Histopathlogy 5: Connective Tissue Disorders Flashcards
List 3 auto-antibodies seen in SLE ?
Anti-dsDNA
Anti-Smith
Anti-histone (drug related)
What type of hypersensitivity reaction is seen in SLE ?
Type 3 hypersensitivity reaction
What is the HLA association with SLE ?
HLA DR3
Name the heart condition that can be caused by SLE ?
Libman-sack endocarditis
List the signs and symptoms of SLE using the mnemonic SOAP BRAIN MD
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood disorders- AIHA, ITP Renal involvement - Type 2 RPGN ANA +ve Immune- anti-dsDNA, anti-smith Neuro symptoms Malar rash Discoid rash
Which auto antibodies are seen in Diffuse scleroderma ?
Anti-scl70
Which autoantibodies are seen in limited scleroderma (CREST) ?
Anti-centromere
Which HLA is associated with scleroderma ?
HLA DR5
List the signs and symptoms of limited scleroderma (CREST) ?
Calcinosis Raynauds Esophageal dysmotility Sclerodactily Telangiectasia
No involvement of the trunk
In scleroderma there is increased ……… deposition in the skin causing tight skin
Collagen
Which autoantibodies are seen in Polymyositis and dermatomyositis ?
anti- Jo1
Patient has proximal muscle weakness. Their bloods show increased CK and abnormal EMG. No rash is seen.
Most likely diagnosis?
Polymyositis
Patient has proximal muscle weakness. Their bloods show increased CK and abnormal EMG. A heliotrope rash is seen on the eyelids. There are Gottron papules on the knuckles
Most likely diagnosis?
Dermatomyositis
Japanese lady with absence of pulse and Claudication.
The Rheumatologist says that its a large vessel arthritis.
Most likely diagnosis ?
Takayasu’s arteritis
Elderly man presents with a temporal headache, jaw claudication and scalp tenderness.
Most likely diagnosis?
Temporal arteritis
Large vessel vasculitis
A man presents with abdo pain and signs of renal impairment. Angiogram shows “rosary beads” because of multiple aneurysms.
Most likely diagnosis?
Associated with which disease?
Polyartheritis nodosa (medium vessel arteritis) Hepatitis B
List the diagnostic features of Kawasaki disease?
Fever >5 days Erythematous Rash on palms and soles Non purulent conjunctivitis Cervical lymohadenopathy Coronary artery aneurysms Mucous membrane involvement (strawberry tongue, cracked lips)
A heavy smoker with ulceration of the toes and fingers has an angiogram which shows a “corkscrew appearance”.
Most likely diagnosis?
Buerger’s disease
List the triad of features seen in Polyangitis with granulomatosis ?
Saddle nose
Pulmonary haemorrhage
Glomerulonephritis (Crescentic)
Which ANCA is associated with polyangitis with granulomatosis (Wagner’s) ?
C-ANCA
List 3 characteristic features of Eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome) ?
Asthma
Eosinophilia
P-ANCA vasculitis
List 3 features of Microscopic polyangitis?
Pulmonary haemorrhage (haemoptysis)
Glomerulonephritis (Pauci immune RPGN)
P-ANCA small vessel vasculitis
Which type of amyloidosis is seen in multiple myeloma ?
AL amyloidosis (primary)
With free light chains, monoclonal Ig and Bence jones proteins in the urine
List some causes of AA amyloidosis?
Rheumatoid arthritis
Chronic TB infection
IBD
Hodgkin’s lymphoma
In amyloidosis associated with haemodialysis you get ………… deposition ?
Beta 2 microglobulin deposition
Name one familial amyloidosis ?
Familial Mediterranean fever
Congo red staining shows ……………. under polarised light if there is Amyloidosis
apple green birefringence
An Afro Caribbean patient presents with SOB and cough. Examination shows erythema nodosum, lymphadenopathy and hepatosplenomegaly. X-ray shows bilateral hilar lymphadenopathy
Histology shows: non-caseating granulomas
Most likely diagnosis?
Sarcoidosis
Which electrolyte is often elevated in Sarcoidosis?
Ca2+
