Page 373 - Medicine and Surgery

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Chapter 8: Connective tissue disorders 369

Nervous system:
Cardiovascular system: Ischaemic changes in central and
Pericarditis, myocardial fibrosis peripheral nervous system. Peripheral
causing a restrictive cardiomyopathy, neuropathy may occur due to
conduction tissue fibrosis causes perineural vascular sclerosis.
arrhythmias.
Respiratory system:
Pulmonary fibrosis especially in lower
Gastrointestinal system:
lobes and pulmonary hypertension.
Motility disorders including gastro-
oesophageal reflux with oesophagitis,
ulceration and aspiration pneumonia,
malabsorption secondary to bacterial Genitourinary system:
overgrowth. Association with primary Renal failure (acute or chronic) and
biliary cirrhosis. hypertension in 30% of cases
secondary to endoarteritis.

Figure 8.4 Multisystem involvement of systemic sclerosis.

Sj ¨ ogren’s syndrome Pathophysiology
There is lymphocytic infiltration of salivary glands and
Definition
other exocrine glands in the respiratory and gastroin-
Achronic inflammatory disorder of the lacrimal and
testinal tract, the skin and the vagina. There is glandular
salivary glands.
enlargement, with atrophy of the acini and proliferation
of the duct lining.
Sex
9F : 1M
Clinical features
Aetiology Ocular manifestations: Sensation of persistent grit-
Sj¨ ogren’s syndrome may be primary, or secondary to tiness, photosensitivity, tiredness and an inability to
rheumatoid arthritis, systemic lupus erythematosus, produce tears (keratoconjunctivitis sicca).
progressive systemic sclerosis or polymyositis. There is Gastrointestinal system: Lack of saliva (xerostomia)
an association with non-Hogkin B cell lymphoma. causes difficulty in chewing and swallowing. There