Chronic lung & Systemic diseases Flashcards
- The CT chest of a 35-year-old-patient reveals parenchymal nodules, emphysema and multiple thin-walled cysts. Some of these have a bizarre configuration and are predominantly located in the upper and middle zones, sparing the costophrenic recesses and tips of the middle lobe and lingual. What is the most likely diagnosis?
A. Lymphangioleiomyomatosis
B. Birt–Hogg–Dubé syndrome
C. Lymphocytic interstitial pneumonia (LIP)
D. Sarcoidosis
E. Langerhans’ cell histiocytosis (LCH)
E. Langerhans’ cell histiocytosis (LCH)
Early findings include interstitial infiltrate centred on the small airways, leading to the development of small parenchymal nodules. These are typically irregular, of soft-tissue density, and measure 1-5mm in diameter, although some may be over 1cm in size.
- The findings of a High Resolution Computed Tomography (HRCT) scan include irregular bronchovascular, interlobular septal and pleural thickening. There is beading along the bronchovascular bundles, traction bronchiectasis and areas of ground-glass opacification. There is also lymph node enlargement at both hilar nodes and some air trapping. Which is the most likely diagnosis?
A. Tuberculosis
B. Sarcoid
C. Lymphoma
D. Usual Interstitial pneumonitis (UIP)
E. Non-specific Interstitial Pneumonitis (NSIP)
B. Sarcoid
These are characteristic features of sarcoid on HRCT. Perilymphatic nodules (beading along the fissures) and architectural distortion are commonly found.
- Which of the following features detected on HRCT would most favour NSIP rather than USIP?
A. Predominant ground-glass opacification
B. Honeycombing
C. Predominant lower zone disease
D. Traction bronchiectasis
E. Subpleural predominance
A. Predominant ground-glass opacification
Although Ground glass opacification (GGO) can be commonly seen in USIP, it is found in 75-100% of cases of NSIP, where it is usually bilateral, symmetrical and subpleural in two thirds of patients and has a lower zone predominance on over 50%. Honeycombing is not a major feature, although it can occur in up to 50%. Typical HRCT features of USIP include reticulation, honeycombing and traction bronchiectasis with basal and subpleural predominance.
1) A 40-year-old male presents with shortness of breath. He also has lower back pain and stiffness of the spine. A chest radiograph shows bilateral upper-zone fibrosis with elevation of the hila. Spinal ligamentous ossification is also noted. High-resolution CT shows peripheral interstitial changes with traction bronchiectasis and paraseptal emphysematous changes in the upper zones. What is the most likely diagnosis?
a. ankylosing spondylitis
b. Reiter’s syndrome
c. tuberculosis
d. sarcoidosis
e. chronic extrinsic allergic alveolitis
a. ankylosing spondylitis
Pulmonary changes occur in ankylosing spondylitis in 1% of patients, with reticulonodular abnormality in the upper zones progressing to confluent opacification and fibrosis. There may be apical bullous formation and cavitation mimicking tuberculosis. No granulomatous disease is seen. Ossification of spinal ligaments is typical in ankylosing spondylitis. Upper-zone fibrosis is seen with tuberculosis, sarcoidosis and chronic extrinsic allergic alveolitis, but spinal ossification is not a feature of these conditions. The reciprocal is true of Reiter’s syndrome.
8) In systemic sclerosis, what is the most common pulmonary finding on CT?
a. consolidation secondary to aspiration pneumonia
b. pulmonary hypertension
c. pleural effusions
d. interstitial lung disease
e. pulmonary emboli
d. interstitial lung disease
All of these features can occur, but 65% of patients have fibrosis on CT, although only 30% of patients with systemic sclerosis have clinically significant lung disease. The commonest interstitial lung disease is nonspecific interstitial pneumonitis, seen in two-thirds of cases. This is less coarse and extensive than usual interstitial pneumonitis, the other type seen, and is almost always associated with ground-glass opacification. Other respiratory features seen in systemic sclerosis are organizing pneumonia, pulmonary hemorrhage, pleural thickening and malignancy.
