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
Symptoms of infancy of cystic fibrosis?
- Meconium ileus (1 in 10)
- Prolonged neonatal jaundice
- Failure to thrive
- Recurrent chest infections
o Cough, purulent sputum, chest deformity
o Initially S.aureus, H.inflenziae
o Pseudomonas aeruginosa, burkholderia cepacia colonisations - Malabsorption (ADEK), steatorrhoea – failure to thrive
Symptoms of young child of cystic fibrosis?
- Bronchiectasis
- Rectal Prolapse
- Nasal Polyp
- Sinusitis
Symptoms of older child of cystic fibrosis?
- Allergic bronchopulmonary aspergillosis
- Diabetes Mellitus
- Cirrhosis/Portal Hypertension
- Intestinal Obstruction
- Pneumothorax
- Sterility in males – congenital absence of vas deferens
Signs of cystic fibrosis?
- Evidence of malnutrition, poor weight gain
- Hyperinflation of the chest
- Coarse inspiratory crackles
- Expiratory wheeze
- Finger clubbing
Testing of cystic fibrosis - in newborns?
- Screened for in Guthrie test
Testing CF - in everyone - when to test?
o FHx, congenital intestinal atresia, meconium ileus, symptoms of intestinal obstruction, faltering growth, undernutrition, recurrent LRTIs, chronic cough, chronic sinus disease, obstructive azoospermia, rectal prolapse
Testing CF - in everyone - what test to perform?
o Sweat Test (children and young people) >60mmol/L
Measuring sodium and chloride concentrations in sweat
o Gene Testing (Adults)
Testing CF - in everyone - when to refer to specialist?
o Positive or equivocal sweat test
o Assessment suggests CF but results normal
o Gene testing reveals 1 or more gene mutations
Management of cystic fibrosis - reviews?
o Annual review
Includes pulmonary (O2 sats, CXR, FBC, aspergillus serology and serum IgE, respiratory samples, LFTs), nutrition, liver disease, complications assessment, DM test
o Routine Reviews - O2 sats, secretion sample, LFT with spirometry Weekly in 1st month Monthly between 1-12 months Every 6-8 weeks between 1-5 years Every 8-12 weeks >5 Every 3-6 months as adults
Management of cystic fibrosis - prevention of cross infection?
o Specialist clinics are organised to prevent-cross infection – separate people individually during clinic & keep people in separate clinics depending on bacteria grown
o In-patients – individual rooms with en-suite facilities
Management of cystic fibrosis - exercise/physio?
Regular exercise improves lung function and fitness
o Individualised exercise programme and review
CT scan for under 12s or if exacerbation does not respond to treatment
Physiotherapy
o Airway clearance techniques taught to patient and carers
o Postural drainage, active cycle breathing or forced expiratory techniques
Management of cystic fibrosis - if lung disease?
o Nebulised rhDNAse (Dornase alpha) as first-line
o Add hypertonic sodium chloride if needed
o If not able to use – mannitol dry powder
Management of cystic fibrosis - if deteriorating lung function or repeated exacerbations?
o Azithromycin long-term at immunomodulatory dose
o If deteriorating – stop azithromycin and start oral prednisolone