Connective Tissue Diseases Flashcards by Jade Fellows

Common connective tissue diseases

SLE
Systemic sclerosis
Myosotis/ dermatmyositis
Sjogrens 
Mixed connective tissue diseases

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What makes up mixed connective tissue disease

SLE + systemic sclerosis+ RNP Ab

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Pathology of SLE

Pathogenic autoantibodies results in immune complex formation and complement mediated damage
IC = deposited in the tissues and are not cleared so this elicits inflammation via the classical pathway and activation of macrophages and neutrophils via surface FC receptors which bind to IgG.

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Epidemiology of SLE

Women>men

Afrocaribean and south Asian > white

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What are the systemic features of SLE

Fatigue acute/chronic
Fever
Lymphadenopathy 
Weight loss 
Myalgia

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Arthritis symptoms in SLE

Symmetrical non-erosive synovitis 
Arthralgia - more common than synovitis 
Jacobs arthropathy - where the deformities are reducible by the patient or dr 
Nodules 
SimilarRA - small joint involvement

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Skin Involvement in SLE

Photosensitivity - rash can appear only where sun exposure has been
Malar - butterfly rash on the face the nose and cheeks affected but the nasolabial folds are not affected
Discoid rash - can be scaring ans can occur in the absence of systemic features - cutaneous LE if ANA +ve more likely to progress to systemic
ALL can be made worse with UV light
Vasculitis rashes
Periungal erythema
Inflamm in the BV - infarction and necrosis

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What is Raynaud

types of raynaud’s you get in SLE

Raynauds in the reflex vasoconstriction of BV in the extremities - finger, hands, feet and nose in response to a drop in temperature
If associated with SLE or another systemic disease it is secondary Raynauds - Raynaud’s phenomena
If not associated with other conditions- Raynauds diseases primary raynauds

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What is livedo reticularis

Can get it wit SLE and vasculitis
It is blotches of purple/brown on the legs in a similar fishnet, distribution on the legs
In SLE they occur in patients who have anti-phospholipid syndrome who are more likely to clot

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Alopecia inSLE

Can be areata or total is
Can be scaring or non scaring
The scaring can be associated with a discoid rash
The scaring form the hair will not grow back

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Pulmonary features or SLE

Pleurisy - can be caused by SLE
But due to tx immunosuppression it may be caused by infection and due to the anti phospholipid syndrome it may be a PE
So it is important to rule these things out before attributing it to being a part of the illness
Fibrosis
Diffuse lung disease with a dry cough distinguish from infection again - inflam in the alveoli - pneumonitis which progresses to fibrosis
Pulmonary hypertension
- normalCXR it is a primary disorder can be characterised by a large heart reduced lung markings and a large pulmonary artery

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What doesn’t rule out infection in SLE

Fever as it is part of SLE

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Cardiac involvement in SLE

Pericarditis - inflamm of the lining of the heart
Myocarditis - cardiac failure and arrhythmia
Libman-sacks - non infectious vegetation of the heart can lead to emboli
CAD - early MI due to blood vessel inflammation

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Muscles membrane affected in SLE

Oral/nasal/ genital ulcers
May be painful and painless
Apthous ulcers no different to normal ulcers
Sicca symptoms -2ndary Sjogrens dry eyes and mouth due to destruction of exocrine glands - salivary and lacrimal

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Abdominal involvement SLE

Anorexia, N/V, pain, diarrhoea, vasculitis affecting intra ab organs
Ischaemic organs to anti phospholipid syndrome

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Kidney involvement SLE

Asymptomatic 
Proteinuria 
Blood cells 
RBC casts in the glomeruli 
Leading to ether a proliferative glomerulonephritis or a membranous glomerulonephritis

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Neurological involvement SLE

Cerebrovascular disease
Seizure
Chorea
Cog dysfunction - dementia/ pseudo dementia from depression as these patients are living and coming to terms with this chronic condition
Psychosis
Mononeuropathy/ mono multi/poly - glove and stocking

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Reproductive features of SLE

Recurrent miscarriages
Fetal growth restriction due to poor flow in the placenta
Neonatal lupus syndrome - due to the mothers anti-Ro and anti-La being passed to the Fetus this means the newborn could get a photosensitive rash if in the sun, may get congenital heart block
Premature menopause - auto ab against ovary or drug induced- cyclophosphamide

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Features of anti-phospholipid syndrome

Recurrent venous thrombosis
-DVT
-PE
Recurrent arterial thrombosis 
MI stroke 
Recurrent miscarriages 
Thrombocytopenia and interference with phospholipid dependent clotting tests

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Lab result features of SLE

Haematology
- anaemia: chronic disease, haemolytic, blood loss - iron def
- leukopenia - lymph and neutropenia common - tx or disease
Thrombocytopenia
- ESR inc
-CRP dec or small rise in comparison to ESR
Renal function
Antibodies - own slide