(Ped) 10) A 12-year-old boy with known Langerhans’ cell histiocytosis presents with tachypnoea, cough and dyspnoea. A chest radiograph and then high-resolution CT are performed. What are the most likely findings?
a. bilateral, symmetrical consolidation
b. bronchiectasis
c. reticulonodular changes in mid-zones
d. bronchial wall thickening
e. bilateral hilar lymphadenopathy
c. reticulonodular changes in mid-zones
Langerhans’ cell histiocytosis presents with features similar to adults in children over 10 years. Typically, there are reticular/reticulonodular changes that progress to cysts and honeycombing. These predominate in the upper zones and mid-zones with sparing of the costophrenic angles. The diagnosis is made by the characteristic radiographic and clinical findings. Bronchoalveolar lavage or biopsy may be necessary if the diagnosis is in doubt.
36) A 68-year-old patient presents with cough and dyspnea. A chest radiograph is performed. Which feature would suggest sarcoidosis as a more likely diagnosis than tuberculosis?
a. pleural effusion
b. cavitating upper lobe lesion
c. calcified lung lesion
d. symmetrical hilar lymphadenopathy
e. consolidation
d. symmetrical hilar lymphadenopathy
Differentiation of tuberculosis and sarcoidosis can be difficult clinically and radiologically. The presence of symmetrical hilar lymphadenopathy is seen more often with sarcoidosis than with tuberculosis. Pleural effusions, and cavitating and calcified lesions are much more common with tuberculosis. Consolidation can be seen in both conditions, though it is again more commonly seen in tuberculosis.
44) A 38-year-old pregnant female presents with hemoptysis and shortness of breath. A chest radiograph shows a large effusion, which is drained and found to be chylous. What is the most likely diagnosis?
a. histiocytosis
b. tuberous sclerosis
c. neurofibromatosis
d. lymphangiomyomatosis
e. idiopathic pulmonary fibrosis
d. lymphangiomyomatosis
Lymphangiomyomatosis is exclusively seen in women of child-bearing age and is worsened in pregnancy. Classically, it causes coarse reticular/ reticulonodular interstitial changes, and patients develop recurrent chylous effusions and pneumothoraces. Lung volumes are preserved or may be increased (the only interstitial lung disease with increased lung volumes). It may progress to honeycombing. Histiocytosis usually affects men, and causes cystic change in the upper lobes, small nodules and septal thickening. Tuberous sclerosis has similar features to histiocytosis but is associated with skin changes, reduced IQ and epilepsy. Neurofibromatosis tends to cause progressive fibrosis, but effusions and pneumothoraces are uncommon. Idiopathic pulmonary fibrosis tends to affect the older age group with a reticulonodular pattern.
51) A 52-year-old female presents with cough. She is on dialysis, but, apart from abnormal urea and creatinine, her bloods are normal. A chest radiograph is abnormal and high-resolution CT is performed. This demonstrates fluffy, nodular, 5–10 mm opacities of airspace-type appearance with foci of calcification, in an upper lobe distribution with subpleural sparing. Calcification of chest wall vessels is noted. What is the most likely cause of the appearances?
a. varicella
b. chronic renal failure
c. tuberculosis
d. histoplasmosis
e. talcosis
b. chronic renal failure
The appearances are suggestive of ‘metastatic’ pulmonary nodular calcification secondary to chronic renal failure. The upper lobe distribution is due to the relative alkalinity of the upper lobes, caused by the higher ventilationto-perfusion ratio. It is often associated with calcification of chest wall vessels. Varicella produces multiple calcified nodules of 1–3mm after the acute episode has resolved. Patients with tuberculosis or histoplasmosis, where calcified nodules are seen, have 2–5mm, well-defined nodules, and most have calcified lymphadenopathy. Talcosis characteristically produces 1mm, very-high density nodules.
52) A 75-year-old man presents with worsening shortness of breath. He was a mine worker. A chest radiograph shows multiple nodules in the upper zones with a large upper-zone mass on the left. CT confirms multiple small nodules up to 5 mm with a sausage-shaped mass paralleling the mediastinum. What is the most likely diagnosis?
a. coal worker’s pneumoconiosis with bronchogenic carcinoma
b. coal worker’s pneumoconiosis with progressive massive fibrosis
c. tuberculosis
d. primary lung carcinoma with metastases
e. chronic extrinsic allergic alveolitis
b. coal worker’s pneumoconiosis with progressive massive fibrosis
The history of mining (dust exposure) with small nodules in the upper zones is typical of pneumoconiosis. The sausage-shaped mass is characteristic of progressive massive fibrosis (PMF), although malignancy cannot be excluded. PMF is often seen to have reduced nodularity surrounding it, as it incorporates the surrounding nodules and migrates towards the hilum. Tuberculosis usually produces a generalized distribution of 2–3mm nodules in its miliary form, often with lymphadenopathy. Metastases from primary lung cancer are not uniformly small (though they can be in thyroid cancer and melanoma). Chronic extrinsic allergic alveolitis produces fibrotic changes in the mid and lower zones.
