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Multisystem Flashcards

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1
Q

Natural Hx of SLE

A

Chronic inflammatory mulitsystem AI disease which releases and remits. Natural progression of the disease and its treatment leads to comorbidites.

Increased rate of atherosclerosis, malignancy and infections

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2
Q

Epidemiology of SLE

A

10:1 women:men, 20/100000
Increased incidence in afrocarribeans 16-40 y/o
HLADR2 and 3

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3
Q

Aetiology of SLE

A

Homozygous deficiencies of C1q, C2 or C4 confer a high risk but are very rare
24% risk in monozygotic twins
Seen in premenopausal women more

Viruses and UV can both trigger flares

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4
Q

Pathogenesis of SLE

A

Cell death is triggered via viral illness, UV light, smoking and occurs via apoptosis leads to DNA and histone presentation on the cell surface. These are interpreted as foreign antigens and phagocytosed . In SLE this process is inefficient leading to APC presenting the cell fragments to T cells which intern activate B cells to produce antinuclear antibodies. These form circulating complexes or deposit in tissues leading to complement activation and influx of neutrophils. This perpetuates the response

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5
Q

PC SLE - Bones and non specific

A

Most patients suffer non specific fatigue, fever, symmetrical small join arthralgia with pain but structurally normal joints +/- some swelling

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6
Q

Discoid lupus

A

Confined to the skin, rash appears on the face as a well defined erythematous plaque that progresses to a plaque and pigmentation

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7
Q

SLE and the skin

A

Photosensitive rash with a butterfly distribution over the bridge of the nose.
Vasculitic lesions on the finger tips, purpura and urticaria
Livedo reticularis
Scarring alopecia can = irreversible bald patches
Raynauds - 20%
Oral and mucosal ulcers

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8
Q

Jaccouds arthritis

A

Deforming non erosive arthropathy characterised by ulnar deviation of the second to 5th fingers with MCP subluxation.

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9
Q

SLICC Critera for SLE

A

Need > 4 criteria (1 clinical and 1 lab)
or
biopsy proven lupus nephritis with +ve ANA or antidsDNA stain

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10
Q

Lung and Heart SLE

A

Pleurisy and recurrent exudative pleural effusions. These can lead to restrictive lung disease

Pericarditis and pericardial effusions (25%). Increased risk of IHD and strokes due to HTN, hypercholestrolemia and chronic inflammation

Very rarely there may be myocarditis - arrhythmias, aortic valve lesions and a non infective endocarditis (Libman-Sachs syndrome)

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11
Q

Renal and SLE

A

Risk of lupus nephritis a RPGN which can lead to end stage renal failure. Screening with urine dipstick for proteinuria and haematuria is crucial

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12
Q

Nervous system and SLE

A

60% of cases may be mild depression but occasionally more severe psychotic features

Epilepsy, seizures, migraine, ataxia, aseptic meningitis etc

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13
Q

Raynaud’s disease

A

Peripheral digital schema often precipitated by cold or emotion.

PC = digit pain can be very severe
white to blue to red
In severe cases can = digital infarction and self amputation

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14
Q

Aetiology of raynauds

A

1 - Raynauds phenomenen

2 - SLE, RA, Sjogrens, systemic sclerosis, polymyositis
Obstructive causes - Buegers, takayasu’s atherosclerosis
B-blockers, occupational drugs involving vibration

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15
Q

Mx Raynauds

A

Look for a diagnosis! Gloves stop outdoor work, CCB’s - DHP i.e. nifedipine or ACEi

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16
Q

O/E SLE

A

Hands - raynauds, thickening of the flexor tendons
Lymphadenopathy
Diffuse scarring alopecia, discoid or photosensitive rash
Oral or mucosal ulcers
BP and urine dip to check for lupus nephritis

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17
Q

Investigations SLE

A

High ESR,
Leucopenia, lymphopenia +/- thrombocytopenia
Normocytic anaemia of chronic disease or AIHA
Low C3 and C4 complement in acute disease

