MSI- AI disease seminar Flashcards by Heather Lilly

A group of diseases that share certain major clinical features.

Key ones are chronic low back pain, positivity with the blood test for the human leukocyte antigen (HLA) B27, and radiographic or MRI changes in the sacroiliac (SI) joints in patients with axial SpA; and (oligo)arthritis, heel enthesitis and dactylitis in patients with peripheral SpA

Spondyloarthritis (SpA)

How well did you know this?

Not at all

Perfectly

Examples of spondyloarthritis disorders:

Ankylosing spondylitis, juvenile ankylosing spondylitis, ________ arthropathy, intestinal arthropathy (CD & UC), and sacroilitis

psoriatic

How well did you know this?

Not at all

Perfectly

** CLINICAL CHARACTERISTICS OF SPONDYLOARTHROPATHIES **

Pattern of peripheral arthritis: predominantly lower limb, asymmetric

Enthesitis

Tendency to radiographic sacroiliitis

Absence of rheumatoid factor / anti CCP

Extra-articular features characteristic of the group (such as anterior uveitis)

Significant familial aggregation

Association with HLA-___

B27

How well did you know this?

Not at all

Perfectly

The criteria for INFLAMMATORY back pain are met if at least four of the following five features are present (and IBP is described as “suggested” in the presence of three of five features):

Onset of back discomfort before the age of 40 years

Insidious onset

Improvement with _________

No improvement with rest

Pain at night (with improvement upon arising)

exercise

How well did you know this?

Not at all

Perfectly

** Other associated features - Axial SpA:
Alternating buttock pain
Heel pain caused by enthesitis
__________ is diffuse swelling of digits of toes or fingers, also termed “sausage digits”
Asymmetric arthritis predominantly of the lower limbs
Anterior uveitis (iritis).
Crohn disease or ulcerative colitis.
Psoriasis– Past or present psoriasis.
Good response of pain symptoms to NSAIDs
Family history of SpA– A positive family history is defined as the presence in a first- or second-degree relative of a diagnosis of SpA, uveitis, reactive arthritis, psoriasis, or inflammatory bowel disease.

Dactylitis

How well did you know this?

Not at all

Perfectly

How often should a spinal examination be performed in SpA conditions to monitor progress/treatment?

Annually

How well did you know this?

Not at all

Perfectly

Investigations in SpA

Routine- FBC, Renal, LFTs, Calcium (esp if on meds)
**___-___ (more common in Caucasians than Black ethnic groups- less than 1%)
Acute phase reactants
Imaging
SI joint X-rays
MRI Whole spine and SI joints if x-ray doesn’t show changes- ASAS protocol (Without NSAID use)

HLA B27

How well did you know this?

Not at all

Perfectly

What type of changes might you see on Xray of a patient with AS?

Fused iliosacral joints- Inflammation leads to fusion of the bone, which progresses until there is hardly any gap there

How well did you know this?

Not at all

Perfectly

Average age of onset for axial SpA/AS?

26 years

70% of patients affected with this condition are 20-49 years old

How well did you know this?

Not at all

Perfectly

Treatment for SpA:

Physiotherapy & exercise
Pain relief: simple analgesics & Anti-inflammatory (NSAIDs)

________ (if peripheral joints affected)
Steroids-oral/intra-muscular/intra-venous

If Spinal disease-Anti-TNF/IL-17 inhibitors agents
Surgery e.g. hip replacement
DEXA-Osteoporosis prophylaxis

DMARDs

How well did you know this?

Not at all

Perfectly

An arthritis which develops soon after (1-4 weeks) or during an infection elsewhere in the body, but in which the microorganisms cannot be recovered from the joint.

reactive arthritis

How well did you know this?

Not at all

Perfectly

Common organisms that can cause reactive arthritis:
_________ / Gonorrhoea
Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile
Flu

Chlamydia

How well did you know this?

Not at all

Perfectly

What is the “reactive arthritis triad?”