56) In patients with rheumatoid arthritis, what is the commonest pulmonary finding seen on the chest radiograph?
a. pleural effusion
b. fibrosis
c. pulmonary nodules
d. bronchiectasis
e. heart failure
a. pleural effusion
Between 2% and 54% of patients with rheumatoid arthritis have pulmonary abnormalities. Pleural abnormalities are most frequent, being either an effusion (unilateral in 92% of cases) or pleural thickening (usually bilateral). Fibrosis occurs in 30% of patients with pulmonary involvement. Nodules are unusual and seen in advanced disease. They are usually peripheral and may cavitate. Bronchial abnormalities are seen in 30% of patients with rheumatoid lung, and include bronchiectasis and bronchiolitis obliterans. Other findings include pulmonary arterial hypertension and heart failure secondary to carditis/pericarditis.
70) A 38-year-old male presents with a cough, not responding to antibiotics. A chest radiograph shows reticular change in both lungs with normal volumes. High-resolution CT confirms multiple thin-walled cysts with centrilobular nodules of 3–10 mm throughout the lungs. The intervening lung is normal. No pleural effusion is present. What is the most likely diagnosis?
a. neurofibromatosis
b. tuberous sclerosis
c. lymphangiomyomatosis
d. histocytosis
e. usual interstitial pneumonitis
d. histocytosis
Honeycombing is seen with all of these conditions, but the combination of normal volumes, cysts and peribronchial nodules is very suggestive of histiocytosis. Often the lung bases are preserved early in the disease but the whole lung is eventually involved. Lung volumes in usual interstitial pneumonitis are reduced. Neurofibromatosis causes interstitial fibrosis with little nodularity. Tuberous sclerosis causes lower-zone fibrotic change with miliary nodules that progresses to honeycombing. Lymphangiomyomatosis produces coarse reticular change due to cyst formation, but nodularity is not seen. Pneumothoraces and pleural effusions are seen with neurofibromatosis, tuberous sclerosis and lymphangiomyomatosis.
91) A 60-year-old female presents with increasing shortness of breath. She is known to have rheumatoid arthritis. A chest radiograph shows reticulonodular changes, and high-resolution CT is performed. Which feature would suggest underlying sarcoidosis as a more likely diagnosis than rheumatoid lung?
a. lower-zone predominance of reticulation
b. mid-zone predominance of reticulation
c. pleural effusion
d. multiple nodules larger than 20 mm
e. cardiomegaly
b. mid-zone predominance of reticulation
Sarcoidosis is seen more commonly in females and affects the thorax in 90% of cases. It usually produces adenopathy with or without parenchymal disease. There is a mid-zone predominance with irregular septal thickening, peri lymphatic nodules, ground-glass opacification, traction bronchiectasis and honeycombing. Rheumatoid lung is more common in males and mostly presents with pleural abnormalities, usually an effusion. It may present with an interstitial fibrotic picture with a lower-zone predominance. Multiple large nodules of up to 7cm in diameter are seen with rheumatoid lung. Cardiomegaly may be seen with both sarcoidosis and rheumatoid, as a result of congestive cardiac failure.
99) A 67-year-old presents with shortness of breath. A chest radiograph shows a reticulonodular pattern with hilar adenopathy. Which feature on high-resolution CT would make silicosis a more likely diagnosis than sarcoidosis?
a. nodules .10 mm
b. calcified hilar lymph nodes
c. traction bronchiectasis
d. honeycombing
e. progressive massive fibrosis
e. progressive massive fibrosis
Silicosis is due to inhalation of silicon dioxide particles, and radiographic changes of chronic silicosis are seen 10–20 years after exposure. The nodules are typically less than 10mm, compared with sarcoid nodules, which can be more variable in size (acinar type 6–7mm, alveolar type .10mm). Calcified hilar lymph nodes (eggshell pattern), traction bronchiectasis and honeycombing occur in both conditions and are not discriminatory. Progressive massive fibrosis is a complication of the pneumoconiosis and is not seen in sarcoidosis.
