)
4. Pulmonary arterial hypertension related to risk
factors or associated conditions:
• Connective tissue disease
• HIV infection
(Continued)
DOI: 10.1201/9781315113937-13
Pulmonary Hypertension192
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Table 12.1 (continued) WHO classification of PH.
WHO PH GROUPS WHO PH SUBGROUPS
• Portal hypertension
• Congenital heart disease
• Schistosomiasis
• Chronic haemolytic anaemia.
5. Pulmonary veno-occlusive disease and/or
capillary haemangiotosis
6. Pulmonary arterial hypertension related to risk
factors or associated conditions:
• Connective tissue disease
• HIV infection
• Portal hypertension
• Congenital heart disease
• Schistosomiasis
• Chronic haemolytic anaemia.
Group 2: Pulmonary 1. Systolic dysfunction
hypertension due to 2. Diastolic dysfunction
left heart disease 3. Valvular disease.
Group 3: Pulmonary 1. COPD.
hypertension due to 2. Interstitial lung disease.
lung disease/ 3. Other pulmonary disease with mixed restrictive
hypoxia and obstructive pattern.
4. Sleep disordered breathing.
5. Alveolar hypoventilation disorders.
6. Chronic exposure to high altitude.
7. Developmental abnormalities.
Group 4: Chronic
thromboembolic
pulmonary hypertension (CTEPH)
Group 5: Pulmonary 1. H aematological disorders: myeloproliferative
hypertension with disorders, splenectomy.
unclear/multifacto- 2. Systemic disorders: sarcoidosis, Langerhans cell
rial mechanism histiocytosis, lymphangioleiomyomatosis,
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neurofibromatosis, vasculitis.
3. Metabolic disorders: glycogen storage disease,
Gaucher disease, thyroid disease.
4. Others: tumoral obstruction, fibrosing
mediastinitis, chronic renal failure on dialysis.
12.2 Idiopathic Pulmonary Arterial Hypertension (IPAH)
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3. DIAGNOSIS OF PH
Symptoms of PH are mainly linked to right ventricle (RV) dysfunction, and
•
typically associated with dyspnoea on exercise in the earlier course of the
disease. e cardinal symptom is dyspnoea on progressively minor exertion.
Other common symptoms and clinical signs are listed in the section below.
•
Identification of underlying diseases, especially left heart dysfunction and
•
lung disease, as well as comorbidities, is essential to ensure proper classification, risk assessment, and treatment.
e diagnostic approach requires a low threshold of suspicion of PH, echo-
•
cardiographic confirmation and then fast-track referral to PH centres in
patients with a high likelihood of PAH, CTEPH, or other forms of severe
PH.
12.2 IDIOPATHIC PULMONARY ARTERIAL
HYPERTENSION (IPAH)
1. DEFINITION
IPAH is PAH in absence of identifiable cause, risk factors or family history.
•
2. EPIDEMIOLOGY
IPAH is very rare with an incidence of 1–2 cases per million per year in
•
Europe and America, and is 2–4 times more common in women than men.
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3. PATHOPHYSIOLOGY
Group 1 PAH is characterised by:
•
• Proliferative vasculopathy of the small muscular pulmonary arterioles.
• Medial hypertrophy.
• Intimal hyperplasia.
• Plexiform lesions:
– A proliferation of endothelium and smooth muscle cells with accu-
mulation of inflammatory cells.
– ey are a hallmark of IPAH but may also be seen in other forms of
PH.
Groups 2–5 have more heterogeneous changes but do share some features
•
with Group 1 disease.
Increased pulmonary resistance causes increased right heart afterload, which
•
in turn leads to right heart failure.
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Pulmonary Hypertension194
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MICRO-facts
PAH Diagnostic Criteria
Right heart catheterisation is the gold standard for diagnosing and
•
classifying PH.
A mean pulmonary artery pressure ≥20 mmHg at rest confirms the
•
presence of PH.
A mean pulmonary wedge pressure <15 mmHg excludes PH due to
•
left heart disease.
This is the pressure measured by wedging a pulmonary catheter
•
with an inflated balloon into a small pulmonary arterial branch.
It provides an indirect measure of left atrial pressure.
•
Chronic lung diseases and other causes of hypoxemia are mild or
•
absent.
Venous thromboembolic disease is absent.
•
Absence of systemic disorders (e.g. sarcoidosis), haematological dis-
•
orders (e.g. myeloproliferative diseases), and metabolic disorders (e.g.
glycogen storage disease).
4. CLINICAL FEATURES
Symptoms:
•
• Shortness of breath on exertion.
• Exertional syncope/presyncope.
• Angina.
• Palpitations.
• Leg swelling.
Signs:
•
• Raised JVP with giant V waves.
• Right ventricular heave.
• Splitting of S2 with loud P2.
• Tricuspid regurgitation murmur.
• Hepatomegaly and exaggerated hepato-jugular reflux.
• Ascites and peripheral oedema.
• Cyanosis.
5. INVESTIGATIONS
Blood tests:
•
• Generally to rule out other causes of PH.
• Autoantibodies to rule out connective tissue disease.
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• rombophilia screen.
• Serum ACE.
12.2 Idiopathic Pulmonary Arterial Hypertension (IPAH)
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• yroid function tests.
• HIV serology.
ECG:
•
• Signs of right ventricular hypertrophy.
• Right axis deviation.
• Dominant R wave in lead V1.
• P pulmonale.
• Incomplete RBBB.
CXR:
•
• Prominent pulmonary arteries.
• Cardiomegaly (See Figure 12.1).
• Pruning of peripheral lung vessels.
195
Figure 12.1 CXR of a patient with pulmonary hypertension showing prominent pulmo-
nary arteries and cardiomegaly.
ECHO:
•
• Raised estimated systolic pulmonary artery pressure (>40 mmHg).
• Dilated right-sided chambers.
• Reduced right ventricular function.
• Right ventricular hypertrophy.
• Enlarged pulmonary artery.
• Paradoxical septal movement.
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