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4 Chest medicine
Table 4.
https://t.me/med1917
11
Dierential diagnosis of granulomatous diseases
Infections Bacteria
Fungi
Autoimmune Primary biliary cholangitis
p
554
Vasculitis (
Organic dust disease Silicosis, berylliosis
Idiopathic Crohn’s disease
Extrinsic allergic alveolitis
)
Protozoa
Granulomatous orchitis
Giant cell arteritis
Polyarteritis nodosa
Takayasu’s arteritis
Granulomatosis with
polyangiitis
De Quervain’s thyroiditis
Sarcoidosis
TB
, leprosy, syphilis, cat
scratch fever
Cryptococcus neoformans
Coccidioides immitis
Schistosomiasis
191
Fig 4.
23
PA chest radiograph showing bilateral hilar lymphadenopathy. The important dierentials for this appearance are: sarcoidosis,
This patient has sarcoidosis but there are no other stigmata (such as the presence of infiltrates,
fibrosis, and honeycombing) on this image.
Image courtesy of Norfolk and Norwich University Hospitals
Causes of bilateral hilar lymphadenopathy (
• Sarcoidosis.
• Infection, eg TB, mycoplasma.
• Malignancy, eg lymphoma, carcinoma, mediastinal tumours.
• Organic dust disease, eg silicosis, berylliosis.
• Hypersensitivity pneumonitis.
• Histiocytosis X (La ngerh ans ce ll his tiocyt osis) .
TB
, lymphoma, pneumoconioses, and metastatic disease.
NHS
Trust Radiology Department.
BHL
)
4 Chest medicine
Interstitial lung disease (
ILD
https://t.me/med1917
192
This is the generic term used to describe a number of heterogeneous conditions
that cause diuse parenchymal lung disease.
inflammation and/ or progressive interstitial fibrosis (
number of clinical and pathological features. See
Pathological features Fibrosis and remodelling of the interstitium; chronic inflam-
mation; hyperplasia of type
Classification The
ILD
Exposure related
• Occupational/ environmental, eg asbestosis, berylliosis, silicosis, cotton worker’s
lung (byssinosis).
• Drugs, eg nitrofurantoin, bleomycin, amiodarone, sulfasalazine, busulfan.
• Hypersensitivity reactions, eg hypersensitivity pneumonitis.
• Infections, eg TB, fungi, viral.
Those ass ociated wit h a conn ective tiss ue diso rder (
• Scleroderma, rheumatoid arthritis.
•
SLE
, mixed connective tissue disease, Sjögren’s syndrome.
• Polymyositis, dermatomyositis.
Granulomatous disease
• Sarcoidosis.
Idiopathic in terstitial pneumonia (
• Idiopathic pulmonary fibrosis (
NSIP
), acute interstitial pneumonia, and smoking- related
(
tive interstitial pneumonia & respiratory bronchiolitis- associated
Other forms
• Pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis.
Clinical features Clubbing; progressive dyspnoea on exertion; non- productive par-
oxysmal cough; abnormal breath sounds ± crackles.
Tests NB: careful history— ask about potential exposures (eg inorganic or organic
dusts, drugs, radiation), features of
Raynaud's), hobbies, home and work environment. Check autoantibodies for spe-
CTD
if clinical suspicion (
cific
myositis blot as indicated. Abnormal
interstitial markings) or high- resolution
BAL
/ biopsy); restrictive pulmonary spirometry with
Management
• Specific treatment depending on the underlying condition, eg immunosuppression
CTD- ILD
or sarcoidosis.
for
• Antifibrotic for
• Supportive care: supplemental oxygen if indicated, pulmonary rehabilitation.
• Assess and treat comorbidities and complications, eg
• Manage acute exacerbations with, eg antibiotics, steroids as indicated.
• Progressive disease: refer for lung transplant; if not a candidate, discuss future
IPF
or for progressive fibrosing
ventilation decisions, and consider palliative care involvement if appropriate.
Table 4.
12
Causes of fibrotic shadowing on a
Upper zone Lower zone
TB
Sarcoidosis
Hypersensitivity pneumonitis Aspiration
Ankylosing spondylitis Asbestosis
Radiotherapy Medications, eg amiodarone, methotrexate
Silicosis, berylliosis Infection
Pneumoconiosis (coal workers)
)
55
They are characterized by chronic
table
table
II
epithelial cells or type II pneumocytes.