100) A 25-year-old male presents with hemoptysis. A chest radiograph shows symmetrical, bilateral, perihilar consolidation, extending to the bases but with sparing of the apices. There is mild hilar enlargement. The patient is also found to be in renal failure. Appearances on the chest radiograph subsequently progress to an interstitial pattern. What is the most likely diagnosis?
a. primary pulmonary haemosiderosis
b. secondary pulmonary haemosiderosis
c. Goodpasture’s syndrome
d. hereditary hemorrhagic telangiectasia
e. histoplasmosis
c. Goodpasture’s syndrome
Goodpasture’s syndrome is an autoimmune disease characterized by glomerulonephritis and pulmonary hemorrhage. Respiratory features are usually preceded by respiratory infection. Mild hemoptysis occurs associated with cough and dyspnea. Bilateral consolidative changes and sometimes enlarged hilar nodes may occur.
Pulmonary haemosiderosis presents in a similar manner, but usually occurs in patients under 10 years of age in the primary form, with adults affected in the secondary form, which is rare. Patchy airspace change occurs in the first 2 days and then resolves. Progression to fibrosis may occur.
Patients with hereditary hemorrhagic telangiectasia usually have multiple arteriovenous malformations.
3- A CXR is requested in a 52-year-old man with chronic dyspnea. The CXR shows bi-apical volume loss and reticulonodular changes within both upper zones. Which of the following is the most likely underlying etiology?
(a) α-anti-trypsin deficiency
(b) Bleomycin toxicity
(c) Silicosis
(d) Systemic sclerosis
(e) Usual interstitial pneumonitis
(c) Silicosis
Causes of interstitial lung disease which predominantly affects the upper lobes include T B, silicosis, sarcoid, prior radiation therapy, ankylosing spondylitis, chronic EAA, histoplasmosis, and PCP in patients who have received aerosol pentamidine prophylaxis. The typical distribution of α1-AT is peripheral and basal, the other conditions predominantly affect the lower zones.
18- Which of the is the following is not feature of idiopathic Pulmonary fibrosis at CT?
(a) ground glass opacification
(b) Tractional dilatation of the airways
(c) honeycombing.
(d) Basal predominance
(e) Sub pleural distribution
(a) ground glass opacification
Answers (b) — (e) are the classical features of I PF on CT. Ground glass opacification is not a significant feature of IPF- IPF is more common in smokers and, as such, may co-exist with emphysema which may preserve total lung volumes. Irrespective of smoking history, the incidence of bronchogenic carcinoma is increased 7 x in IPF.
(Ped) 22 A 15 year old boy under investigation for polyuria presents to A&E with an acute episode of dyspnoea. CXR reveals a small right-sided pneumothorax and lung cysts are noted: What is the most likely underlying diagnosis?
(a) a1-anti-trypsin deficiency
(b) Langerhans Cell Histiocystosis
(c) Lymphangiomyomatosis
(d) Neurofibromatosis
(e) Tuberous sclerosis
(b) Langerhans Cell Histiocystosis
All are causes of cystic lung disease which may present in young patients. LAM is seen in female patients. In chronic disseminated LCH (Hand-Schuller-Christian disease) there is a classic triad of exophthalmos, diabetes insipidus (due to pituitary stalk lesions) and lytic skull lesions. In 25% of these patients there are lung cysts associated, with bleb formation and spontaneous pneumothorax. The history of polyuria makes LCH the likely diagnosis.
24- A 20-year-old male smoker presents with a history of recurrent spontaneous pneumothoraxes. CXR and subsequent CT show bilateral upper zone fine reticulonodular change with some larger discrete upper thin-walled cysts and increased lung volumes. What is the likeliest diagnosis?