Biopsy = deposition of IgG and complement

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18
Q

Autoantibodies in SLE

A

95% ANA +ve but poor sensitivity seen in old age and increased in many other AI conditions

anti-dsDNA high specificity - homologous pattern of staining

Anti smith - increase prevalence in black african populations

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19
Q

SLE and Sjogrens

A

Anti Ro70 and Ro52 showing a speckled ANA pattern and Anti Ui RNP showing nuclear ANA pattern are linked to an increased risk of Sjogrens and high risk of Ab crossing the placenta and causing congenital heart block and rash in foetus

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20
Q

Drug induced SLE

A

Sulphasalazine, procaimide, antieplieptics all anti-histone +ve

Anti-TNF = anti dsDNA +ve

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21
Q

Mx of SLE

A

Immunosuppression - hyrdroxychoroquine and NSAIDs

IV steroids for acute flares (prednisolone and cyclophosphamide)

Monitor anti ds-DNA and C3/C4 levels - sensitive for relapse

Check urine for evidence of lupus nephritis

Reduce risk of IHD and osteoporosis

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22
Q

Lofgrens syndrome

A

Subtype of sarcoidosis. Arthralgia, bilateral hilarlympadenopathy and erythema nodosum

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23
Q

Antiphospholipid syndrome PC

A

Thrombosis
Recurrent miscarriage
Livedo reticularis
Thrombocytopenia, high APT, +ve Coombs test

20% of pt suffer ischemic strokes, 40% DVTs. 27% of women who have 2+ spontaneous miscarriage = anti phospholipid syndrome

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24
Q

SLE and preganacys

A

Hydroxycholoquine and azathioprine, low dose steriods are safe. Beware those with anti-Ro and La. 2% risk of fatal heart block