S can be grouped into the following categories:
CTD- ILD
IIP
)
IPF
, p
194
), non- specific interstitial pneumonitis
CTD (
eg joint pain, dry eyes/ mouth, myalgia,
ANA, RF/ CCP, JO- 1, CK, SA- A, SA- B, SCL- 70, PM1
CXR
(reticular, nodular, or mixed pattern of
CT
(to assess pattern of
DLCO
ILD,
eg pirfenidone, nintedanib.
CXR
Idiopathic interstitial pneumonias, eg
CTD,
eg
RA
4.12
4.12
and fig
)
ILD
including desquama-
ILD
ILD
± guide potential
(p
154
).
GORD
and
PH.
), and share a
4.24
.
.
). Add CK and
IPF
4 Chest medicine
Hypersensitivity pneumonitis (HP)
https://t.me/med1917
Hypersensitivity pneumonitis (HP) represents an immunological reaction occurring
within the lung parenchyma caused by hypersensitivity to an inhaled agent, such as
microbial, avian, and animal antigens and, less commonly, organic compounds. In the
acute phase, the alveoli are infiltrated with acute inflammatory cells. Early diagnosis and
prompt allergen removal can halt and reverse disease progression, so prognosis can be
good. With chronic exposure, granuloma formation and obliterative bronchiolitis occur.
Causes
• Bird- fancier’s and pigeon- fancier’s lung (proteins in bird droppings).
• Farm er ’s an d mu sh ro om wo rk er ’s l un g ( Micropolyspora faeni, Thermoactino-
myces vulgaris).
• Malt worker’s lung (Aspergillus clavatus).
• Bagassosis or sugar worker’s lung (Thermoactinomyces sacchari).
Classification Acute, subacute, and chronic. Non- fibrotic (purely inflammatory)
vs fibrotic (mixed inflammatory plus fibrotic or purely fibrotic) phenotypes based
on the predominant presence or absence of fibrosis on imaging or histopathologic
examination.
Clinical features Acute (less frequent; associated with non- fi brotic HP) Fever,
rigors, myalgia, dry cough, dyspnoea, fine bibasal crackles.
Finger clubbing (
50
%), increasing dyspnoea, weight, exertional dyspnoea, type I
respiratory failure, cor pulmonale.
Te st s 59 Acute Blood:
posure/ previous sensitization rather than disease).
tion; hilar lymphadenopathy (rare). Lung function tests: restrictive defect; reduced gas
transfer during acute attacks.
fibrosis; honeycomb lung.
ground- glass or nodular opacities ± fibrotic changes. Lung function tests: restrictive
defect. Bronchoalveolar lavage
FBC
(neutrophilia);
ESR; ABG
S; serum antibodies (may indicate ex-
CXR:
Chronic Blood tests: serum antibodies.
CT
chest: mid- to- upper zone predominance of centrilobular
(BAL)
flui d shows lymphocytes and mast cells.
Management Acute Remove allergen, PO prednisolone (0.5mg/ kg/ 24h (up to 30mg)
PO
), reducing course. Chronic Allergen avoidance, or wear a facemask or + ve pres-
sure helmet. Trial of steroids. Azathioprine/
MMF
sometimes eective in steroidresistant cases. Lung transplant for advanced fibrotic disease. Compensation (
Industrial Injuries Act) may be payable.
Chronic (fibrotic HP)
up per- zone mottling/ consolida-
CXR:
upper- zone
UK
193
Fig 4.
24
AP chest radiograph showing air- space shadowing in the left upper zone. Although this
appearance often represents infection, it is non- specific. Dierential diagnosis for this distribution
of shadowing include lymphoma, alveolar cell carcinoma (both to be considered if not resolving in
appearance on follow- up imaging), and haemorrhage.
Image courtesy of Nottingham University Hospitals
NHS
Trust Radiology Department.
