Respiratory - Level 2.2 Flashcards
Definition of sarcoidosis?
o Multisystem inflammatory chronic condition characterised by non-caseating epithelioid granulomata
Epidemiology of sarcoidosis?
- Prevalence highest in Northern Europe, 20-40 years old, Afro-Caribbeans
Symptoms of sarcoidosis - pulmonary disease?
o Bilateral hilar lymphadenopathy, pulmonary infiltrates or fibrosis
o Dry cough, progressive dyspnoea, decreased exercise tolerance and chest pain
Symptoms of sarcoidosis - skin?
Papules on face
Maculopapular rash on body or extremities
Brownish-red infiltrative plaques on extremities or trunk
Erythema nodosum on legs
Lupus pernio – raised, hardened lesion affecting nose and cheeks (pathognomonic)
Symptoms of sarcoidosis - eye?
Granulomatous uveitis • Anterior & posterior Keratoconjunctivitis Dry eyes Glaucoma
Symptoms of sarcoidosis - neurological?
Bell’s palsy Lymphocytic meningitis Diabetes insipidus Neuropathy – numbness, weakness Pituitary dysfunction
Symptoms of sarcoidosis - CV?
Arrhythmias or heart failure in young patient – cardiomyopathy
Symptoms of sarcoidosis - others?
Lymphadenopathy Hepatomegaly, splenomegaly Hypercalcaemia and hypercalciuria, renal stones Bone marrow failure Lacrimal and parotid gland enlargement
Investigations in sarcoidosis?
o Bloods FBC (raised WCC, lymphopenia, anaemia) ESR raised U&E Ca (elevated) LFTs (deranged) Serum ACE raised
o Tuberculin skin test (negative)
o If raised Ca:
24-hour urine collection – raised calcium
Imaging of sarcoidosis
CXR
• Bilateral hilar lymphadenopathy
CT
Further testing of sarcoidosis
o Lung function tests – restrictive pattern o ECG (arrhythmias or BBB) o Bronchoalveolar Lavage Raised lymphocytes, raised neutrophils with pulmonary fibrosis
Diagnostic testing of sarcoidosis
o Tissue Biopsy
Lung, liver, lymph nodes, skin
Non-caseating granulomas
Staging of sarcoidosis of CXR?
Staging on CXR
o Stage 0 =normal
o Stage 1= bilateral hilar lymphadenopathy (+/- paratracheal adenopathy)
o Stage 2 = Bilateral hilar adenopathy with pulmonary infiltrates
o Stage 3 = Parenchymal infiltrates without hilar adenopathy
o Stage 4 = Parenchymal involvement gives volume loss (fibrosis) and other features (calcifications, hilar retraction, cysts, bullae)
Management of sarcoidosis - acute sarcoidosis?
o Bed rest
o NSAIDs
o Remission is common
o If severe – oral prednisolone or IV Methylprednisolone
Management of sarcoidosis - chronic sarcoidosis - immunosuppression?
Prednisolone (0.5mg/kg/day) for 4-6 weeks, then lower dose over 6 months according to response and give for 6-24 months
• NOT INDICATED IN STAGE 1, 2, 3 IF ASYMPTOMATIC
• Indications
o Parenchymal lung disease (symptomatic, progressive, static)
o Uveitis
o Hypercalcaemia
o Neurological or cardiac involvement
• Oral bisphosphonates to minimise bone loss
Management of sarcoidosis - chronic sarcoidosis - other therapies?
- Methotrexate/azathioprine/hydroxychloroquine /ciclosporin/cyclophosphamide
- Anti-TNF alpha
- Rituximab
Management of sarcoidosis - chronic sarcoidosis - lung transplant?
Lung Transplant
• End stage lung disease
Management of sarcoidosis - chronic sarcoidosis - specific eye and skin features?
Eye – Topical prednisolone
Skin – Topical Triamcinolone or oral prednisolone
Management of sarcoidosis - chronic sarcoidosis - monitoring?
o Follow up every 3-6 months o Monitoring FBC DEXA every 2 years Serial lung function tests, CXR and ACE levels
Complications of sarcoidosis?
- Pulmonary – Infection, PE, fibrotic lung disease, pulmonary hypertension
- Cardiac – arrhythmias, HF, SCD
- Ocular – Uveitis, conjunctivitis
- Neurological – Nerve damage, TIA/CVA, CNS impairment
- ENT – salivary gland dysfunction, nosebleeds, obstruction
- Others – lymphoma, hypercalcaemia, renal stones, arthritis
Prognosis of sarcoidosis?
- 60% with thoracic sarcoidosis resolve over 2 years
- 20% respond to steroids
o If not, improvement unlikely
Definition of cystic fibrosis?
- Autosomal recessive condition causing a defective CFTR protein on chromosome 7
- CFTR is a cAMP dependent chloride channel found in membrane of cells
Pathology of cystic fibrosis?
- Leads to excessively thick mucus in many body systems
o Reduction in airway surface liquid layer and impaired ciliary function
o Chronic endobronchial infection ensues
o Thick meconium is produced
o Pancreatic ducts also become blocked by thick secretions
o Abnormal sweat glands
Epidemiology of cystic fibrosis?
- Incidence of 1 in 2500 live births with a carrier rate of 1 in 25
- Average life expectancy is mid-30s