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Station 5 notes by Dr RAPHAEL SCHEE JIE

PING

STATION 5 : BRIEF CLINICAL CONSULTATION


RHEUMATOID ARTHRITIS

1. Respiratory manifestations of rheumatoid arthritis : Basal pulmonary fibrosis


Bronchiolitis obliterans Bronchiectasis Pleural effusion Pleural thickening
Pulmonary nodules Caplan's syndrome Pneumonitis Pulmonary
hypertension

2. Anaemia in rheumatoid arthritis : Anaemia of chronic illness Iron-deficiency (GI


bleed dt NSAIDs) Pernicious (associated autoimmune) Folate deficiency
(methotrexate) Bone marrow suppression (methotrexate) Autoimmune
haemolytic Felty's syndrome Anaemia of CKD

3. Renal involvement in rheumatoid arthritis : I. Renal diseases secondary to disease


progress Membranous glomerulonephritis Chronic tubulointerstitial nephritis
Renal amyloidosis II. Renal diseases secondary to medications Membranous
glomerulonephritis (gold or penicillamine) Acute tubulointerstitial nephritis
(NSAIDs) Renal papillary necrosis (analgesic abuse) Minimal change disease
(NSAIDs)
SYSTEMIC LUPUS ERYTHEMATOSUS

1. Skin Malar (butterfly) rash Discoid rash Photosensitivity Vasculitic lesions


Purpura Livedo reticularis Telangiectasia Subcutaneous nodules

2. Hands Palmar erythema Non-erosive polyarthritis of small joints (Jaccouds


arthropathy) Raynauds phenomenon Poly- or dermatomyositis

3. Nails Splinter haemorrhage Nail-fold capillaries Periungal infarct


4. Scalp - scarring alopecia
5. Mouth - ulcers
6. Eyes Sjogrens Roth spots Papilloedema
7. Respiratory Interstitial lung disease Pleural effusion Pleurisy / pleural rub
8. Cardiovascular Pericarditis Myocarditis Cardiac failure Libman-Sacks
endocarditis Accelerated coronary atherosclerosis Pulmonary hypertension

9. Neurological Seizure Vasculitic CVA hemiparesis, ataxia Chorea Aseptic


meningitis Mononeuritis multiplex Polyneuropathy

10. Renal - lupus nephritis Hypertension Oedema Proteinuria Heamaturia


11. Haematological NCNC anaemia Haemolytic anaemia Leukopaenia
Thrombocytopaenia Pancytopaenia Lymphadenopathy Splenomegaly
Autoantibodies : ANA in 95% Anti-dsDNA specific ENA (anti-Ro, anti-La)
in ANA negative lupus Anti-histone drug-induced lupus Antiphospholipid
anticardiolipin, lupus anticoagulant
Drug-induced lupus : Procainamide Isoniazid Hydralazine Phenytoin
Carbamazepine Oral contraceptive

Non-pharmalogical management Sunscreen Avoid high dose oestrogen pill


Remove offending drugs Smoking cessation Primary prevention of
cardiovascular disease

Pharmacological NSAIDs Anti-malarial hydroxychloroquine Corticosteroids


Cytotoxic azathioprine, cyclophosphamide, mycophenolate mofetil, cyclosporin

http://www.rheumtutor.com/2012-slicc-sle-criteria/

http://aperito.org/uploads/pdf/ADTAOA-2-114.pdf

RAYNAUDS PHENOMENON / SECONDARY RAYNAUDS

1. Rheumatological / Connective tissue diseases Systemic sclerosis


Rheumatoid arthritis Systemic lupus erythematosus Antiphospholipid
syndrome Polymyositis Dermatomyositis Mixed connective tissue disease
Sjogrens syndrome Ehlers-Danlos syndrome

2. Medications Beta-blockers Oral contraceptive pills Ciclosporin


Chemotherapeutic agents esp Bleomycin

3. Haematological Polycythaemia Thrombocytosis Monoclonal gammopathy


(paraproteinaemia) Myeloproliferative diseases Cryoglobulinaemia Cold
agglutinin diseases e.g. Mycoplasma