(a) Sarcoidosis
(b) Non-specific interstitial pneumonitis
(c) Usual interstitial pneumonitis
(d) Langerhans cell histiocytosis
(e) Lymphangiomyomatosis
(d) Langerhans cell histiocytosis
Pulmonary L CH is a rare condition affecting young male smokers, characterized by bilateral upper and mid zone changes starting ‘With nodules (which may cavitate) and progressing through the spectrum of reticular change finishing with true fibrosis. It results in increased lung volumes in up to a third of patients
27- A 46-year-old man with a dry cough is referred for a CT of the thorax. This demonstrates multiple pulmonary nodules, each less than 4 mm in diameter, in a peri lymphatic and sub pleural distribution. What is the most likely diagnosis?
(a) Sarcoidosis
(b) Metastases
(c) Tuberculosis
(d) Varicella pneumonia
(e) Extrinsic allergic alveolitis
(a) Sarcoidosis
This is the typical distribution of pulmonary sarcoid nodules. TB, varicella pneumonia, and metastatic disease are randomly distributed. EAA nodules are typically centrilobular.
29- The Intonational Labor Office (ILO) 1980 Intonational Classification of Radiographs pertains to which of the following conditions?
(a) Pneumoconiosis
(b) Sarcoidosis
(c) Extrinsic allergic alveolitis
(d) Hypersensitivity pneumonitis
(e) Asbestos-related lung disease
(a) Pneumoconiosis
This is the system most widely used to code pneumoconiosis by comparing against a standardized set of chest radiographs for nodule size, shape and extent. This semi-quantitive measure enables intonational epidemiological studies to be carried out.
32- With regard to emphysema, which of the following statements is not true?
(a) It is clinically evident in 15% of smokers
(b) Panacinar emphysema has a predilection for the lower lobes
(c) The chest radiograph has a high sensitivity for diagnosing emphysema
(d) Heterogeneous involvement of the lungs improves outcome following volume reduction surgery
(e) Panacinar emphysema is associated with bronchiectasis
(c) The chest radiograph has a high sensitivity for diagnosing emphysema
The chest radiograph has a low sensitivity, but high specificity, for diagnosing emphysema.
Centrilobular and panacinar emphysema predominantly affect the upper and lower lobes respectively, the latter being associated with bronchiectasis in 40% of cases.
Centrilobular emphysema is the result of pollutants, notably smoking, whilst α1-antitrypsin deficiency is the commonest cause of the panacinar type.
45- A previously healthy 32-year-old man presents to A&E with progressive breathlessness. He has a 3-day history of a flu- like illness- Initial chest radiography demonstrates patchy bilateral consolidation. Over the next few days he develops fulminant respiratory failure. A CT performed 1 week after the onset of symptoms demonstrates areas of consolidation and ground glass opacification; in addition, tractional dilatation of the airways is evident on a CT performed at 2 weeks. What is the most likely diagnosis?
(a) Desquamative interstitial pneumonia
(b) Pneumocystis infection
(c) Lymphoid interstitial pneumonia
(d) Accelerated-phase usual interstitial pneumonia
(e) Acute interstitial pneumonia
(e) Acute interstitial pneumonia
The clinical history and CT appearances are characteristic of AIP, formerly known as Hamam-Rich syndrome, which pathologically resembles ARDS.
12 A 32 year old man presents to his GP with increasing shortness of breath. He has never smoked. The initial bloods show a normal full blood count, but abnormal liver function. A CXR is requested and shows emphysematous change within both lower zones only. A subsequent CT is requested. What sub-type of emphysema is likely to be seen?
(a) Centriacinar emphysema
(b) Centrilobular emphysema
(c) Panacinar emphysema
(d) Paracicatricial emphysema
(e) Paraseptal emphysema
(c) Panacinar emphysema
α1-anti-trypsin deficiency is a genetically inherited disorder. In individuals with the PiZZ phenotype there is insufficient cx1 AT to protect the lungs from neutrophil elastase. Even non-smokers develop emphysema in the lower zones which involves the pulmonary acinus diffusely (another cause is i. v. methylphenidate abuse). Liver fibrosis/ cirrhosis can also occur in this condition. Centriacinar emphysema (centrilobular and focal subtypes) occurs in smokers. Paracicatricial emphysema is often seen in scar tissue (e.g. in pulmonary fibrosis).
29 A 62 year old otherwise well woman presents with chronic and progressive dysponea. CXR reveals fine reticular opacification in the upper zones bilaterally with volume loss.
Which of the following is the likeliest diagnosis?