Conjunctivitis: discharge, erythema, burning, photophobia
Urethritis: dysuria, urgency, frequency, discharge
Arthritis: Knees, ankles, feet

How well did you know this?

Not at all

Perfectly

Nail changes (such as nail pitting) have the strongest association with which type of arthritis?

psoriatic

How well did you know this?

Not at all

Perfectly

Features of psoriatic arthritis:

Arthritis may precede the onset of psoriasis / can also be first degree relative with psoriasis

Prevalence 0.04%-0.1%

Sex distribution is equal

** Nail changes have the strongest association with arthritis

Spondyloarthropathy seen in about 5%

__________ joint involvement can be symmetric or asymmetric

Sacroiliac

How well did you know this?

Not at all

Perfectly

Psoriatic arthritis can have different presentations/affected joint patterns in the hands.

Categories of Moll & Wright classification

● Arthritis with DIP joint involvement predominant

● Arthritis mutilans

● Symmetric polyarthritis –
indistinguishable from RA (what should be tested when you see this presentation?)

● Asymmetric oligoarticular arthritis

● Predominant spondylitis

anti-CCP

How well did you know this?

Not at all

Perfectly

Psoriatic arthritis can have different presentations/affected joint patterns in the hands.

Categories of Moll & Wright classification

● Arthritis with DIP joint involvement predominant

● Arthritis mutilans (bone destruction, very rare)

● Symmetric polyarthritis –
indistinguishable from RA (what should be tested when you see this presentation?)

● Asymmetric oligoarticular arthritis

● Predominant spondylitis

anti-CCP

How well did you know this?

Not at all

Perfectly

Tx for psoriatic arthritis

NSAIDs- bridging tx until DMARDs or biologics start working

DMARDs- _________, sulphasalazine, Leflunomide

Biologics- Anti- TNF, IL-17 inhibitors

JAK inhibitors (not a core drug)

Methotrexate

How well did you know this?

Not at all

Perfectly

Seronegative Spondyloarthropathy Summary:

Oligoarticular
Asymmetrical	
Chronic inflammatory
Sacroiliac involvement
\_\_\_\_\_\_\_\_
Uveitis
Spinal involvement
HLA B27 (and CCP/RF -ve)
Dactylitis

Enthesitis

How well did you know this?

Not at all

Perfectly

HLA B27 association in white vs black ethnicity?

8% whites, 1% blacks

How well did you know this?

Not at all

Perfectly

Takayasu’s and giant cell arteritis are vasculitis conditions affecting _________ vessels

Large

How well did you know this?

Not at all

Perfectly

Henoch Schonlein purpura, EGPA and GPA, MPA are vasculitis conditions affecting ________ vessels.

(Eosimophilic Granulomatosis with Polyangiitis, Granulomatosis with Polyangiitis, Microscopic Polyangiitis)

Small

How well did you know this?

Not at all

Perfectly

Polyarteritis nodosa, and Kawasaki’s are vasculitis conditions affecting __________ vessels

Medium

How well did you know this?

Not at all

Perfectly

Wegener’s granulomatosis, Churg-Strauss vasculitis, microscopic polyangitis, and drug-induced vasculitis are classified as

ANCA-associated

How well did you know this?

Not at all

Perfectly

GIANT CELL ARTERITIS ``` ** Age > 50 (Peak age ___-___) ** Caucasian Scalp tenderness and headache (temporal and unilateral) Jaw or limb claudication ** Blindness or diplopia Weight loss, anorexia, fever, night sweats, malaise ** Raised ESR and CRP Biopsy or USS ```

70-79

Examination features in GCA ** Abnormal Superficial Temporal Artery Tender Thickened _____/_______ Pulsation Scalp Tenderness Cranial Nerve Palsies Vascular Bruits Asymmetric Pulses

Reduced/ Absent

Visual loss in GCA: ``` Field Defect Afferent Pupillary Defect Anterior Ischaemic Optic Neuritis Central Retinal Artery Occlusion Diplopia Early Ophthalmology Review ``` ** HOW SOON CAN VISION LOSS OCCUR WITHIN ONSET OF SX OF GCA?