4. Endocrinological - Hypothyroidism

5. Obstructive (vascular) Atherosclerosis Buergers disease Takayasus


arteritis Subclavian aneurysm Thoracic outlet syndrome Cervical ribs

6. Occupational - Vibrating tools

MANAGEMENT

General measures Patient education Smoking cessation Hand warmer /


gloves Avoid causative medications

Pharmacological Calcium channel blockers Nitrates Prostacyclin analogues

Surgical - Digital sympathectomy


Painful joints 1. Rheumatoid arthritis 2. Osteoarthritis 3. Gouty arthritis 4.
Pseudogout 5. Seronegative "PEAR" 6. Systemic lupus erythematosus 7. Septic
arthritis - gonococcal or non-gonococcal, mycobacteria, fungal 8. Systemic - Still's
disease, rheumatic fever, Lyme

Seronegative arthritis / spondyloarthritis Mnemonic "PEAR" ->Psoriatic


arthropathy ->Enteropathic arthritis ->Ankylosing spondylitis ->Reactive arthritis

7 A's of ANKYLOSING SPONDYLITIS

1. Anterior uveitis

2. Atlanto-axial subluxation

3. Apical pulmonary fibrosis

4. Aortic regurgitation

5. Atrioventricular conduction defect

6. Achilles tendonitis

7. Amyloidosis

DERMATOMYOSITIS

Features : ->Heliotrope rash ->Gorton papules ->Malar erythema ->Telangiectasia


->Raynaud's phenomenon ->Proximal myopathy ->Arthritis ->Dysphagia
->Interstitial lung disease

Screen for malignancy : ->Lung ->Gastrointestinal ->Breast ->Ovarian ->Prostate

Investigation : ->Serum CK ->EMG ->Muscle biopsy

Treatment : ->Educate ->Physiotherapy ->Sunscreen ->Skin - MTX, MMF ->Muscle


- steroid, MTX, Azathioprine, MMF, IVIG
VITILIGO Common associations : Alopecia areata Hashimotos disease
Graves disease Addisons disease Diabetes mellitus HypoPTH Primary
biliary cirrhosis Atrophic gastritis Pernicious anaemia

PURPURA

1. Platelet defect Thrombocytopenia ** Uraemia Chronic liver disease

2. Capillary defect Old age Corticosteroid Vasculitis*** Cholesterol


embolism Ehlers-Danlos Hereditary haemorrhagic telangiectasia Amyloidosis

3. Coagulation defect Meds aspirin, heparin, warfarin Von-Willebrands


Haemophilia DIVC

**thrombocytopenia Meds DIVC Bone marrow (haemato malignancy nd


myeloproliferative disease) Hypersplenism Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura Haemolytic uraemic syndrome
Aplastic anaemia

***vasculitis Henoch-Schonlein purpura Polyarteritis nodosa Churg-Strauss


syndrome Wegeners granulomatosis Systemic lupus erythematosus
Sjogrens syndrome Infective endocarditis

Medications that can cause purpura Aspirin Heparin Warfarin NSAIDs


Penicillins Tetracyclines Phenytoin

ERYTHEMA NODOSUM

1. Meds OCP Penicillin Tetracycline Sulphonylurea

2. Bacterial Streptococcal (group A beta-haemolytic) TB Leprosy Syphilis


Mycoplasma

3. Gastrointestinal Inflammatory bowel disease Salmonella Campylobacter

4. Viral EBV CMV

5. Fungal histoplasmosis

6. Myeloproliferative disorders

7. Behcets disease recurrent oral and genital ulcers, uveitis, arthritis, colitis,
vasculitis, myocarditis, pericarditis
ERYTHEMA MULTIFORME

