Musc2 Flashcards
Vasculitides and autoimmune conditions
What is sarcoidosis?
Multisystem granulomatous inflammatory disorder characterised by the presence of non-caseating granulomas
What is the epidemiology of sarcoidosis?
Afro-Caribbeans and Scandinavians
>50yrs
What are the clinical features of sarcoidosis?
General: fever, malaise, weight loss Pulmonary: SOB, dry cough Musc: arthralgia Eyes: uveitis, keratoconjunctivitis Skin: lupus pernio, erythema nodosum Cardio: arrhythmia, heart failure
What are the investigations for sarcoidosis?
Bloods: -high Ca -high ACE -high ESR CXR: -bilateral hilar lymphadenopathy -pulmonary infiltrates/fibrosis Biopsy: non-caseating granuloma
What is the management for sarcoidosis?
Steroids
NSAIDs
Steroid-sparing agents
What is systemic lupus erythematosus?
A multi-system inflammatory autoimmune disorder
What is the epidemiology for SLE?
Young
Afro-Caribbean
Female
What are the symptoms of SLE?
SOAP BRAIN MD
Serositis Oral ulcers Arthritis Photosensitivity Bloods (low) Renal (proteinuria, casts) ANA Immunological (Anti-dsDNA) Neurological (psychosis, seizures) Malar rash Discoid rash
What are the investigations for SLE?
Bloods: ESR, U+E, FBC
Autoantibodies: anti-dsDNA, ANA, anti-cardiolipin
Urine: casts, proteinuria, haematuria
CXR
What is a common complication of SLE?
Anti-phospholipid syndrome
What is anti-phospholipid syndrome?
Triad of: -thromboembolism -recurrent miscarriage -thrombocytopenia Presence of anti-cardiolipin antibody
What is systemic sclerosis (scleroderma)?
Rare connective tissue disorder characterised by widespread blood vessel damage and fibrosis in skin and internal organs
What are some types of systemic sclerosis?
Diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis (CREST)
What are the characteristics of diffuse cutaneous systemic sclerosis?
Skin changes involving the trunk Raynaud's phenomenon Tendon friction Early lung disease Heart, GI, renal disease Anti-topoisomerase II antibody
What are the characteristics of limited cutaneous systemic sclerosis?
Calcinosis Raynaud's phenomenon oEsophageal dysmobility Sclerodactyly Telangectasia Anti-centromere antibody
What is vasculitis?
Inflammation of the blood vessel walls, with often an unknown aetiology, systemic effects, and distinctive features.
What is giant cell arteritis?
Idiopathic large vessel vasculitis, associated with polymyalgia rheumatica
What are the symptoms of GCA?
Unilateral headache Jaw claudication Scalp tenderness Loss of vision Systemic upset (fever, malaise, weight loss) Age >50
What are the investigations for GCA?
ESR
Temporal artery biopsy (not 100% accurate due to skip lesions)
What is the management for GCA?
High dose oral prednisolone straight after measuring ESR
What is polymyalgia rheumatica?
Inflammatory condition of unknown cause, characterised by bilateral pain and morning stiffness in the shoulder, neck, and pelvic girdle.
What is the aetiology of PMR?
> 50
15% of PMR Pts develop GCA
What are the clinical features of PMR?
Chronic
Bilateral shoulder/pelvic pain
Morning stiffness
No weakness
What is polyarteritis nodosa?
A rare vasculitis that affects medium sized vessels
What is the background of PAN?
Idiopathic
Associated with Hep B
What are the clinical features of PAN?
Constitutional upset Skin rash Abdo pain Testicular pain Rectal bleeding Peripheral neuropathy Renal failure Hypertension
What can be seen in a renal angiogram in PAN?
Rosary sign
What is granulomatosis with polyangiitis?
Triad of: -URT (rhinitis, epistaxis) -LRT (haemoptysis, heamorrhage) -kidneys (glomerulonephritis) Saddle nose
What are the investigations for GPA?
Serum cANCA
CXR- cavitating lesions
What is eosinophilic granulomatosis with polyangiitis?
Tri-phasic:
-allergic: asthma/rhinitis
-eosinophilic: eosinophils damage the lungs/gut
-vasculitic: can lead to widespread vessel damage and death
Other features: haemoptysis, rash, focal neuropathy, kidney damage
What are the investigations for eGPA?
pANCA
What is Behcet’s disease?
Triad of oral ulcers, genital ulcers, and uveitis
What is the background of Behcet’s disease?
Common in the Mediterranean (Greasy Turkeys)
Associated with HLA-B51
What are the clinical features of Behcet’s disease?
Triad of: -recurrent oral ulcers -genital ulcers -uveitis Also colitis, rash, arthritis, pericarditis (CRAP)
What are the investigations for Behcet’s disease?
Clinical diagnosis
Can confirm with Pathery test
What is the Pathergy test?
Needle skin prick forms a sterile pustule within 48h
What is Takuyasu’s arteritis?
Inflammation of the aortic branches
What is the background of Takuyasu’s?
Affects Asian women in the 20-40s
What are the symptoms of Takuyasu’s?
Inflammatory phase: FLAWS
Pulseless phase: weak/absent UL/LL pulses, claudication
What is the background of Henoch-Schönlein purpura?