4 Chest medicine
Idiopathic pulmonary fibrosis (
IPF
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194
IPF
(also known as usual interstitial pneumonia (
pathic interstitial pneumonia (
IIP
)
UIP
)) is the most common type of idio-
). Risk factors include genetic predisposition, smoking,
environmental pollutants, and possibly chronic microaspiration. Repetitive alveolar epithelial injury triggers the early development of fibrosis. It is the commonest cause of
interstitial lung disease. The age of onset is typically
60
+ years ( > ).
Symptoms Dry cough; exertional dyspnoea; malaise; weight; arthralgia.
Signs Cyanosis; finger clubbing; fine end- inspiratory crepitations.
Complications Respiratory failure; PH; risk of lung cancer; comorbid emphysema
and obstructive sleep apnoea.
Tests Blood
toid factor may be + ve if associated with
reticular markings.
ABG
(PaO2; if severe, PaCO2);
HRCT:
characteristic features include an apicobasal gradient of
CRP
; immunoglobulins;
CTD
. Imaging (fig
4.25
)
peripheral (subpleural), reticular opacities associated with architectural distortion, including honeycomb changes and traction bronchiectasis or bronchiolectasis.
Spirometry Restrictive (p
154
); transfer factor.
BAL
Limited role in diagnosis and
usually only indicated to rule out alternative diagnosis, eg sarcoidosis. May indicate
activity of alveolitis: lymphocytes (good response/ prognosis) or neutrophils and
eosinophils (poor response/ prognosis).
discussion. Biopsy may be required for diagnosis depending on
clinical and imaging features are inconclusive. The histology observed on biopsy in
UIP
is a heterogeneous appearance with alternating areas of normal lung, fibrosis,
Lung biopsy All patients must have
MDT
fibroblast foci, and honeycomb change.
Management Supportive care: oxygen, pulmonary rehabilitation, influenza and
GORD
pneumococcal vaccination, treat
. Give antifibrotic therapies— pirfenidone or
nintedanib— which have been shown to slow disease progression.
should be considered for current clinical trials or early referral for lung transplantation (for those with progressive disease and minimal comorbidities).
recommended that high- dose steroids are not used except where the diagnosis of
IPF
is in doubt or as a short course to treat acute exacerbations along with broad-
spectrum antibiotics.
Prognosis Median survival ranges from 2– 5 years.
ANA
or rheuma-
CXR
: increase in
consensus if
60,61
All patients
55
It is strongly
MDT
Fig 4.
25
Interstitial lung disease due to idiopathic pulmonary fibrosis (a similar appearance to the
interstitial oedema of moderate left heart failure, but without a big heart).
Courtesy of Prof P Scally.
4 Chest medicine
Occupational lung diseases
https://t.me/med1917
Coal worker’s pneumoconiosis (
have or have had underground coal mines. It results from inhalation and deposition of coal dust particles (
macrophages which die, releasing their enzymes and causing fibrosis.
Clinical features Asymptomatic, but coexisting chronic bronchitis is common.
CXR
: many round opacities (1– 10mm), especially in upper zone.
Management Avoid exposure to coal dust; smoking cessation; vaccination; treat
coexisting chronic bronchitis; claim compensation (in the
Injuries Act).
Progressive massive fibrosis (
gressive dyspnoea, fibrosis, and, eventually, cor pulmonale.
upper- mid zone fibrotic masses (
Management Avoid exposure to coal dust; claim compensation (as for
Caplan’s syndrome The association between rheumatoid arthritis, pneumoconi-
osis, and pulmonary rheumatoid nodules.
Silicosis (See fig
4.26
.) Caused by inhalation of silica particles, which are very
fibrogenic. A number of jobs may be associated with exposure, eg metal mining,
stone quarrying, sandblasting, and pottery/ ceramic manufacture.
Clinical features Progressive dyspnoea, incidence of TB,
or nodular pattern in upper and mid- zones and eggshell calcification of hilar nodes.
Spirometry: restrictive ventilatory defect.
Management Avoid exposure to silica; claim compensation (as for
Asbestosis Caused by inhalation of asbestos fibres. Asbestos was commonly used
in the building trade for fire proofing, pipe lagging, electrical wire insulation, and
roofing felt. Most patients are asymptomatic for
posure. Degree of asbestos exposure is related to degree of pulmonary fibrosis.