Within ONE day

GCA investigations: FBC, U&Es, LFTs, CRP, ESR. CXR (can show dilation of the aorta in large vessel vasculitis) Urinalysis ** ________ artery biopsy- often confirms dx, but not always as it can occur in skip lesions USS Temporal Arteries (USS limited as it requires a high level of experience and training)

temporal

Next steps when a dx of GCA is strongly suspected?

High dose steroids, straight away! and temporal artery biopsy

Treatment of GCA ** __________ – immediately (tapering regimen) Uncomplicated GCA (no jaw claudication or visual disturbance) 40mg prednisolone daily. Evolving visual loss or amaurosis fugax (complicated GCA): 500 mg to 1 g of i.v. methylprednisolone for 3 days before oral glucocorticosteroids. Established visual loss 60 mg prednisolone daily to protect the contralateral eye.

STEROIDS

A systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries.

Polyarteritis nodosa (PAN)

Clinical manifestations of _____ include | Systemic sx such as fever, malaise, weight loss. Also neuropathy, arthralgias/myalgias, cutaneous sx and renal or GI sx

systemic polyarteritis nodosa (PAN)

_____ is a necrotizing vasculitis that primarily affects capillaries, venules, or arterioles, most commonly manifesting as necrotizing glomerulonephritis and/or pulmonary capillaritis. Involvement of medium- and small- sized arteries may also be present. Granulomatous inflammation is usually absent. ** ANCA is present in >90 percent of patients with MPA.

Microscopic polyangitis (MPA)

__________ cytoplasmic autoantibodies (ANCA) are associated with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss), "renal-limited" vasculitis, and certain drug-induced vasculitis syndromes. In these conditions, ANCA consistently have specificities for either proteinase 3 (PR3) or myeloperoxidase (MPO) but almost never for both. (If someone presents with sx of vasculitis, you would always order an ANCA, particularly with a lot of systemic sx)

Antineutrophil

Granulomatosis with polyangiitis (previously known as __________) GPA is a necrotizing vasculitis predominantly involving small- to medium-sized vessels (eg, capillaries, venules, arterioles, arteries, and veins). It typically produces granulomatous inflammation of the upper and lower respiratory tracts (* COMMONLY AFFECTS THE CHEST) as well as necrotizing, pauci-immune glomerulonephritis. ANCA is present in >80 percent of patients with GPA.

Wegener's

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) EGPA is an eosinophilic-rich necrotizing vasculitis predominantly affecting small- to medium-sized vessels. Patients often have chronic rhinosinusitis, ________, and prominent peripheral blood eosinophilia. ANCA is present in approximately 40 percent of patients with EGPA, usually anti-MPO ANCA. The presence of ANCA is more frequent in patients with glomerulonephritis.

ASTHMA

Anti-glomerular basement membrane disease: Anti-glomerular basement membrane (GBM) disease is a vasculitis affecting __________ capillaries, pulmonary capillaries, or both, with basement membrane deposition of anti-basement membrane autoantibodies. Lung involvement typically causes pulmonary hemorrhage, and renal involvement causes glomerulonephritis with necrosis and crescents.

glomerular

Cryoglobulinemic vasculitis Cryoglobulinemic vasculitis, previously termed 'essential cryoglobulinemic vasculitis', is characterized by the presence of cryoglobulins, which are serum proteins that precipitate in the cold and dissolve upon rewarming. In this disorder, which is most often due to _______ __ virus infection, cryoglobulin immune complexes are deposited in the walls of capillaries, venules, or arterioles, thereby resulting in inflammation in small vessels. Skin, glomeruli, and peripheral nerves are often involved.

hepatitis C

(Henoch-Schönlein purpura) Immunoglobulin __ vasculitis is a systemic vasculitis characterized by the tissue deposition of ___1-dominant immune complexes affecting mostly small vessels (predominantly capillaries, venules, or arterioles). ___ vasculitis typically affects the skin and gastrointestinal tract, and often causes arthritis. A glomerulonephritis indistinguishable from ___ nephropathy may be observed.