(a) Tuberous sclerosis
(b) Extrinsic allegic alveolitis
(c) Sarcoidosis
(d) Usual interstitial pneumonitis
(e) Ankylosing spondylitis
(c) Sarcoidosis
Tuberous sclerosis and UIP typically affect the lower zones.
Lung volumes are preserved in EAA.
Ankylosing spondylitis is a possibility but is much less common than sarcoid.
41 Which of the following is not a risk factor for developing radiation pneumonitis?
(a) Withdrawal of steroids
(b) Increasing interval between radiotherapy fractions
(c) Concomitant chemotherapy
(d) Pulmonary fibrosis
(e) Smoking
(b) Increasing interval between radiotherapy fractions
Giving higher doses (>2Gy), more frequent fractions, or a total dose in excess of 10 Gy to the lung are also treatment-specific risk factors. Patient-specific risk factors are increasing age and decreasing performance status.
47 Which of the following is the earliest radiological feature of asbestos-related lung disease?
(a) Pleural thickening
(b) Asbestosis
(c) Pleural effusion
(d) Folded lung
(e) Pleural calcification
(c) Pleural effusion
A pleural effusion develops in up to 3% of workers, often occurring between 10 and 20 years after the initial exposure; effusions can be unilateral or bilateral. The effusion is usually small, with the fluid serous or mildly blood stained. The effusion persists up to 6 months, and recurs in up to of 30% cases. Some workers are left with blunting of the costo-phrenic angle, diffuse pleural thickening or round atelectasis. Mesothelioma appears to be unrelated to benign pleural effusions.
50 Which of the following is not a feature of amyloidosis?
(a) Tracheobronchial strictures
(b) Recurrent pneumothoraxes
(c) Calcified parenchymal nodules
(d) Septal thickening
(e) Lymphadenopathy
(b) Recurrent pneumothoraxes
Amyloidosis is an uncommon condition in which there is deposition of amyloid protein in the tissues. Its manifestations are manifold and may be localized or diffuse.
53 Which of the following causes of pneumoconiosis is considered a fibrogenic dust?
(a) Silver
(b) Coal dust
(c) Tin
(d) Silica
(e) Iron oxide
(d) Silica
Silica and Beryllium are considered fibrogenic dusts, i.e. they elicit a fibrogenic response in the lung parenchyma. The remaining dusts are inert within the lung parenchyma and cause very few clinical symptoms even though the radiographs may appear quite dramatic.
56 A 37 year old lady with Sjogren’s syndrome presents with a 2 year history of progressive breathlessness, lowgrade fever and malaise. A CT of the thorax demonstrates diffuse ground glass opacification and a number of discrete thin-walled cysts within the lungs. The CT is otherwise normal.
What is the most likely diagnosis?
(a) Pneumocystis infection
(b) Non-specific interstitial pneumonitis
(c) Cryptogenic organising pneumonia
(d) Desquamative interstitial pneumonitis
(e) Lymphocytic interstitial pneumonitis
(e) Lymphocytic interstitial pneumonitis
Histopathologically characterised by a lymphocytic infiltrate in the lung parenchyma, LIP has an insidious onset and is often diagnosed many months after onset of non-specific symptoms. LIP is also seen in HIV patients, presumably as a consequence of impaired immune function.
59 A 35 year old female is under investigation for known sarcoidosis. Her most recent CXR and HRCT show widespread mid and upper zone reticular opacification with multiple small nodules and no mediastinal or hilar lymphadenopathy.
What is the stage of her disease?
(a) 0
(b) I
(c) II
(d) III
(e) IV
(d) III
There is a normal CXR in stage 0. Stage I shows hilar and mediastinal lymphadenopathy only. Stage II shows lymphadenopathy and parenchymal disease. Stage Ill shows parenchymal disease only. Stage IV is characterized by pulmonary fibrosis.
70 At a multidisciplinary team meeting, the pathologist shows a surgical specimen with destroyed and fibrotic tissue. It contains numerous; cystic airspaces with thick fibrous walls and complete loss of acinar architecture. How might you best describe this at CT?
