Autoimmune Msk Problems Flashcards by Rahim Akram

What is the lab work of non-infectious inflammatory myopathies

Positive ANA
Anti-Jo-1 AB
Anti-Mi-2 AB’s
Increased creatinine and aldolase

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Give two examples of non-infectious inflammatory myopathies. What are they?

Don’t matter myositis + polymyositis

Immune-mediated disorders with symmetrical muscle involvement and involvement of other organs

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What does muscle biopsy show for polymyositis

Necrotic and regenerating muscle with the lymphocytic and macrophage infiltrate

atrophy not prominent

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What does muscle biopsies show for dermatomyositis

Inflammatory reaction i.e. lymphocytic

Atrophy of muscle fibres = prominent

Capillary damage + muscle – >ischaemia + atrophy

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If a patient presents with the matter my situs what else do you need to consider the possibility of?

Gastric carcinoma

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What is the aetiology of dermatomyositis

CD4 cells target capillaries + skeletal muscle

Antibodies + complement damage capillaries

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What are the clinical features of the dermato myositis

Rash + upper eyelid = purple = heliotrope/raccoons

Malar rash

Red GOTTRON papules + elbows knuckles knees

Hallmark bilateral proximal muscle weakness – Can’t come hair due to weak shoulders

Morning stiffness fatigue + dysphagia @ upper oesophagus

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Inside the muscle fascicle where exactly does the information occur in dermatomyositis?

Inflammation of the perimysium ->

perifascicular atrophy

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In polymyositis is their proximal or distal muscle weakness?
Is the skin involved?
Where is inflammation occurring?
What immune cell is hallmark?

Proximal muscle weakness
Skin not involved
Inflammation @endomysium with necrotic muscle fibres
CD8+

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What are antibodies directed against in polymyositis?

Transfer RNA synthetases

Nuclear + cytoplasmic antigens @ skeletal muscle

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What initiates the CD8 positive cells?

HIV,

HTLV – 1,

coxsackie B

Alter MHC 1 MHC 2 antigens

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Give the two types of X-linked muscular dystrophies

Duchenne muscular dystrophy

Becker muscular dystrophy

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What is Duchenne muscular dystrophy the characterised by

Muscle wasting: skeletal muscle –> adipose tissue

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Explain the genetics of Duchenne muscular dystrophy

X-linked

Frameshift mutation –Deletion of dystrophin gene – >

cannot anchor muscle fibres in skeletal + cardiac muscle– >

Can’t connect intracellular muscle cytoskeleton (actin)
to
transmembrane proteins alpha + beta dystroglycan. Which are connected to the extracellular matrix

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What does the truncated dystrophin protein lead to

Inhibited muscle regeneration

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Where does the muscle weakness begin from?
@ what age?
where does it progressed to?

Proximal muscle weakness @ 1 year old – >progress to distal muscle

E.g. begins in pelvic girdle muscle – >progress superiorly

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Explain the Hallmark issue related to the leg in Duchenne muscular dystrophy

Pseudohypertrophy of the calf

Learn to walk + Proximal muscle starts to we can – >child puts more force on calf muscle to walk – >hypertrophy

Overtime disease progresses – >
Muscle weakens as disease moves distally and affects calf – >convert calf muscle to fat– > calf looks big but is full of fat

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What does the damaged muscle cell release into the Serum?

Creatinine kinase + aldolase

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Why does the child normally die from Duchenne muscular dystrophy?

Cardiac/respiratory (diaphragm) failure

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Explain the difference between Becker muscular dystrophy + Duchenne muscular dystrophy

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In Becker muscular dystrophy disorder is due to non-frameshift insertion in dystrophin Jean i.e.

Protein is partially functional instead of truncated like Duchenne

Onset in Beckers is @adolescence/early adulthood
Onset intuitions is before 5 years old

Beckers is milder

Explain the genetics behind myotonic type one muscular dystrophy

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Autosomal dominant

CTG trinucleotide repeat @DMPK gene ->

Abnormal expression of mytonin protein kinase – >

atrophy type 1 fibres –>

MytoniA + cAtArAct
my tEsticles – Atrophy
My tIcker - arrhythmia
my tOUpee – Frontal balding

What is the pathogenesis of SLE

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Look at pic on 04/03/15

What are the genetic and environmental aetiologies?

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Genetic – (HLA–DR 2+3) & (compliment deficiency)

Environmental

Drugs = isoniazid procainamide hydralazine – >
bind to histones –>histones become immunogenic
->
auto-AB against histones = positive ANA test – > lupus like syndrome -
see all symptoms except CNS + renal involvement

Infections e.g. HIV/CMV/EBV -> polyclonal activation of B lymphocytes – >formation of autoAB against host tissue

Estrogen – > 
DECREASES p(apoptosis of self reactive B cells)

UV light

What is epitope spreading

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Self reactive lymphocyte – >
react against self-antigen – >
destroyed tissue – >
expose new antigens immune system hasn't seen before – >
produce new self reactive lymphocytes

Who does systemically lupus Erythematosus affect?