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25
Aetiology of antiphospholipid
``` 70% = idiopathic 30% = SLE ```
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Autoantibodies in APS
Anti-cardiolipin - Detects antibodies that bind to the negatively charged phospholipid cardiolipin Lupus anticoagulant - Caused by anti phospholipid antibodies causes increased APTT (opposite effect to within the body) Anti B2 glycoprotein
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Mx APS
Aspirin If recurrent thrombosis = anticoagulation with warfarin INR 3.5
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Vasculitis
Inflammation of the blood vessels often characterised by multi-system involvement, fatigue, wt loss. Classified by size of vessels effected and the presence of ANCA
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Large cell vasculitis
GCA and takayasu's
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Giant cell arteritis
Inflammatory granulomatous arteritis of the large cerebral arteries. Seen above the age of 50, 2:1 female:male, highest incidence in northern europe.
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PC GCA
Often debilitating bitemporal headache - not improved by analgesia, lasts the whole day Ocular symptoms Scalp tenderness, neck ache, jaw claudication Systemically - wt loss, fever, limb claudication due to subclavian stenosis, abdo pain SMA stenosis, HTN
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Ocular symptoms in GCA
Amaurosis fungax - temporary blindness , reduced visual acuity, RAPD, reduced colour vision and visual field defects O/E - diffuse pale optic disc swelling, cotton wool spots - nerve ischemia 60% due to arctentic anterior ischemic optic neuropathy this occludes the posterior ciliary vessels leading to infarction of the optic nerve head
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Complications of GCA
Increased risk of thoracic aneurysms Stenosis of cerebral and subclavian arteries Ischemic consequences of coronary and mesenteric arteries
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Investigations into GCA
ESR >50 Normocytic anaemia, thrombocytopenia Deranged LFTs - high ALP USS doppler - Black halo sign shows vessel ischemia, , loss of radio-echo signal = infiltrate and oedema
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Biopsy in GCA
From affected side. Inflammatory infiltrate from T cell, B lymphocytes and giant cell. Can be -ve biopsy due to skip lesions
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Central retinal artery occlusion
Rapid visual loss, reducing acuity and RAPD. | O/E = cherry red spot @ fovea due to alternate blood supply
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Diagnostic criteria of GCA
Age >50, new headache, temporal artery abnormality, ESR >60, +ve biopsy
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Mx GCA no visual symptoms
60mg prednisolone
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Mx GCA + visual symptoms
3 days methyprednisolone 0.5-1g stat
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Steroids in GCA
Reduced the dose by 5mg every 2weeks, as the dose gets lower the dosing interval should shrink to 1mg SE = wt gain, infection, cataracts, skin fragility and purpura, mood changes, proximal weakness, Cushings, osteoporosis
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Polymyalgia rheumatica PC
Sudden onset severe pain and stiffness in the muscles of the shoulders and the neck. Often symmetrical crucially with no muscle weakness. Worse in morning lasting from 30 mins to several hours. 1/3rd of its have wt loss, fever, lethargy and depression
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Invx in to PMR
high ESR, ALP normal CK possibly a normocytic anaemia
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Diagnostic criteria of PMR
``` Morning stiffness > 45mins = 2 Rh or anti-CCP -ve = 2 Pelvic girdle pain = 1 No other painful joints = 1 USS changes @ shoulders = 1 ```
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Takayau's arteritis PC
Isolated aortitis with granulomatous nodules. Young japanese females Pre vasculitis stage of fever, fatigue, wt loss PC - vascular bruits @ carotids and abdo vessels renal artery stenosis = HTN diminished or absent pulses in arms due to complete stenosis upper limb claudication with BP difference >20mmHg symptoms of visual loss and dizziness if involving the carotids coronary artery involvement = HF and aortic root dilation
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Mx Takayasu's
CT PET to show areas of inflammation | Immunsupression with methotrexate
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Medium vessel vasculitis
Polyarteritis nodosa
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Polyarteritis nodosa (PAN)
Seen in middle aged men, no ANCA association; 30% of cases are linked to hep B
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PC PAN