IgA

Hypocomplementemic urticarial vasculitis (anti-___ vasculitis) Hypocomplementemic urticarial vasculitis (HUV) is a vasculitis associated with urticaria and hypocomplementemia, and predominantly affects small vessels. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation may also be observed. The presence of anti-___ antibodies is one of the most distinctive findings in HUV

C1q

Systemic lupus erythematosus (SLE) Chronic, relapsing, remitting disease Broad spectrum of clinical features involving almost all organs and tissues- _____ _______ DISEASE Prevalence in the UK: 97 per 100,000 F:M= 10-20:1 Peak onset between 15- 40 years More common and severe in those of Afro-Caribbean, India, Hispanic and Chinese origin living in USA and Europe> Caucasians

CONNECTIVE TISSUE

Two of the most common clinical features of Lupus?

Malar rash and Raynaud's phenomenon

Features of SLE ``` ** Malar rash Discoid rash Serositis – Pleuritis / Pericarditis Oral ulcers ** Arthritis – non-erosive Photosensitivity Blood disorders Renal disorders ** ANA + anti _____ / dsDNA / antiphospholipid abs Neurologic symptoms ```

Smith

SLE investigations: Urinalysis (urinary protein:creatinine ratio) FBC Urea & electrolytes ** ESR- ELEVATED ** CRP- __________ LFT's ** Abs: ANA; ENA, Anti0dsDNA, Lupus anticoagulant; Anti C1q C3, C4 (complement proteins can drop in active lupus)

NORMAL

Non-pharm management of SLE | sun protection; smoking cessation; CVD risk modification; __________ prevention

osteoporosis

Sjogren’s syndrome Lymphocytic infiltration of _________ glands ** Sicca: Dry eyes (lacrimal gland involvement) ** and xerostomia (salivary gland involvement) Extra-glandular disease: joint pain, skin disease and pulmonary disease Fatigue, myalgia Women more affected than men

exocrine

Test for measuring lacrimal output in suspected Sjogren's?

Schirmer’s Test

Complications of Sjogren's syndrome: Non-Hodgkins __________ Chronic dry eyes (leading to corneal and conjunctival damage) Chronic dry mouth (leading to dental caries) Congenital heart block

Lymphoma

Two key sx for suspecting Sjogren's syndrome?

Dry eyes and dry mouth x 3 months

Sjogren's syndrome dx: (need 4/6 criteria, incl ONE of the bottom three ``` Dry eyes > 3 months Dry mouth > 3 months Shirmer's test <5mm/5min Abnl salivary flow test **focal lymphocytic sialoadenditis on lip **biopsy **Anti-__/_______ positive ```

Anti-Ro/Anti-La

Systemic sclerosis (scleroderma) Immune activation, vascular damage and excessive deposition of collagen More frequent in ages 30-50 5:1 female Antibodies: anti topoisomerase (anti-______) Anti centromere (ACA) anti-RNA polymerase III ANA

Scl-70

Systemic Sclerosis Clinical Features: ``` Sclerodactyly Raynaud’s phenomenon Heartburn ** Abnormal nailfold capillaries ** ___________ Pulmonary arterial hypertension Interstitial lung disease Lower bowel: distension / diarrhea / constipation Renal failure / proteinuria Erectile dysfunction ```

Telangiectasia

Types of systemic sclerosis: Types: Limited = _______ (calcinosis, Raynaud’s, Esophageal dysmotility, sclerodactyly, telangiectasias) Diffuse= scleroderma proximal to elbows Overlap syndromes with SLE / Sjogren’s Sine scleroderma

