CF / Goodpastures etc

Question 1

rheumatoid arthritis

methotrexate pneumonitis

Answer 1

methotrexate-DMARD
can cause fibrosis,
methotrexate pneumonitis

honeycombing pattern
interstitial pneumonia
bi basal chronic respiratory failure
non specific
diffuse inflammation and fibrosis

can get further infections

mx: stop methotrexate

Question 2

cystic fibrosis

Answer 2

Cl- ion transport
area becomes too dry to clear mucus
pseudomonas classic infection

persistent cough
thick mucus
wheeze, SOB
chest infection
sinusitis
bowel disturbance
weight loss
obstruction
constipation
osteoporosis
arthritis

creon (pancreas)

heel prick test*
sweat test

Question 3

TB
symptoms
investigation
treat

Answer 3

travel/living accommodation
fever 
night sweats
persistent cough
bloody phlegm
chest pain, SOB

PA XRAY
diffuse parenchymal disease multiple cavities and bullae formation
sputum smear

test with SPUTUM CULTURE

tuberculin skin test can tell you if you’ve been exposed to TB. tissue biopsy for extra pulmonary TB not pulmonary.

tx:

• isoniazid
• pyridoxine
• rifampicin
• ethambutol

Question 4

Granulomatosis with polyangitis (Wegners)

Answer 4

weakening of blood vessles

three main areas affected:

upper respiratory (nose and sinus)

lung pathologies
- haemoptysis, dyspnoeic cough

kidney pathology
haematuria

ANCA antibodies

Question 5

Goodpasture’s

Answer 5

cough, dyspnoea, haemoptysis, glomerulonephritis

renal and lung biopsy
anti GBM antibodies*

mx: supportive, prednisolone, cyclophosphamide, plasmapheresis

Question 6

squamous cell carcinoma

Answer 6

caveatting mass on CXR
tumour outgrows blood supply- necrotic- cavity (can become infective)

centrally located
ectopic hormone
parathyroid (hypercalcaemia)

cavitating: pulmonary TB, bacterial pneumonia, rheumatoid nodules, septic emboli

Question 7

CF investigation

Answer 7

CF transmembrane conductance regulator (CFTR) genetic screening and sweat test (>60mmol/l of Chloride)

• azoospermia caused by congenital absence of the vas deferent. must use assisted-conception technqiues such as sperm retrieval and intracytoplasic sperm injection
• bronchiectasis (long-standing cough productive purulent sputum)

Question 8

EBV

Answer 8

worsening pharyngtis
flu like symptoms
swelling of lymph nodes in neck
petechial rash over soft palate
tender cervical lymphadeopathy
splenomegaly

diffs: EBV, CMV, toxoplasmosis, HIV, lymphoma

investigations:
FBC, blood film and mono spot test, Paul-Bunnell test, specific antibody test for EBV, CMV, toxoplasmosis, throat swab, ASO titre, lymph node biopsy

Question 9

GPA - granulomatosis with polyangitis

Answer 9

cough
dyspnoea
haemoptysis
chest pain (can be pleuritic)

signs:
fine creptiations
bronchial brathing
pleural rubs and signs of pleural effusion

CXR and CT shows single or multiple rounded lesions which can cavitate

bronchoscopy- granulomatous inflammation

Question 10

chronic cough

Answer 10

cough variant asthma
GORD reflux
post nasal drip

Question 11

PAP - pulmonary alveolar proteinosis

Answer 11

gradually progressive dyspnoea
SOB
bouts of dry cough
clubbing
end inspiratory crackles
pulmonary oedema on CXR
PEFR restrictive pattern

more common in male smokers, 20-50 y/o

abnormal accumulation of surfactants in the alveoli so decreased gas exchange

Question 12

imaging for PAP

Answer 12

CXR: bilateral perihilar alveolar opacities similar to pulmonary oedema

CT: airspace consolidation

lung biopsy- dense granular eosinophilic materials that are acid-Schiff positive

Question 13

PAP management

Answer 13

can remit spontaenosly
most active htherapy - whole lung lavage is neeed
bronchoscope lavage
milky and continues until clear
lung transplant