sarcoidosis
Table of Contents
sarcoidosis
Introduction
- a chronic disease in which granulomas form as nodules in multiple organs, especially lungs and LNs, but also skin (25%), eyes, liver, heart, kidneys
- commonly improves or clears up spontaneously - most have no symptoms after 9 years but 50% have relapses and 10% develop serious disability - usually respiratory failure
Aetiology
- cause is unknown
- one case seems to be clearly related to silica in cat litter 1)
Epidemiology
- more common patients who are Black and more common in women
Clinical features
- may be asymptomatic
- symptoms may include chronic fatigue, LOW, aches and pains, arthritis, dry eyes, blurry vision, SOB, dry hacking cough, skin rashes, skin nodules
- Löfgren syndrome: combination of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia
- 90% eventually develop an abnormal the chest X-ray (CXR)
- 75-90% develop hilar &/or paratracheal LN enlargement
- generalised lymphadenopathy is common, especially cervical nodes
- 5-15% develop pulmonary fibrosis
- anaemia occurs in 4-20%
- 40% develop mild-mod lymphopenia
- 20-30% develop hepatomegaly
- 5% develop symptomatic cardiac disease
- 5% develop neurologic involvement such as cranial nerve palsies, chronic meningitis or peripheral neuropathy
- uveitis
- up to 5% develop parotid involvement which is usually bilateral and may cause xerostomia
- uveoparotid fever - combination of anterior uveitis, parotitis, VII cranial nerve paralysis and fever
- may cause hypervitaminosis D ⇒ compensatory hypoparathyroidism, fatigue, metallic taste, cognitive dysfunction, immune dysfunction
- up to 1/3rd develop hyperprolactinaemia
- some may develop thyroid dysfunction
- association with coeliac disease
Diagnosis
- essentially is based on clinical features combined with radiographic features and preferably histology showing non-caseating granulomas.
- histology of granulomas reveal higher than normal levels of type 1 innate lymphoid cells (ILC1s) 2)
- inhibiting the CXCR4 can inhibit granulomas from forming
- CXR
- lung function tests
- serum ACE is controversial
- is a marker of sarcoid granuloma burden
- has poor sensitivity (60%) and specificity for diagnosis though
- may also be elevated in other conditions such as Gaucher’s disease, disseminated tuberculosis, hyperthyroidism and fungal infections
- ACE levels are reduced in patients on ACE inhibitors
sarcoidosis.txt · Last modified: 2024/09/04 00:21 by gary1