Clinical features Similar to other fibrotic lung diseases with progressive dyspnoea,
clubbing, and fine end- inspiratory crackles. Also causes pleural plaques, risk of
bronchogenic carcinoma and mesothelioma.
Management Symptomatic. Patients are often eligible for compensation through
UK
Industrial Injuries Act.
the
Mesothelioma See p
182
CWP
) A common dust disease in countries that
1– 3
µm in diameter) over 15– 20yrs. These are ingested by
UK
, via the Industrial
PMF
) Due to progression of
1– 10
cm), develop from periphery towards hilum.
CWP
, which causes pro-
CXR
: usually bilateral,
CXR
shows diuse miliary
CWP
20– 30
years after the initial ex-
.
CWP
).
).
195
Fig 4.
26
PA chest radiograph showing diuse nodular opacities with a focal area of irregular soft
tissue shadowing in the right upper zone, consistent with silicosis and developing progressive massive fibrosis (
PMF
).
Image courtesy of Derby Hospitals
NHS
Foundation Trust Radiology Department.
5
https://t.me/med1917
Endocrinology
Contents
The essence of endocrinology
Endocrine physiology
Diabetes mellitus (DM)
Classification and diagnosis
Treating diabetes mellitus
Complications of established
diabetes
Diabetic neuropathy and diabetic
foot care
Hypoglycaemia
Insulinoma
Thyroid and parathyroid disease
Thyroid function tests (
Thyrotoxicosis
Hypothyroidism (myxoedema)
Parathyroid hormone and
hyperparathyroidism
Hypoparathyroidism
The adrenal gland
Adrenal cortex and Cushing’s
syndrome
Addison’s dis ease (adre nal
insufficiency)
Hyperaldosteronism
Phaeochromocytoma
Hirsutism, virilism, gynaecomastia,
and impotence
The pituitary gland
Hypopituitarism
Pituitary tumours
Hyperprolactinaemia
Acromegaly
Diabetes insipidus (DI)
204
206
209
218
232
208
212
220
226
224
228
216
222
230
198
222
TFT
234
216
s)
202
197
200
210
214
We thank Helen Turner, Rustam Rea, and Michael Matheou, our Specialist Readers and Oliver Mowforth,
our Junior Reader for this chapter. We also thank Michael Matheou for contributing to this chapter.
Fig 5.
1
Akhenaten, as depicted here, was an important Egyptian pharaoh of the
reigning from
changes in Egypt including establishing a new capital
and forming a new religion associated with a pacifist
philosophy and a deep respect for nature. He also encouraged a new concept of art in which sculptures or
reliefs portraying the pharaoh were no longer highly
idealized. Akhenaten was then often portrayed as a
person with a prominent jaw, sunken chest, bulky abdomen, and gynecoid fat distribution. Several authors
have suggested that Akhenaten suered from acromegaly, with or without hypogonadism, isolated
hypogonadism, or rickets. His sometimes androgynous
portrayal, with wide hips, an elongated head, almond
eyes, square jaw, gynaecomastia, and no male genitalia
have also led some to suggest possible diagnoses of
Klinefelter’s syndrome or even gender dysphoria.
1365
to
1348
BC. Akhenaten made radical
18
th dynasty,
Artwork by Gillian Turner.
5 Endocrinology
The essence of endocrinology
https://t.me/med1917
For scie nti sts
• Define a syndrome, and match it to a gland malfunction.
• Measure the gland’s output in the peripheral blood. Define clinical syndromes
associated with too much or too little secretion (hyper- and hypo- syndromes,
respectively; eu- means normal, neither nor , as in euthyroid). Note factors
that may make measurement variable, eg diurnal release of cortisol.
• If suspecting hormone deficiency, test by stimulating the gland that produces it
• If suspecting hormone excess, test by inhibiting the gland that produces it (eg
• Find a way to image the gland. NB: non- functioning tumours or ‘incidentalomas’
• Aim to halt disease progression; diet and exercise can stop progression of im-
Endocrinologists love this reductionist approach, but have been less successful at
understanding emergent phenomena— those properties and performances of ours
that cannot be predicted from full knowledge of our perturbed parts. We understand the diurnal nature of cortisol secretion, for example, but the science of relating this to dreams, the consolidation of memory, and the psychopathology of
families and other groups (such as the endocrinology ward round you may be about
to join) is in its infancy. But as doctors we are steeped in the hormonal lives of patients (as they are in ours)— and we may as well start by recognizing this now.