Constitutional symptoms of fever, malaise, wt loss and myalgia Acute features Neuro - mononeuritis multiplex due to arteritis of the vasa nervorum Skin - subcutaneous haemorrghe and gangrene. In chronic cases persistent livedo reticularis Cardiac - can = MI, pericarditis and HF Abdo - most common 1/3rd can mimic pancreatitis, appendicitis. GI haemorrhage due to mucosal ulceration
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Invx and Mx PAN
Anaemia, leucocytosis and a raised ESR Mx = corticosteriods
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Kawasaki's PC
Acute systemic vasculitis. fever lasting >5 days, bilateral conjunctival congestion, dryness and redness of the lips and oral cavity cervical lymphadenopathy Polymorphic rash Redness and oedema of the palms and soles 2-5 days after onset Mx = single stat dose of immunoglobulin 2g/kg
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Small vessel vasculitis
ANCA associated = GPA, eGPA, MPA Immune complex associated (ANCA -ve) = SLE, anti-GBM, HSP, cryoglobulinemia
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Pulmonary renal syndromes
SLE, Goodpastures, GPA, MPA
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Vasculitic signs and symptoms
Non specific - wt loss, fever, night sweats Skin - purpuric rash - painful and non-blanching digital infarction - terminal capillary livedo reticularis oral and nasal ulcers and polyps ENT - Epistaxis, crusting, sinusitis, hoarse voice, can get a saddle shaped nose deformity due to loss of nasal cartilage Eyes - Episcleritis - red eye ball, pain and photophobia, tis is also seen in RA, IBD and systemic vasculitis. Can lead to scleromalacia where the pigment of the underlying choroid can be seen MSK - arthralgia, myalgia and muscle wasting Abdo - Iron deficiency anaemia from GI blood loss, abdo pain, diarrhoea and intestinal obstruction Cardio and resp - Pain, SOB, haemoptysis - pul haemorrhage Neuro - transverse myelitis, peripheral neuropathy, mononeuritis multiplex
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Mono-neuritis multiplex
Simultaneous involvement of individual nerve trunks evolving over days to years. Acute/subacute sensory and motor loss of individual neurones Causes = DM, vasculitis, SLE, sarcoid
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Granulomatosis with polyangitis (GPA) PC
Upper airway disease - recurrent sinusitis and epistaxis, can lead to destruction of the nasal cartilage = saddle shaped nose deformity. Bronchial/tracheal stenosis Pulmonary parenchymal disease - granuloma formation leads to cough, SOB and pleuritic chest pain - can lead to pulmonary haemorrhage - CXR multiple nodules, consolidation and ground glass Renal - RPGN Eyes - scleritis and conjunctivitis
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Invx GPA
PR3 +ve in 90%, biopsy - granulomatosis inflammation in vessel walls,
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Microscopic polyangitis (MPA) PC
Never URTI involvement. Usually presents with RPGN and skin involvement - maculopapular purpura. Has a higher incidence of neurological involvement.= Low incidence of pulmonary parenchymal disease but high risk of pulmonary haemorrhage.
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Inv MPA
Mixed picture MPO (p-anca) +ve in 90%, can have +ve c-anca PR3 in 20% of cases Crucially on biopsy = no granulomas
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Eosinophilic granulomatosis with polyangitis PC
Early adulthood onset allergic rhinitis and asthma like symptoms. Features of a systemic vasculitis often appear later. Skin - tender subcutaneous nodules, petichae and purpura Neuro (80%) - mononeuritis multiplex Very rare = alveolar haemorrhage.
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EGPA Inv
MPO, panca +ve | Biopsy = eosinophils, no granulomas
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Mx of all small vessel vasculitis
Induced remission with high dose of steroids | Maintenance with methotrexate and azithprione
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Henoch-Scholein purpura PC
Most common childhood vasculitis 3-8y/o post URTI Triad of colicky abdo pain, palpable purpuric rash over buttocks and thighs. Haematuria and proteinuria leading to nephrotic syndrome +/- arthralgia, GI haemorrhage or intusseception
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HSP Inv and Mx
High IgA, CRP, high platelets - not ITP May need steroids, most return to baseline with no renal impairment. Much higher incidence of ESRF in adults
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Cryoglobulinemia
Simple -  Monoclonal IgM due to myeloma / CLL / Waldenstroms PC = Hyperviscosity Visual disturbance Bleeding from mucus mems  Thrombosis Headache, seizures Mixed (80%)  Polyclonal IgM secondary to SLE, Sjog, HCV, Mycoplasma  PC = Immune complex disease GN Palpable purpura Arthralgia Peripheral neuropathy
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SLE auto antibodies
Anti-dsDNA = specific for SLE and TNFa induced SLE Anti histone = drug induced - isoanzid, methotrexate, hydralazine, procainmide Both show homogenous patterns of staining Anti-smith = speckled staining