(a) Interlobular septal thickening
(b) Idiopathic pulmonary fibrosis
(c) Honeycombing
(d) Panacinar emphysema
(e) Non-specific interstitial pneumonitis
(c) Honeycombing
This is the pathological correlate of honeycombing, a descriptive term only, which is seen in end-stage fibrosis from a number of causes. It is an important criterion for IPF, but is not exclusive to it, and it is not a dominant feature of NSIP.
72 A 23 year old patient presents with acute dyspnoea . and pleuritic chest pain. The CXR shows a right-sided pneumothorax and multiple bilateral cystic changes. Following successful treatment, a CT chest is requested. Which feature favours a diagnosis of lymphangioleiomyomatosis over Langerhans cell histiocytosis?
(a) Chylous pleural effusion
(b) Pulmonary nodules
(c) Smoking history
(d) Thin-walled cysts > 1 cm
(e) Upper lobe predominance
(a) Chylous pleural effusion
LAM occurs in young women and is a form fruste of tuberous sclerosis. There is a proliferation of smooth muscle cells along the lymphatics (which may also result in chylothorax). The cysts are < 5-10 mm, thin-walled and surrounded by normal lung. Abdominal and pelvic LAM is associated and renal angiomyolipomas may be present. LCH occurs in young heavy-smoking men, there is an evolution from pulmonary nodules through to thin-walled small, then large cysts, often with a bizarre shape.
@# 73 In the assessment of a thoracic CT, which of the following features is least supportive of a diagnosis of nonspecific interstitial pneumonitis?
(a) Traction bronchiectasis
(b) Volume loss
(c) Ground glass opacifrcation
(d) Thin-walled cysts
(e) Reticular abnormality
(d) Thin-walled cysts
Thin-walled cysts are not a feature of NSIP but should raise the possibility of LIP. The remaining features are typical. Consolidation, honeycombing and nodules are variably reported in the literature as being present or absent.
98) A 59-year-old with a history of ventricular arrhythmia presents with dyspnea on exertion. A chest radiograph shows alveolar and interstitial opacification with frank consolidation at the right lung base, which is of high density. There is no peripheral edema or cyanosis, and the heart size is normal. What is the most likely diagnosis?
a. congestive heart failure
b. sarcoidosis
c. extrinsic allergic alveolitis
d. allergic bronchopulmonary aspergillosis
e. amiodarone pulmonary disease
e. amiodarone pulmonary disease
Amiodarone lung disease occurs after 1–12 months of treatment. Features are of alveolar and interstitial infiltrates and high-density areas of consolidation (iodine attenuation).
Heart failure usually gives a ‘bat-wing’ distribution of consolidation with increased heart size and vascular congestion, and often cyanosis.
Sarcoidosis may produce reticulonodular changes, which coalesce to form areas of consolidation.
Acute extrinsic allergic alveolitis may produce a consolidative pattern, but reticular changes are seen in the chronic stage.
Allergic bronchopulmonary aspergillosis occurs in people with asthma and commonly produces fleeting areas of pneumonitis, with less common features being lobar consolidation, atelectasis and cavitation.
@#e 1. A 50 year old male presents with a history of occasional haemoptysis and exertional shortness of breath which has been getting progressively worse. Plain chest radiograph demonstrates bibasal reticular shadowing with volume loss. HRCT demonstrates bibasal fibrosis and traction bronchiectasis. Incidental note is made of a patulous oesophagus. Which of the following is the most likely cause?
a. Tuberculosis
b. SLE
c. Rheumatoid arthritis
d. Wegener’s granulomatosis
e. Scleroderma
- e. Scleroderma
Whilst haemoptysis may be a presentation in tuberculosis and Wegener’s and bibasal fibrosis maybe seen in all of the above except tuberculosis (where apical fibrosis is the more likely feature), scleroderma is the only condition resulting in a patulous lower oesophageal sphincter, oesophageal shortening and stricture formation.