For thos e do ing exams
‘What’s wrong with him?’ your examiner asks, boldly. While you apologize to
the patient for this rudeness by asking, ‘Is it alright if we speak about you as if
you weren’t here?’, think to yourself that if you were a betting person you would
wager that the diagnosis will be endocrinological. In no other discipline are gestalt impressions so characteristic. To get good at recognizing these conditions,
spend time in endocrinology outpatients and looking at collections of clinical
photographs. Also, specific cutaneous signs are important, as follows.
Thyrotoxicosis: hair loss; pretibial myxoedema (confusing term, p
(nail separation from the nailbed); bulging eyes (exophthalmos/ proptosis).
Hypothyroidism: hair loss; eyebrow loss; cold, pale skin; characteristic face. You
might, perhaps should, fail your exam if you blurt out ‘Toad- like face’.
Cushing’s syndrome: central obesity and wasted limbs (= ‘lemon on sticks’ see fig
proximal myopathy; moon face; bualo hump; supraclavicular fat pads; striae.
Addison’s disease: hyperpigmentation (face, neck, palmar cre-
ases), postural hypotension.
Acromegaly: acral (distal) + soft tissue overgrowth; big jaws
(macrognathia), hands, and feet; the skin is thick; facial features
are coarse.
Hyperandrogenism (): hirsutism; temporal balding; acne.
Hypopituitarism: pale or yellow- tinged thinned skin, resulting in
fine wrinkling around the eyes and mouth, making the patient
look older.
Hypoparathyroidism: dry, sc aly, puy skin; brittle nails; coarse hair.
Pseudohypoparathyroidism: short stature, short neck, and short
4
th and 5th metacarpals.
ACTH
(eg short
not functioning normally, there will be a blunted response to stimulation.
dexamethasone suppression test in Cushing’s). If there is a hormone- secreting
tumour then this will fail to suppress via normal feedback mechanisms.
may be found in health, see
paired fasting glucose to frank diabetes.
will depend on understanding autoimmunity, and the interaction of genes and
environment. In thyroid autoimmunity (an archetypal autoimmune disease), it is
possible to track interactions between genes and environment (eg smoking and
stress) via expression of immunologically active molecules (
adhesion molecules, cytokines,
stimulation test or Synacthen® test in Addison’s). If the gland is
p
218
. Imaging alone does not make the diagnosis.
1,
2
For other glands, halting progression
HLA
CD40
, and complement regulatory proteins).
class I and II,
212
3
); onycholysis
Fig 5.
2
‘Lemon
on sticks.’
197
5.2
);
5 Endocrinology
Endocrine physiology
https://t.me/med1917
198
Hormones are chemical messengers which act directly on nearby cells (paracrine
eect), on the cell of origin (autocrine eect), at a distant site (endocrine eect),
or as neurotransmitters (brain and gastrointestinal tract). Thirst, thermal regulation, appetite, sleep cycles, menstrual cycle, and stress/ mood are all controlled
by the hypothalamus. Releasing factors produced by the hypothalamus reach the
pituitary via the portal system (pituitary stalk), see
stimulate or inhibit the production of hormones from the anterior pituitary,
Vasopressin and oxytocin are produced in the hypothalamus and stored and released from the posterior pituitary.
fig
5.3
. The releasing factors
fig
5.4
.
5 Endocrinology
Fig 5.
https://t.me/med1917
3
Hypothalamic– pituitary axis.
199
Fig 5.
4
Neuroregulation and integration of endocrine axes makes us who we are— and who we
are and what we do feeds back into our hormonal milieu. Multifactorial disruptions within the
growth hormone (
and insulin axes play a major role in healthy maturation and ageing.
GH
), luteinizing hormone (LH)– testosterone, adrenocorticotropin (
ACTH
)– cortisol,
5 Endocrinology
Diabetes mellitus (DM): classification and diagnosis
LADA
https://t.me/med1917
200
DM
results from lack, or reduced effectiveness, of endogenous insulin.