Middle-aged females Africans + Hispanics Happens to children and OLD ADULTS @Kids + older adults: 2 females: 1 male @Middle aged people: 10 females: 1 male

In lupus what does type 3 HSR rxn lead to? In lupus what does type 2 HSR rxn lead to?

Immune complexes = Information @ skin, BM, glomeruli, joints, vessels Auto antibodies = cytopenias E.g. anaemia thrombocytopenia leukopenia

What are the symptoms of SLE

Look at pic on 04/03/15

What are the pregnancy related findings in SLE

Heart block in the newborn as IgG anti-Ro/SS-A AB crosses placenta and attacks cardiac conduction system Antiphospholipid antibody binds to proteins on the trophoblast and causes abortion

Explain drug induced lupus in comparison to systemic lupus erythematosus

``` anti HHHHistone antibodies form decreased IIIIIncidence of renal + CNS issues No AAAAB against native DNA No decreased serum CCCCCompliment SSSSSSymptoms disappear when drugs stop ```

Treatment for lupus

Corticosteroids NSAIDs immunosuppressants hydroxychloroquine

What are the common causes of death due to lupus

Renal failure Infection – due to immunosuppressants/auto antibodies against white blood cells Later on in life = increased p(accelerated coronary atherosclerosis)

What is Sjogren's syndrome

Autoimmune destruction of exocrine glands especially lacrimal + salivary glands mediated by lymphocytes type 4 HSR + fibrosis

What does destruction of lacrimal + salivary glands result in?

Kerato conjunctivitis sicca / xerophthalmia - ⬇️ tear production – > dry eyes – > corneal damage Dental Caries Xerostomia - ⬇️ saliva production - dry mouth

Like every other autoimmune disease who is most likely to be affected by Sjogren's syndrome

Older women

Sjögren's can be a primary or a second airy disorder. As a secondary syndrome what can it be associated with?

Rheumatoid arthritis

What Ab can be present in the blood in sjogrens? | Can this be associated with rheumatoid arthritis

Rheumatoid factor No not necessarily

What are the extraglandular manifestations of Sjogren's syndrome

Joint pain CNS/skin issues

What are the lab findings and Sjogren's syndrome

Rheumatoid factor Positive ANA Anti ribonucleoprotein = anti SSA/SSB Anti-SSA: crosses placenta – > attack fetus --> neonatal lupus – > congenital heart block

Is the anti-SSA/SSB enough to make a diagnosis of Sjogren? What do you need to confirm Sjogrens?

Biopsy of minor saliva gland @lip – > look for lymphocytic SIALADENTIS = lymphocytes attack minor salivary glands = MALT

Explain the relationship between Sjogren's syndrome and parotid gland

Sjogren's syndrome = inflame parotid gland – > enlarged parotid gland – > B cell lymphoma cause unilateral enlargement of parotid gland

How do you treat Sjögren's syndrome

Artificial tears Cyclosporine/pilocarpine eyedrops Civimeline

What does the schimear test and Rose Bengal staying reveal for Sjögren's

Filterpaper placed inside of lower eyelid Wetting of <10 mm @ 5 minutes = ⬇️ tear problem Rose Bengal stain shows FILAMENARY keratitis + PUNCTATE keratitis

Explain the pathogenesis of systemic sclerosis scleroderma

AI rxn against AG @connective-tissue / endothelial cell – > endothelial cell dysfunction – >increased expression of Cellular adhesion molecules --> Th2 enter skin --> the endothelial cell secrete: PDGF + TGF beta – > activate fibroblast --> fibrosis around BV --> organ damage = systemic sclerosis Endothelin = vasoconstrictor –> ischaemia – > more fibrosis – >organ damage

What are the two forms of systemic sclerosis

Limited and diffuse

Explain limited systemic sclerosis scleroderma

Skin involvement = minimal = distal to knee + elbow Late visceral involvement Calcinosis = calcium deposition @ subcutaneous connective-tissue anticentromere Ab Raynaud phenomenal Esophageal dysmotility Sclerodactyly - fibrosis of skin + hands: tight skin – >lose wrinkles – >constrict blood vessels Telangiectasia– Dilated capillaries

Explain the pathogenesis of mixed connective-tissue disease

B + T-cell activation – >Ab against U1-ribonucleoprotein

What are the lab findings for mixed connective-tissue disease

Positive ana Auntie ribonucleoprotein against you U1-RNP

Clinical findings of mixed connective-tissue disease

``` Hypertension + pleuritis Arthralgia + arthritis of hands Raynaud Pericarditis Oesophageal dysfunction/dysmotility Neuralgia Trigeminal Sclerodactyly ```

Lab findings for systemic sclerosis

Positive ana For diffuse scleroderma – anti DNA topoisomerase 1 Ab = anti-Scl – 70 Ab

Autoimmune Msk Problems Flashcards

(49 cards)