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Sjogrens auto antibodies
Anti Ro and anti La speckled pattern
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Systemic sclerosis
Diffuse - Scl70 antitopisomerase Limited - anti-centromere
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PC Raynauds
Classically white - blue - red. Worse at times of stress and cold. In severe can lead to digital ulceration and gangrene
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Inv Raynauds
Rule out a secondary cause. Nail fold cappilaroscopy
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Causes of Raynauds
1 - idiopathic 2 - B blockers, systemic sclerosis, SLE, RA, Sjorgens, Vasculitis, polycythemia
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Pathogenesis of systemic sclerosis
Widespread vascular damage involving small arteries, arterioles and capillaries. There is initial endothelial cell damage this perpetuates with cytokine involvement leading to fibroblast activation. Increased levels of type I and III collagen in lower layer of dermis and internal organs
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Limited systemic sclerosis PC
Calcinosis - fingers Raynauds - 100% often begins 15yrs before skin changes Eosophegal dysmotility Sclerodactyl - shortened fat fingers + microstomia Telengectasia (facial blanching on pressure) Crucially skin involvement is limited to the hands, face, feet and forearms. No chest or neck involvement 15% cases = pul HTN
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Investigations limited systemic sclerosis
Anti-centromere Abx Dilated nail fold capillary loops Barium swallow shows duck neck.
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Diffuse systemic sclerosis PC
Usually begins with oedema and Raynauds and progresses rapidly. More severe involving multiple organs systems. Skin involvement above the elbows on the neck and trunk Lung = 40% develop ILD - restrictive lung disease with basal reticular nodular shadowing on X-ray 20 % also develop pulmonary HTN Cardiac = rare involvement - conduction defects GI = Very common to have oesophageal dysmotility - dysphagia, reflux, heartburn, occasionally anal incontinence. Atony of small bowel, GAVE, malabsorption due to bacterial overgrowth. Renal - acute hypertensive crisis
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Inv for diffuse systemic sclerosis
Antitopisomerase - scl70 RNA polymerase III - increased risk of renal involvement Enlarged capillary loops with significant loss
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Systemic sclerosis renal crisis
PC malignant HTN, high renin, microangiopathic haemolytic anaemia. Protein and haematuria Affects 10% with dSS. Can be precipitated by steriods Mx = aggressive ACEi. Prognosis linked to level of skin involvement, RNA III polymerase levels and tendon friction rub
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MAHA (microangiopathic haemolytic anaemia)
red cells are ripped apart by physical trauma. Often in the small vessels. SLE and SS are also includes. Crucially schisocytes on blood film 1. Disseminated intravascular coagulation (DIC) – a nasty condition in which there is bleeding and clotting at the same time in the patient. Lots of things can cause DIC (like malignancy, obstetric complications, trauma, and sepsis) – and it’s complicated enough that we’ll get into it in a future post. 2. Thrombotic thrombocytopenic purpura (TTP) – a syndrome in which the patient gets little thrombi within the microvasculature anywhere in the body, but especially the CNS and kidneys. We’ve talked a little about TTP before. 3. Hemolytic-uremic syndrome (HUS) – a disorder often related to ingestion of food (especially raw hamburger, but also spinach, other vegetables, you name it) containing E. coli 0157:H7. The bug makes a toxin that damages endothelial cells, and for some reason, the kidneys are hit the hardest.
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GI complications of SS
GORD - Barretts oesophegus (squamous - glandular) Duodenum - dilation can lead to atony GAVE - gastric central vascular ectasia 25% of pt with high RNA polyermase III. Dilated submucosal vessels on antrum may precipitate GI bleed.
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Investigations in systemic sclerosis
FBC - normocytic anaemia Urine dip - proteinuria, A:CR Serial PFT's, echo and BNP to monitor Barium swallow may show beak shaped deformity ILD usually manifests with reticulonodular shadowing @ the bases. However can develop apical if due to recurrent aspiration. HRCT to diagnose
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Mx of SS
Avoid the cold, wear gloves Rarely any management options for oesophageal problems - PPI if symptomatic Malabsorption - Abx for bacterial overgrowth, nutritional substitutes Monitor for renal involvement - ACEi stat! Pulmonary fibrosis = aggressive steroid therapy IV cyclophosphamide and azathioprine
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Localised scleroderma