1. Idiopathic

2. Meds OCP Penicillin Tetracycline Sulphonylurea Phenytoin


Carbamazepine Salicylate NSAIDs

3. Bacterial Streptococcal (group A beta-haemolytic) TB Syphilis


Mycoplasma

4. Gastrointestinal Salmonella Campylobacter

5. Viral HSV EBV CMV HIV Varicella zoater virus HAV, HBV, HCV

6. Fungal histoplasmosis

7. Parasitic toxoplasmosis

Stevens-Johnson Syndrome 1. Identify & treat underlying causes 2. Withdrawal


of causative meds 3. Analgesics PRN 4. Anti-pyretics 5. IV fluids and electrolytes
6. Antibiotics for secondary infection 7. Wound care 8. Eye care

VASCULITIS

1. Large vessel :

-> Giant cell (temporal) arteritis -> Takayasu's arteritis

2. Medium-sized vessel :

-> Polyarteritis nodosa -> Kawasaki disease

3. Small vessel : -> p-ANCA : microscopic polyangiitis, glomerulonephritis, Churg-


Strauss syndrome -> c-ANCA : Wegener's granulomatosis -> ANCA -ve : Henoch-
Schonlein purpura, Goodpasture's syndrom, cryoglobulinaemia

HYPERTHYROIDISM
1. Inspection : thin, staring, fidgety

2. Hands : warm sweaty palms thyroid acropachy fine tremor

3. Pulses : tachycardia, atrial fibrillation

4. Limbs : proximal myopathy weakness

5. Eyes : Lid retraction Exophthalmos Periorbital oedema Chemosis


Proptosis Lid lag Ophthalmoplegia

6. Neck : cervical lymphadenopathy, scar

7. Thyroid : Diffused or nodular Terderness Can get below it? Percussion for
retrosternal extension Auscultation for bruits

8. CVS : cardiomyopathy - displaced apex, failure features

9. Lower limbs : pretibial myxoedema or pedal oedema

Aetiologies

1. Increased hormone synthesis : Geaves disease Hashimotos (thyrotoxicosis


prior to hypothyroidism) Toxic adenoma Toxic multinodular goitre Thyroid
malignancy Iodine-induced contrast agents, amiodarone Over replacement
of levothyroxine TSH-secreting pituitary tumor

2. Gland inflammation and release of preformed hormone Subacute (de


Quervains) thyroiditis Infective thyroiditis (bacterial, fungal, TB) Radiation
thyroiditis Postpartum thyroiditis Medication-induced thyroiditis (amiodarone,
lithium)

Radioactive iodine uptake scan : Multiple areas of increased uptake (MNG)


Single area of increased uptake (toxic adenoma) Diffuse increased uptake
(Graves) Cold nodule (thyroid carcinoma)

GRAVES' DISEASE 60-80% of hyperthyroidism

Graves' ophthalmopathy : 1. Periorbital oedema 2. Conjunctival oedema


(chemosis) 3. Exophthalmos 4. Ophthalmoplegia 5. Exposure keratitis / corneal
ulcer 6. Optic nerve compression - acuity, colour, RAPD

Features of Graves' disease independent of hyperthyroidism : 1. Goitre 2. Graves'


ophthalmopathy 3. Thyroid acropachy 4. Pretibial myxoedema
B and T lymphocyte-mediated autoimmunity : 1. TSH receptor Ab 2. Anti-
thyroglobulin Ab 3. Anti-thyroperoxidase Ab 4. Sodium-iodide symporter Ab

Possible associated autoimmune conditions : 1. Type 1 DM* 2. Addisons disease*


3. Pernicious anaemia* 4. Chronic atrophic gastritis* 5. Vitiligo* 6. Alopecia
areata* 7. Coeliac disease* 8. Primary hypogonadism* 9. HypoPTH 10.
Rheumatoid arthritis 11. SLE 12. Sjogren's syndrome *Autoimmune Polyendocrine
Syndrome type 2

NEUROFIBROMATOSIS (NF)

Autosomal Dominant

Features of NF-1 :