Hyperglycaemia is one aspect of a far- reaching metabolic derangement,
which causes serious microvascular (retinopathy, nephropathy, neuropathy)
or macrovascular problems: stroke,
So think of
DM
as a vascular disease:1 adopt a holistic approach and consider
MI,
renovascular disease, limb ischaemia.
other cardiovascular risk factors too.
Categories of diabetes
1
Type 1 DM Usually adolescent onset but may occur at any age. Cause: insulin defi-
ciency from autoimmune destruction of insulin- secreting pancreatic cells. Patients
must have insulin, and are prone to ketoacidosis and weight loss. Associated with
other autoimmune diseases (>
tical twins, indicating environmental influence. >
diagnosis will have positive islet cell antibodies (
(antibodies to glutamic acid decarboxylase
insulinoma- associated protein
2
Type 2 DM (Formerly non- insulin- dependent DM.) This is at ‘epidemic’ levels in
many places, mainly due to changes in lifestyle, but also because of better diagnosis and improved longevity.
18
%). Most are over 40yrs, but teenagers are now getting type 2
(up to
p
246
). Cause: insulin secretion ± insulin resistance. It is associated with obesity,
lack of exercise, calorie and alcohol excess.
indicating stronger genetic influence than in type
preliminary phase of impaired glucose tolerance (
IFG)
. (This is a unique window for lifestyle intervention.)
(
3
Latent autoimmune diabetes of adulthood (
90
%
HLA DR3
±
DR4
). Concordance is only ~30% in iden-
90
% of people with type 1 DM at
ICA
) or other islet autoantibodies
65
(
GAD65
2
(
IA- 2
) and
IA- 2
4
Higher prevalence in Asians, men, and the elderly
), the tyrosine phosphatases,
beta; and zinc transporter (ZnT8)).
80
% concordance in identical twins,
1
DM. Typically progresses from a
IGT
) or impaired fasting glucose
) A form of type 1 DM, with
slower progression to insulin dependence in later life.
4
Monogenic forms of diabetes Including maturity- onset diabetes of the young
MODY
), and other genetic forms of diabetes, eg mitochondrial diabetes, genetic
(
lipodystrophies.
herited in an autosomal dominant pattern;
tations in genes encoding the transcription factors
glycolytic enzyme glucokinase (
5
Gestational diabetes May develop when there is failure to balance insulin se-
MODY
is thought to account for 1– 2% of people with diabetes, in-
GCK
MODY
is most commonly caused by mu-
HNF1A, HNF4A
, and
).
cretion with the composite of prepregnancy and pregnancy- induced insulin resistance (created by the anti- insulin hormones secreted by the placenta). It is
an increasingly prevalent condition (aecting between
women worldwide) and is associated with multiple adverse maternal and fetal
5
outcomes.
6
Pancreatic causes Diseases of the exocrine pancreas, eg pancreatitis; surgery
90
(where >
% pancreas is removed); trauma; pancreatic destruction (haemo-
2
% and 38% of pregnant
chromatosis, cystic fibrosis); pancreatic cancer.
7
Endocrinopathies Eg Cushing's syndrome, acromegaly, phaeochromocytoma.
8
Drug- induced diabetes Eg steroids, anti-
Categories of increased risk for diabetes (prediabetes)
• Impaired fasting glucose: fasting plasma glucose (5.6– 6.9mmol/ L).
• Impaired glucose tolerance: 2- hour post glucose load on the 75g oral glucose toler-
• Non- diabetic hyperglycaemia: impaired fasting glucose, or HbA1c 6.0– 6.4%
Incidence of
ance test
(
42– 46
mmol/ mol).
7.8– 11.0
mmol/ L.
DM
if
IFG
and HbA1c at high end of normal (42– 46mmol/ mol) is ~25%.
HIV
drugs, newer antipsychotics.
HNF1B
DM (OHCS
, and the
6
1
Chicken or egg? Most type 2 diabetes- associated genes have a function in the vasculature, and stress in
- cells can result from vascular defects in the pancreas, so maybe vascular events trigger
DM
.
7
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