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Antibodies in SLE

ANA. Sensitive but not specific 
Anti-dsDNA -60% 
Ani - ENA - extractable nucleotide antibody 
Ro, La, Sm, RNP
Antiphospholipid ab
APTT prolonged 
Anti-cardiolipin ab 
Lupus anticoagulant

Complement levels in SLE why are they this way

Low
C3/4
They are low because they are being used up quickly

At is a marker of SLE disease activity

How Low complement is

How high do-DNA is

What are Ro and La associated with

Neonatal lupus

What are RNP ab associated with

SLE and systemic sclerosis | Mixed connective tissue disease

What are ant-smith ab associated with

No definite | ? Nephritis and CNS involvement

Mx of SLE

Con - sunblock, education, infection risk, fertility - contraception and what to do to ensure safe pregnancy Med - NSAIDS - not a lot due to the si - PPI, renal impairment - not use, CV risk and hypertension so used sparingly Corticosteroid - IM, IV, PO Hydroxychloroquine Introduce a immunosuppression Azathioprine Methotrexate, cyclophosphamide Mycofenalate Refractory disease - rituximab or anti CD22mab Use cyclosporine in leukopenia Mycophenolate and cyclophosphamide in renal impairment

What is systemic sclerosis

Generalised disorder of connective tissue affecting the skin and internal organs It is characterised by fibrotic arteriosclerosis or peripheral and visceral vasculature Extracellular matrix accumulation (collagen) in the skin and viscera Associated with auto ab

What is localised sclerosis

``` CREST Calcinosis Raynauds Oesophageal dysmotility Sclerodactyly Telangectasia ```

Systemic sclerosis effects on the skin

Starts inflammatory -oedema, tightening, thicken and waxy but then progresses to atrophy where the skin thins more collagen deposition and muscle decreases

What happens to the joints in systemic sclerosis

Skin may pull the joints into an abnormal position | Face hand and feet and then Arms legs and chest involvement

How does systemic sclerosis affect the face

Small tight mouth Vitiligo Hyperpigmentation Telangectasia

Systemic sclerosis affect on the gut

Hiatus hernia Poorly coordinated muscle activity Dysphagia Strictures - which inc risk of perf on endoscopy so test recommended is barium swallow

Pulmonary involvement systemic sclerosis

``` Intestitial fibrosis -breathless -dry cough Exclude infection Tx with immunosuppression - pulmonary hypertension ```

Cardiac affects of systemic sclerosis

CCF and arrhythmia

Nail involvement systemic sclerosis

Under nailfold capillaroscopy | See dilated capillaries which are tortuous and a dec vessel number

Renal involvement systemic sclerosis

Accelerated hypertension Renal crisis- blood vessel arterosclerosis Shrink the glomeruli

Ix systemic sclerosis

``` BP monitoring Renal function Pulm function ECG CXR Barium swallow ANA Anti-ENA Anti-centromere Anti-SC ```

Mx systemic sclerosis

Symp - raynauds - con and med Skin - moisturise Immunosuppression or anti-fibrotic Tx aimed at organ

What is myosotis

``` Chronic inflam of striated muscle There are 4 types -myositis - dermatomyositis - mixed CT - malignancy associated ```

What antibody associated with myositis

Anti-Jo1

Clinical features of myositis

Pain stiff and tender muscles with progressive weakness mainly in the proximal arms and legs Progressive facial muscle involvement - facial, bulbar, speech and language, resp - breathing Lung fibrosis And heart involvement

Skin involvement - myositis

Heliotrope rash around the eyes Rash on the skin of the chest Gettron papules on the knuckles MCP, PIP, DIP MAchnists hand -dry and cracked

Ix myositis

``` Blood- CPK, LDH, AST, ALT, ESR ANA Anti Jo1 ECG CXR MRI Biopsy of the muscle Ix - malignancy ```

What cancer are associated with dermatomyosits

Breast Lung Ovarian Dermatomyositis can result from these cancers presumably as an autoimmune response

Therapy myositis

Steroids - but not for long as they can accuse muscle weakness Immunosuppressants - methotrexate, azathioprine Severe and lung - cyclophosphamide Refractory - cyclosporine mycophenalate Rituximab IV Ig Monitor disease progress

Sjogrens what is it

Slowly progressive inflammatory disease affecting the exocrine glands - lacrimal - salivary Overlap SLE Lymphocyte infiltration Ab - Ro and La Mucosal dryness and eye dry, genital tract dry

Signs and test for Sjogrens

Schirmer tear test | Parotid swelling

Xtra glandular disease in Sjogrens

``` Polyarthritis Lung Kidney Liver BV ```

When in association with other Autoimmune disease sjogrens is And what other AI diseases are they like

RA, SLE, systemic sclerosis | 2ndary sjogrens