1. Skin Caf-au-lait spots Axillary and inguinal freckling Neurofibromata


subcutaneous, cutaneous, plexiform

2. Musculoskeletal Kyphoscoliosis Dysplasia of skull (sphenoid) Charcots


joint

3. Eyes Lisch nodules (hamartomata) on the iris (slit lamp) Retinal


hamartomata Hypertensive retinopathy

4. Respi Pulmonary fibrosis Pneumothorax (lung cysts)

5. CVS restrictive cardiomyopathy

6. Renal renal artery stenosis

7. Endocrine phaeochromocytoma

Complications : Mild learning disability Nerve root compression weakness,


pain, paraesthesia GI bleed, obstruction Bone cystic lesion, scoliosis
Hypertension renal artery stenosis, phaeochromocytoma Malignancy
sarcomatous changes in neurofibromas Epilepsy increased risk Rare
association carcinoid syndrome
Features of NF-2 : Vestibular schwannoma / acoustic neuroma Neurofibroma
Meningioma Optic glioma Juvenile cataract

NF-1 diagnostic criteria : (2 and above of the following)

Six or more caf-au-lait spots or hyperpigmented macules >5 mm in diameter in


prepubertal children and >15 mm postpubertal Axillary or inguinal freckles (>2
freckles) Two or more typical neurofibromas or one plexiform neurofibroma
Optic nerve glioma Two or more iris hamartomas (Lisch nodules), often identified
only through slit-lamp examination by an ophthalmologist Sphenoid dysplasia or
typical long-bone abnormalities such as pseudarthrosis First-degree relative (eg,
mother, father, sister, brother) with NF1

NF-2 diagnostic criteria : (At least one of the following)

1. Bilateral vestibular schwannomas

2. A first degree relative with NF2 AND Unilateral vestibular schwannoma OR


Any two of: meningioma, schwannoma, glioma, neurofibroma, posterior
subcapsular lenticular opacities

3. Unilateral vestibular schwannoma AND Any two of: meningioma,


schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities

4. Multiple meningiomas AND Unilateral vestibular schwannoma OR Any two


of: schwannoma, glioma, neurofibroma, cataract

YOUNG HYPERTENSION

1. Essential / primary / idiopathic

2. Renal Glomerulonephritis Polycystic kidney disease Systemic sclerosis


Lupus nephritis Polyarteritis nodosa Renal artery stenosis Fibromuscular
dysplasia

3. Endocrine Cushings syndrome Conns syndrome Phaeochromocytoma


Acromegaly Hyperparathyroidism

4. Medications Oral contraceptives MAOI Steroids

5. Others Coarctation of aorta Obstructive sleep apnoea

http://www.aafp.org/afp/2010/1215/p1471.html
HYPOCALCAEMIA

1. Hypoalbuminaemia

2. Hypomagnesaemia

3. Hypophosphataemia

4. Vitamin D deficiency

5. Hypoparathyroidism**

6. Pseudohypoparathyroidism

7. Acute Pancreatitis

8. Autosomal dominant hypercalciuric hypocalcaemia

**can be part of autoimmune polyendocrine syndrome type 1 with Addisons


disease, chronic mucocutaneous candidasis, hypogonadism, autoimmune primary
hypothyroidism, pernicious anaemia, vitiligo, alopecia, chronic active hepatitis

SHORT STATURE 1. Down's syndrome 2. Turner's syndrome 3. Achondroplasia 4.


Osteogenesis imperfecta 5. Hypothyroidism 6. Hypopituitarism 7. Chronic renal
failure 8. Renal tubular acidosis 9. Fanconi's syndrome 10. Malabsorption

TALL STATURE 1. Marfan's syndrome 2. Homocystinuria 3. MEN 2b 4. Acromegaly


/ gigantism 5. Thyrotoxicosis 6. Klinefelter's syndrome 7. Familial

MARFANS SYNDROME (Autosomal Dominant)

1. Inspection : Tall Arm span > height Kyphoscoliosis Pectus excavatum

2. Limbs : Arachnodactyly hand and wrist sign Joint hypermobility

3. Ocular : Upward lens displacement Myopia Heterochromia Blue sclera

4. Mouth : high-arched palate


5. Respi : Pectus excavatum Pneumothorax

6. Cardiovascular : Aortic regurgitation Aortic dilatation Aortic root aneurysm


Aortic dissection

Management of aortic disease : Lifelong beta-blockade Periodic routine


imaging of aorta Prophylactic replacement of aortic root before diameter
exceeds 5.5cm (5cm if family history or dissection) Moderate restriction of
physical activity

OSTEOPOROSIS secondary causes : 1. Multiple myeloma 2. Osteomalacia 3.