Morphea - localised subcutaneous confined to the skin in young adults and children.Seen in bluish patches on the trunk can progress to central white atrophy.
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Sjogrens syndrome PC
SICCA symptoms = dryness of eyes, mouth, vaginal, larynx. These can = nutritional defects, tooth decay, dyspareunia, salivary gland enlargement and chronic eye infections - dry gritty eyes Fatigue
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Systemic signs of Sjogrens
Arthralgia - non progressive polyarthritis Raynauds TIN leading to renal tubular acidosis 20x increase of MALT - non hodgkin B cell lymphoma Rare - oesophageal dysmotility, pulmonary fibrosis
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Primary Sjogrens
9:1 Female:males Onset 40-50y/o Absence of RA, SLE,
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Secondary Sjogrens
Linked to HLA DR3 - AIH, PBC, RA, SLE, SS, MCTD, thyroid disease
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Investigations into Sjogrens
100 % for ANA and RF - Specific = antiRo and La High ESR, low C3, hypergabbaglobulinemia Labial and parotid biopsy shows focal infiltration of B cells and lymphocytes. Crucial to palpate for lymphadenopathy
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Schirmer test
Tests for tear production strip of filter paper placed on inside of lower eyelid for 5 minutes ``` >15mm = normal <5mm = Sjogrens ```
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Mx Sjogrens
Artifical tears and saliva replacements NSAIDS and hydroxchloroquine for arthralgia Immunosuppres for systemic disease Check for lymphoma!!!
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Myalgia vs myositis
Myalgia - common feature of many AI, no weakness normal muscle enzymes Myositis - Inflammatory myopathies characterised by proximal > distal weakness often symmetrical in pt 50+
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Polymyositis
Idiopathic inflammation of striated muscle, 3x more common in female
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PC polymyositis
Progressive symmetrical proximal weakness often affecting the shoulders and the pelvic girdle. Spares distal and ocular muscles. Systemic features of wt loss, fever, anorexia Can lead to dysphonia, dysphagia and involvement of laryngeal muscles
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Invx of polymyositis
Biopsy - cytotoxic CD8 T cells in endomysium. High CK and LDH (1000s in active disease) Crucial to take trop T for baseline Anti-Jo
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Livedo reticularis
Anti phospholipid, RA, TB, SLE, RCC, dermatomyositis Polyarteritis nodosa, cryoglobulinemia, myeloma
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Bechets
Oral and genital ulceration, anterior and posterior uveitis, arthralgia
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Dermatomyositis
Polymyositis + skin involvement - Heliotrope rash on eyelids +/- oedema - Gottrans papules lichenoid changes over knuckles - Macular rash (Shawls sign) photosensitive over back and shoulders - Mechanics hands painful rough cracked skin without vesicles Always hunt for Cancer approx 40% linked to.
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Inv dermatomyositis
High CK, LDH, ALT Raised ESR and CRP Anti Mi 2 EMG = triad of spontaneous fibrillation @ rest, polyphasic potentials on voluntary contraction, repetitive potentials on mechanical stimulation Biopsy - CD4 infiltrate, lowest possible steroid dose, screen for malignancy
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Cancer linked to dermatomyositis
Ovary, lung, Non-hodgkin lymphoma, pancreatic and stomach
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Complication of inflammatory myopathies
High incidence of ILD and oesophageal dysmotility
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Anti Jo +ve (anti trna synthase)
Mechanics hands are highly suggestive, increased risk of ILD and raynauds
100
Anti Mi
Better prognosis. low incidence of ILD, more cutaneous signs
101
MCTD
PC = ILD, Raynauds, skin and muscle involvement, fatigue and arthritis Anti U1 RNP
102
Antiphospholipid syndrome diagnosis
1 x clinical = vascular thrombosis (FHx), pregnancy >3 unexplained miscarriages >1 late abortion 1 x lab = Lupus anticoagulant (2 x 12 weeks apart) anticardiolipin / anti B2 glycoprotein
103
DMARDs in pregnancy
Safe = azathioprine and hydroxychlorquine Stop known teratogens i.e. MMF, methotrexate and cyclophosphamide. Rituxamib linked to 2nd/3rd trimester B cell depletion.
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Pre-pregnancy counselling
Achieve disease quiscense 6 months prior to conception. Measure C3, dsDNA, BP and urine @ baseline Maternal Ro/La = 2% risk of fetal HB at birth, Low dose aspirin for all with SLE, Manage flares with lowest dose possible methylpred. Pregnancy makes rheumatic conditions much worse! High dose folic acid. Consultant led care
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