Page 127 - Medicine and Surgery

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Chapter 3: Granulomatous/vasculitic lung disorders 123

1 Non-pharmacological: Postural drainage is crucial Pathophysiology
and requires training by physiotherapists. Patients are Unknown but there is strong evidence for an im-
taught to tip and hold themselves in the correct posi- munopathological basis:
tions several times a day. 1 The granulomas consist of macrophages and mainly
2 Pharmacological: Tlymphocytes, particularly helper (CD4+)cells, and
Antibiotics are given for acute infections and exac- even before granulomas form bronchoalveolar lavages

erbations. reflect this cell mix.
Bronchodilator therapy may be useful if there is 2 Peripheral blood has decreased T cells and relatively

airflow obstruction. increased B cells (overall lymphopenia).
3 If hypoxic long term oxygen therapy may delay the 3 Depressed cell mediated immunity is demonstrated
onset of cor pulmonale. by the depressed responses to tuberculin or other im-
4 Surgery may be needed for massive haemoptysis. munological skin testing (this is called ‘anergy’).

Prognosis Clinical features
Variable depending on cause and severity. Around half present with respiratory symptoms or are
diagnosed following an incidental finding of bilateral
hilar lymphadenopathy or lung infiltrates on chest X-
Granulomatous/vasculitic ray. Next most common are skin and ocular presen-
lung disorders tations. Other presentations include arthralgias, non-
specific symptoms of weight loss, fatigue and fever.
Pulmonary manifestations:
Sarcoidosis Bilateral hilar lymphadenopathy with or without pul-
monary infiltration.
Definition
Pulmonary infiltration may be progressive leading to
Multisystem chronic granulomatous disorder of un-
known origin. fibrosis, increasing effort dyspnoea and eventually cor
pulmonale and death.
Extra pulmonary manifestations:
Incidence
Anyorgan of the body can be affected, most com-
19 per 100,000 in United Kingdom.
monly:
Skin: Sarcoid is the commonest cause of erythema

Age
nodosum (see page 390), when associated with bi-
Under 40 years; peak 20–30 years.
lateral hilar lymphadenopathy it is diagnostic. Viola-
ceous plaques on the nose, cheeks, ears and fingers
Sex known as lupus pernio or skin nodules may occur.
F > M Eyes: Anterior uveitis is common.
Enlargement of lacrimal and parotid glands.

Geography Arthralgia and joint swelling with associated bone
Affects American Afro Caribbeans more than Cau- cysts.
casians. Uncommon in Japan. Cranial nerve involvement particularly facial nerve
palsy occurs but is uncommon.
Aetiology Hepatosplenomegalyoftenpresentthoughrarelyclin-
NoHLAassociationbutfamilialcasesseen,suggesting ically relevant.

environmental factors. Cardiacinvolvementisrelativelycommon:ventricular
Because of the granulomata, atypical mycobacterium, dysrhythmias, conduction defects and cardiomyopa-

Epstein–Barr virus or other infections have been sug- thy with congestive cardiac failure.
gested but never proven. Tenper cent of patients with established sarcoidosis
Beryllium poisoning can cause an identical clinical have hypercalcaemia and hypercalciuria which can

picture. lead to renal calculi. This is thought to be due to