CREST

Myositis: Muscle inflammation The idiopathic inflammatory myopathies (IIMs) include __________ (DM), polymyositis (PM), myositis as part of a rheumatic disease overlap syndrome, myositis of the antisynthetase syndrome, immune-mediated necrotizing myopathy (IMNM) and inclusion body myositis (IBM).

dermatomyositis

Sx of myositis: Patients presenting with evidence of muscle weakness. Patients usually report a history of the _________ or subacute development of the muscle weakness, with gradual worsening over a period of several months before medical attention is sought. Patients may describe increasing difficulty climbing stairs, getting up from a chair, carrying heavy groceries, or picking up their children due to the proximal muscle involvement.

insidious

Patients with classic ____________ (DM) typically present with symmetric muscle weakness, elevated muscle enzymes, and characteristic cutaneous findings. Antinuclear antibodies (ANAs) may be present, and some patients have DM-specific autoantibodies, such as anti-Mi-2, among others. Notably, myositis-specific antibodies are present in only 20 to 40 percent of patients with DM, and hence are not required to make a diagnosis of DM. Increased risk of malignancy.

dermatomyositis

``` Skin changes associated with dermatomyositis (DM) include pathognomonic findings such as ________ papules (pink-violaceous papules overlying interphalangeal and metacarpophalangeal joints), _________ sign (macular, pink-violaceous erythema overlying other joints, such as the elbows or knees) and the heliotrope eruption (pink-violaceous erythema, with or without edema, involving the periorbital skin) Pink-violaceous erythema of the scalp, V of the neck, shoulders, extensor surfaces of the upper extremities, upper chest, and upper back are additional characteristic findings. ```

Gottron's

Violaceous erythema on the periorbital skin/ heliotrope eruption is a sign of

dermatomyosititis

Patients presenting with weakness and elevated muscle enzymes in the absence of characteristic cutaneous findings of DM present a more difficult diagnostic challenge as there are no specific cutaneous findings of other IIMs and diagnostic serologic tests are less likely to be present.

polymyositis

This type of myosititis is similar to dermatomyositis, but without the skin changes. Not associated with malignancy

polymyositis

__________ syndrome group is defined by the presence of an antibody directed against one of several aminoacyl-transfer RNA (tRNA) synthetases. Clinical findings include inflammatory myopathy and extramuscular findings including interstitial lung disease, **"mechanic's hands”**, inflammatory arthritis, and Raynaud phenomenon. Onset is often more acute than in primary DM. There does not appear to be an increased risk of malignancy in this group.

Antisynthetase syndrome

Inflammatory myopathy may occur in the setting of another systemic rheumatic disease such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc; scleroderma), mixed connective tissue disease (MCTD), and, less often, rheumatoid arthritis (RA) and Sjögren's syndrome (SS). Thiis is known as

overlap syndromes

Inclusion body myositis: Although classified with the IIMs, IBM has epidemiologic, clinical, and histopathologic findings that distinguish it from the other IIMs. IBM affects older individuals, more commonly men. It generally has a more insidious onset and slower progression than the other inflammatory myopathies. The distribution of weakness tends to be more ______ in the upper extremities and less symmetric.

distal Other notes: Non inflammtaory Weakenss more distal than prox Differentiate with a biopsy

Investigations of myositis Most patients with IIM will have an elevation of one or more of the muscle-derived enzymes, creatine kinase, lactate dehydrogenase (LDH), aldolase, aspartate aminotransferase (AST), and alanine aminotransferase (ALT). _______ _________ is the most sensitive muscle enzyme and is the one most commonly tested for and followed.

Creatine kinase

What antibody is commonly seen in anti-synthatase syndrome?

Jo 1

Other investigations for myositis: Electromyography MRI ** Muscle _______ will confirm dx and subtype

biopsy

Treatment for vasculitis and myositis: _________ DMARDs Cyclophosphomide Rituximab

Steroids

MSI- AI disease seminar Flashcards

(67 cards)