Affects 3-15 yr olds
IgA vasculitis
What are the symptoms of Henoch-Schönlein purpura?
Triad of:
- purpuric rash on buttocks and extensors of lower limbs
- abdo pain
- arthralgia
What is the background of Goodpasture’s syndrome?
Anti-GBM antibodies
What are the symptoms of Goodpasture’s syndrome?
Triad of:
-glomerulonephritis
-haemoptysis (pulmonary haemorrhage)
Anti-GBM antibodies
A 40-year-old Afro-Caribbean man has suffered from shortness of breath and a dry cough for the last 3 months. He also complains of some ‘sore lumps on his shins’. Closer inspection reveals tender violet nodules on both shins. A chest X-ray is requested, which shows bilateral hilar lymphadenopathy. Blood tests are also requested, including U&Es – which parameter would you expect to be raised?
A. Sodium B. Potassium C. Calcium D. pH E. Urea
C. Calcium
A 33-year-old female with SLE presents to the fertility clinic complaining that she has been desperately trying to start a family but has had repeated miscarriages. She has had 3 miscarriages in the past 5 years. She has a past medical history of asthma and two DVTs. Given the likely diagnosis, which of the following antibodies is associated with this disease?
A. Anti-CCP antibody B. Anti-Jo-1 antibody C. Anti-centromere antibody D. Anti-cardiolipin antibody E. Anti-smooth muscle antibody
D. Anti-cardiolipin antibody
A 58-year-old woman presents to her GP complaining of difficulty swallowing which started 6 months ago. On examination, the skin on her hands appears thickened and tight around her fingers. Furthermore, a hard lump is felt under the skin on her left thumb. Which of the following additional clinical features would support a diagnosis of limited cutaneous systemic sclerosis?
A. Telangiectasia B. Thickened skin on the chest C. Macroglossia D. Buccal pigmentation E. Cherry haemangioma
A. Telangiectasia
A 63-year-old woman presents to A&E with a headache, mainly affecting the left half of her forehead, that has gradually been getting worse over the past week. She has been eating less as she experiences pain in her jaw when she chews her food. She has, generally speaking, been healthy aside from experiencing some stiffness and pain in her shoulders over the past 6 months. What is the first step in her management?
A.Check ESR B.Temporal artery biopsy C.IV hydrocortisone D. Oral prednisolone E.IV antibiotics
A.Check ESR
Which of the following conditions is strongly associated with temporal arteritis?
A. Takayasu’s aortitis B. Myalgic encephalomyelitis C. Fibromyalgia D. Polymyalgia rheumatica E. Polymyositis
D. Polymyalgia rheumatica
A 47-year-old man visits his GP having developed a skin rash. He has been feeling ‘generally unwell’ for the past 3 months, and has suffered from abdominal pain accompanied by some rectal bleeding. An angiogram reveals ‘Rosary sign’. He regularly attends hospital for check-ups since he was diagnosed with chronic hepatitis B, 2 years ago. What is the most likely diagnosis?
A. Giant cell arteritis B. Dermatomyositis C. Polyarteritis nodosa D. Granulomatosis with polyangiitis E. Behcet’s disease
C. Polyarteritis nodosa
A 52-year-old man has suffered from rhinitis and recurrent nosebleeds for the past 5 months. Initially, he did not think much of it, until he began coughing up a small about of blood about 3 weeks ago. A urine dipstick reveals proteinuria and haematuria. Blood tests reveal:
ESR: 72 mm/hr (< 20 mm/hr)
cANCA: positive
What is the most likely diagnosis?
A.Microscopic polyangiitis B.Goodpasture’s syndrome C.Granulomatosis with polyangiitis D.Churg-Strauss syndrome E. Behçet’s disease
C.Granulomatosis with polyangiitis
A 45-year-old man from Cyprus presents with recurrent ulcers on his penis. He has not noticed any discharge from his penis or pain whilst urinating. He adds that he has also developed mouth ulcers several times over the past year. During the consultation, you notice that his eyes are quite red. When questioned, he says that his eyes have been itchy recently, and thinks that he might have hay fever. What is the most likely diagnosis?
A.Inflammatory bowel disease B.Behçet’s disease C.Herpes simplex virus D.Syphilis E.Reactive arthritis
B.Behçet’s disease
Churg-Strauss syndrome is associated with:
A. pANCA B. cANCA C. Anti-GBM antibodies D. Anti-LKM antibodies E. Anti-smooth muscle antibodies
A. pANCA
A 43-year-old gardener is brought into A&E after having a seizure. His seizure lasts about 2 mins and disappears by itself. He says that this has never happened before and his only health problem has been a persistent headache that has developed over the past 6 months. On examination, a single heavily pigmented skin lesion is noticed on his right forearm. An MRI head scan reveals several lesions in both hemispheres.
What is the most likely diagnosis?
A. Neurofibromatosis Type 1 B. Meningioma C. Tuberous sclerosis D. Metastases E. Glioblastoma multiforme
D. Metastases
New onset seizure, chronic persistent headache
Indicates SOL
Heavily pigmented skin lesion indicates malignant melanoma
Several lesions in both hemispheres leads to a suspicion of metastases