Thyrotoxicosis 4. Cushings syndrome 5. HyperPTH 6. Male hypogonadism

PROXIMAL MYOPATHY

non-neurological causes : 1. Cushings syndrome 2. Hyperthyroidism 3.


Polymyositis 4. Dermatomyositis 5. Polymyalgia rheumatica 6. Osteomalacia 7.
Paraneoplastic syndrome

PROXIMAL WEAKNESS

A. Myopathies 1. Endocrine -> Cushings syndrome -> Hyperthyroidism ->


Acromegaly 2. Inflammatory myositis -> Polymyositis -> Dermatomyositis 3.
Drug-induced -> Statins -> Alcohol 4. Metabolic -> Glycogen storage disease
(McArdles disease) -> Mitochondrial myopathy

B. Neuromuscular junction -> Myasthenia gravis -> Lambert-Eaton myasthenic


syndrome

C. Muscular dystrophies -> Duchenne muscular dystrophy X-lined recessive ->


Becker muscular dystrophy X-linked recessive -> Facioscapulohumeral muscular
dystrophy autosomal dominant in 80% -> Limb-girdle muscular dystrophy

DIZZINESS & VERTIGO

1. Cardiac Arrhythmia Aortic stenosis Hypertrophic obstructive


cardiomyopathy
2. Neurological Vertebra-basilar insufficiency Multiple system atrophy Chiari
malformation Cerebellar stroke Multiple sclerosis Brain tumour Wernickes
encephalopathy Lateral medullary syndrome Vertebral artery dissection

3. Peripheral Vestibular neuronitis Benign paroxysmal positional vertigo


Otitis media Menieres disease

4. Endocrine Hyperglycaemia Hypoglycaemia Addisons Hyperthyroidism

5. Haematological - anaemia

6. Dehydration

7. Vasovagal

8. Drugs Acute alcohol intoxication Phenytoin Anti-hypertensives


Ototoxicity from aminoglycosides / vancomycin Illicit drugs such as LSD

9. Psychological Hyperventilation Anxiety

SYNCOPE

1. Cardiac -> Arrhythmia -> Aortic stenosis -> HOCM

2. Respiratory - pulmonary embolism

3. Neurological -> Seizure -> TIA / stroke -> Tuberous sclerosis -> Alcohol -> Drug
withdrawal -> Electrolyte disturbance -> Lack of sleep -> Infection -
meningoencephalitis -> Scar epilepsy -> Autonomic dysfunction (DM / Parkinsons
/ MSA) -> Intracranial haemorrhage -> Brain tumour

4. Vasovagal -> Dehydration -> Carotid sinus hypersensitivity -> Micturition


syncope -> Postural hypotension

5. Drugs -> Benzodiazepines -> Opiates -> Unintentional overdose

6. Endocrine -> Hypoglycaemia -> Addisons

7. Symptomatic anaemia

8. Vertebro-basilar insufficiency - subclavian steal syndrome

9. Anxiety disorder

Gradual visual loss : -> cataract -> diabetic maculopathy -> age-related
macular degeneration -> retinitis pigmentosa -> glaucoma -> optic atrophy
Sudden visual loss : -> vitreous haemorrhage -> retinal artery occlusion ->
retinal vein occlusion -> haemorrhagic age-related macular degeneration ->
ischaemic optic neuropathy -> pituitary apoplexy -> CVA -> trauma

Rapid but not sudden : -> retinal detachment -> optic neuritis

Loss of peripheral vision : -> retinitis pigmentosa -> glaucoma -> laser
photocoagulation -> branch retinal artery/vein occlusion affecting periphery -
unilateral -> optic chiasmal lesion - bitemporal -> CVA - homonymous

Loss of central vision : -> diabetic maculopathy -> age-related macular


degeneration -> cataract -> optic neuropathy -> branch retinal artery/vein
affecting macula

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