Sarcoidosis Flashcards
What is sarcoidosis?
diagnosis of exclusion of granulomatous lung diseases, including tuberculosis and histoplasmosis.
What are causes of sarcoidosis?
- Genetic
- Immunological
- Infections: TB, viruses
- 20-40% discovered incidentally
What are RF for sarcoidosis?
- Age 20-40
- Fhx sarcoidosis
- Scandinavian origin
- Afro-caribeean (esp extrapulmonary)
- TB
- Women
- Agricultral exposures + insecticides
What genes are associated with sarcoidosis?
with HLA-DRB1 and DRB1 alleles
What are key non-pulmonary symptoms of sarcoidosis?
- Arthralgia
- Skin lesion: lupus pernio (pathognomonic and erythema nodusm) – so look at leg in rep
- Eye problems: photophobia/red painful eye/blurry vision
- Lupus pernio (cheek, tip of nose and love of ear rash)
What are acute symptoms and signs of sarcoidosis?
- Fever
- Erythema nodosum
- Polyarthralgia
- Bilateral hilar lymphadenopathy
What are pulmonary disease symptoms and signs in sarcoidosis?
- Dry cough
Progressive dyspnoea - Decrease exercise tolerance
- Chest pain
What are pulmonary disease signs in sarcoidosis?
- Wheeze
2. Rhonci
What are non-pulmonary disease signs in sarcoidosis?
- Lymphadenopathy
- Hepatomegaly
- Splenomegaly
- Uveitis
- Conjuncitivieits
- Bells palsy
- Neuropathy
- Erythema nodosum
- Symptoms of hypercalacaemia
What are DDx for sarcoidosis?
- TB
- Histoplasmosis
- Non-small cell lung cancer
- Lymphoma
- Berylliosis
- Hypersensitivity pneumonia
What is the histo and pathophysiology of sarcoidosis?
- Non-caseating granulomas with multinucleated giant cells in centre
- CD4 lymphocytes are spread out through the granuloma and CD8 cells cluster around periphery
- CD4 lymphocyte and associated cytokines such as interferon gamma, IL-2, IL-12 promote and maintain the granulomas
What are bloods are taken in sarcoidosis?
- FBC
- Serum urea
- Creatinine
- Liver enzymes
- Serum calcium
- ESR
- High serum ACE in 60%
What would you find in FBC for sarcoidosis?
anaemia in 4%-20%; leukopenia in 40%
What is serum calcium in sarcoidosis?
hypercalcaemia due to dysregulated production of calcitriol by activated macrophage and granulomas
What is ESR in sarcoidosis?
high
Why do you do other bloods?
Elevated if renal/liver involvement
What other tests are done for sarcoidosis?
- CXR
- PFTs
- ECG
- Purified protein derivative of tuberculin
- Tissue biopsy
- Bronchoalveolar lavage
- Bone x ray
- HRCT
What is stage 1 CXR in sarcoidosis?
bilateral hilar lympahdenopathy
What is stage 2 CXR in sarcoidosis?
bilateral hilar lymphadenopathy plus pulmonary infiltrates
What is stage 3 CXR in sarcoidosis?
pulmonary infiltrates without hilar lymphadenopathy
What is stage 4 CXR in sarcoidosis?
extensive fibrosis with distortion
Why do you do PFTs in sarcoidosis?
to monitor (restrictive, obstructive or mixed)
What may an ECG show in sarcoidosis?
conduction defects
What is purified protein derivative of tuberculin?
negative
What is diagnostic of sarcoidosis?
tissue biopsy that shows non-casaeting granuloms
What would boncholaveolar lavage (BAL) show in sarcoidosis?
high lymphocytes in active disease, high neutrophils with pulmonary fibrosis and transbronchial biopsy may be diagnostic
What would bone x ray show in sarcoidosis?
punched out lesions in terminal phalanges
What is the management for acute sarcoidosis?
bed rest and NSAIDs
What is the other management for sarcoidosis?
1st line: IV or oral corticosteroid e.g. prednisolone 40mg
2nd line: cytotoxics e.g. methotrexate
3rd line: hydroxychloroquine
What are indications for corticosteroids in sarcoidosis?
- Parencyhmal lung disease (symptomatic, static or progressive)
- Uveitis
- Hypercalcaemia
- Neurological or cardiac involvement
What are possible complications in sarcoidosis?
- Severe or life threatening haemoptysis
- Pulmonary arterial hypertension
- Corticosteroid osteoporosis/infection/hyperglycaemia
- CHF
- Meningeal disease
Etc
What is the mortality of sarcoidosis?
1 to 6%
When is there poorer prognosis of sarcoidosis?
- black ancestry
- chronic pulmonary involvement
- lupus pernio
- chronic hypercalcaemia
What is the prognosis for different stage of sarcoidosis?
- Spontaneous remissions occur in 55% to 90% of patients with stage I
- 40% to 70% of patients with stage II
- 20% of patients with stage III disease
- no remissions are expected in stage IV
How many patients respond to steroid therapy?
- 60% of patients with thoracic sarcoidosis resolve over 2yrs
- 20% respond to steroid therapy
What is the patho of sarcoidosis?
Systemic non-ceasating granulomas (collection of macrophages around core), deposted around body causing disease
What organ is mostly affect?
affects lungs in 90% of cases but also other orgams
What would HRCT show in sarcoidosis?
ground glass, reversible, cystic: irreversible
What is Lofgrens syndrome?
- common and spontaneously resolve
1. Bilateral lymphadenopathy
2. Erythema nododsum
3. Arthralgia
4. Fever
What is acute sarcoidosis referred to as?
Lofgren’s syndrome
What is diagnostic for sarcoidosis?
Bronchoscopy with biopsy
What pattern do the granulomas follow?
loosely formed granulomas follow lymphatic pathways:
- Broncho vascular bundles
- Interlobular septa
- Pleura
What is protective ins sarcoidosis?
smoking
What may be seen on CT?
nodular pattern:
- Broncho centric
- Peri-fissural
- Subpleural
What is the induction dose of corticosteroids?
- Moderate dose oral prednisolone (20-40 mg od)
- Or in selectedcases with severe disease - High dose iv MP treatment (1 Gr x 3) followed by 20 mg od, then tapered
What is the maintenance dose of corticosteroids?
Low dose 7.5-10 mg od
What biologics are used in sarcoidosis?
infliximab favoured
When are biologics used?
- Active and progressive disease despite conventional (non-biologics) treatment
- Toxicity or lack of tolerability to converntional treatments
What gene is associated with Logfren’s?
HLA DR3