(Ped) 4. A 16 year old presents with recurrent pneumothoraces. Past history reveals the presence of a lytic lesion of the parietal bone with a tender soft-tissue mass. Which of the following features is most likely seen on HRCT?
a. Evenly distributed smooth thin-walled cysts
b. Centrilobular nodules
c. Extensive paraseptal emphysema with bulla formation
d. Sparing of the apices
e. Cystic lesions along the course of the bronchial tree
- b. Centrilobular nodules
The patient had an eosinophilic granuloma and presented with recurrent pneumothoraces due to Langerhans’ cell histiocytosis, which is characterised by centrilobular nodules which cavitate, initially forming thick-walled and then thin-walled cysts. The fibrosis that follows typically involves the upper zones. Cystic lesions along the bronchial tree are a feature of cystic bronchiectasis
- A 70 year old man, previously working in a ship-building yard, presents with progressive breathlessness. Chest radiograph demonstrates bilateral calcified pleural plaque disease with volume loss. Lung function shows a restrictive pattern. HRCT reveals pulmonary fibrosis. The most likely site of these changes would be:
a. Perihilar
b. Apical
c. Peribronchial
d. Subpleural
e. Fissural
- d. Subpleural
Pulmonary fibrosis associated with asbestos exposure is seen mainly in a subpleural distribution towards the lung bases
- A 25 year old woman with a longstanding history of non-erosive arthritis of the hands and a malar rash presents with progressive breathlessness and respiratory dysfunction. Blood serology demonstrates anti-DNA antibodies. Which of the following is the most common feature on the chest radiograph?
a. Pleural effusion
b. Consolidation
c. Cavitating nodules
d. Pulmonary oedema
e. Pulmonary fibrosis
- a. Pleural effusion
Whilst all the above are seen in SLE, pleural effusions are the commonest radiographic abnormality
@#e 34. A 26 year old female patient with an optic nerve tumour and café-au-lait spots presents with exertional breathlessness. Imaging of the chest is most likely to reveal which of the following?
a. Multiple small lower lobe cysts
b. Emphysema
c. Lower zone fibrosis
d. Thick-walled cavities in the upper zone
e. Asymmetrical upper zone fibrosis
- c. Lower zone fibrosis
The case describes neurofibromatosis I, which is associated with lower zone fibrosis and thin-walled bullae, mainly in the upper zones.
Apart from the pulmonary changes, skeletal abnormalities involving the ribs and spine and mediastinal masses may also be seen
- A 22 year old female patient with a known phakomatosis presents with anaemia and hypotension. CT angiogram reveals evidence of active bleeding in some of the multiple areas of low attenuation (approximately –20) seen scattered throughout both her kidneys. Which of the following features may be seen on chest CT?
a. Multiple pulmonary AVMs
b. Multiple bilateral small cysts
c. Mediastinal mass
d. Thin-walled upper zone bullae
e. Cardiac rhabdomyomas
- b. Multiple bilateral small cysts
Phakomatoses are a group of neurocutaneous syndromes, several of which have other multi-system abnormalities, including intrathoracic findings. These include neurofibromatosis I and II, Sturge–Weber syndrome, von Hippel Lindau syndrome, Osler–Rendu–Weber syndrome and tuberous sclerosis. The above features describe multiple renal angiomyolipomas, which are a feature of tuberous sclerosis. These patients may demonstrate multiple thinwalled cysts with lower zone fibrosis (forme fruste of LAM).
- A 33 year old female patient presents with a longstanding history of fever, dry cough and weight loss. The chest radiograph reveals mediastinal lymphadenopathy. Blood investigations reveal hypercalcemia and elevated angiotensin-converting enzyme (ACE). Which of the following appearances of lymphadenopathy on CT would be the least likely feature in favour of the clinical diagnosis?
a. Bilateral hilar lymphadenopathy
b. Egg-shell calcification
c. Predominant involvement of the right paratracheal lymph nodes
d. Lymphadenopathy without any parenchymal involvement
e. Posterior mediastinal lymph nodes
- e. Posterior mediastinal lymph nodes
All the above are features of mediastinal lymphadenopathy in sarcoidosis. Whilst sarcoidosis can involve different mediastinal and hilar groups, posterior mediastinal lymphadenopathy is a feature of NHL
- The HRCT of a 35 year old patient with shortness of breath and reticulonodular disease pattern on plain chest radiograph reveals cavitating nodules with interstitial septal thickening. Which of the following diagnoses is the least likely?
a. Lymphangioleiomyomatosis
b. Langerhans’ cell histiocytosis
c. Wegener’s granulomatosis
d. Sarcoidosis
e. Rheumatoid lung
- a. Lymphangioleiomyomatosis (LAM)
The cysts in LAM are thin-walled air-containing cysts, rather than cavitation in nodules