Seronegative (RHEUM)

PEAR mnemonic

P - psoriatic arthritis

E - enteropathic spondyloarthropy

A - ankylosing spondylitis

R - reactive arthritis

PEAR diseases share

- RF negative

- HLA-B27 association, family associated

- Inflammatory oligioarthritis or monoarticular

- Inflammation of fascia, ligaments, tendon, bone (enthesitis)

- Extra articular involvement

Psoriatic arthritis (PsA)

Patients with psoriasis (strongest risk factors)

HIV

30-55 y/o

Joint/tendon trauma

Upper extremities and small joints in hand

Symmetric or asymmetric, gradual

PsA pathophysiology

Autoimmune (environmental trigger —> autoimmune overreaction)

T cell infiltration, angiogenesis

Chemokines/cytokines

Pencil in cup finding

Most common in psoriatic arthritis (PsA)

Erosion of DIP in pointed manner

PsA medicine

DMARD

MTX > suldasalazine, leflunomide

Anti-TNF: etanercept, adalimumab, infliximab

Ankylosing spondylitis (AS)

Males 20-30 y/o

MC seronegative spongyloarthropathy

Chronic dull low back pain and stiffness worse at night

- Improves with heat, exercise

SI - bilateral SI joint involvement

Spine - fusion in ascending manner, kyphosis, reduced mobility, "bamboo spine", question mark posture

5A's

5 A's of AS

1. Anterior uveitis

2. Aortic regurg

3. Achilles tendinitis

4. AV block

5. Amyloidosis (rare and/or late)

AS dx and tx

HLA-B27

Dx:

- Elevated ESR, CRP

- ANA neg

- Imaging of back = bamboo spine

Tx: NSAIDs >

- anti-tnfs: etancercept, infliximab

- Surgery for deformity

- Immobilization when back trauma

- PT

Bamboo spine

AS finding

Reactive arthritis

Reiter's syndrome

Extra articular:

- Keratoderma blennorhagicum

- Mouth ulcer

Reactive arthritis dx and tx

Dx: Joint aspiration - cloudy

- Test serum for chlamydia

Tx: NSAIDs, intra-articular steroids

- DMARDs > anti-TNF for perisistent or refractory disease

Reactive arthritis x ray findings

X ray - normal at beginning

- marginal erosion

- plantar spurs

- sacroilitis

- asymmetrical syndesmophytes

Enteropathic Arthritis

Stomach linked - IBD (crohns or ulcerative colitis) bacterial & parasitic infections, celiac, pseudomembranous colitis, whipple's disease

Peripheral arthritis, asymmetric and large joints

Enthesitis, dactylitis

Uveitis, erythema nodosum

Fistulas

Enteropathic Arthritis dx and tx

Dx: HLA-B27, CPR and ESR increased

- elevated wbc in synovial

- x-ray like AS

Tx: different for inactive vs active

Inactive enteropathic arthritis tx

NSAIDs >

- Celebrex preferred

Intra-articular steroids

DMARDs for refractory

- sulfasalazine or MTX for peripheral

- Adalimumab for axial

Active enteropathic arthritis tx

Include GI in treatment

- Celebrex if tolerated

Sulfasalazine, MTX

Large vessel vasculitis examples

Giant Cell Arteritis

Takayasu's arteritis

Medium vessel vasculitis examples

Polyarteritis nodosa

Behcet's disease

Small vessel vasculitis examples

Eosinophilic Granulomatosis with

Polyangitis

Granulomatosis with Polyangitis (GPA)

Vasculitis

Inflammatory diseases of blood vessels that lead to

vessel damage and organ dysfunction

Common presentations include systemic symptoms, skin

lesions, organ damage, and vascular occlusion

Giant cell arteritis

AKA temporal arteritis

Women > 50 y/o (with polymyalgia rheumatica)

Granulomatosis inflammation of large vessels

Temporal artery > aorta, external carotid, vertebral

Blindness and aneurism risks

Headache, scalp tenderness

Absent temporal pulses

Palpable nodules

Giant cell arteritis dx and tx

Dx: ESR > 50 mm/hr but not requires

- Biopsy of temporal artery --> multinucleated giant cells

- Ultrasound with thickened blood vessel wall = halo sign

Tx: High dose steroids prednisone >

- IMMEDIATE treatment, especially for vision loss

- Vision loss = IV steroids

- Referral to opthalm

Takayasu Arteritis

Granulomatous vasculitis of aortic arch and pulmonary arteries causing fibrosis, stenosis, and narrowing

Women > 10-40 y/o

Limb ischemia and absent pulses in upper extremities

Aortic aneurysm, stroke

Secondary HTN due to renal artery stenosis

> 10 mmHg BP difference between arms

Visual disturbances

Takayasu Arteritis dx and tx

Dx: angiography --> narrowing of aorta or primary branch

- elevated ESR and CRP

- CBC: normochronic, normocytic anemia, leukocytosis

Tx: High dose corticosteroids >

- DMARDs (MTX or azathoprine)

- Revascularization

Polyarteritis nodosa (PAN)

Middle aged men

Nervous, heart, GI, kidney system vasculitis --> necrotizing arteritis

- PMN invasion into all layers of vessel = reduced luminal area

- Ischemia, infarct, aneurysms

- Sx affecting all symptoms (abdominal pain, MI, HTN, renal failure, neuropathy, stroke)

Raised diastolic > 90 mmHg

Hep B, HIV, drug reactions

Rosary sign

Livedo reticularis rash, skin ulcers

Polyarteritis nodosa (PAN) dx and tx

Dx: microaneurisms

- Fecal occult blood test

Tx: Corticosteroids

- Graduate to cyclophosphamide if severe

Behcet disease/Syndrome

Turkish, middle eastern men

Oral, genital ulcers, erethyma nodosum

Uveitis

Mono / oligio arthritis

Neurological features

THROMBOSIS !

Behcet disease dx and tx

Dx: skin pathergy

- needle prick --> papule < 48 hrs

Tx:

- Topical steroids - ulcers, opthalmic for eye

- Sucralfate

- Colchicine, apremilast for prevention of lesions

Eosinophilic Granulomatosis with Polyangitis (EGPA)

AKA churg-strauss syndrome

(CSS)

Necrotizing granulomatous inflammation = eosinophilia

- Asthma

- Chronic rhinosinusitis

- Widespread involvement, other than respiratory; cardiac, GI, skin, renal, neurologic = skin lesions, peripheral neuroathy, heart failure, myocarditis, pericarditis, VTE, renal failure, MI

EGPA dx and tx

Dx: elevated eosinophils (BAL)

- Lung or skin biopsy

- p-ANCA

- CXR/CT opacities

- Sinus CT opacification

Tx: Prednisone, IV steroids when systemic

- Cyclophosphamide for cardio

- Rituximab if ANCA +

Granulomatosis with Polyangiitis (GPA)

c-ANCA associated, necrotizing, granulomatous affecting older adults

- Respiratory: septal perforation, saddle nose, mucosal ulceration, OM, rhinorrhea, hemoptysis, pulm nodules, tracheal hoarseness

- Renal: nephritic syndrome (blood, protein, HTN, uremia)

GPA dx and tx

Dx: + c-ANKA

- Cxy/CT nodules

- Anemia (monochronic, normocytic), thrombocytopenia, leukocytosis

- Increased Cr, urine sediment

- Skin, kidney, renal biopsy

Tx: steroids + rituximab

- Cyclophosphamide if needed (instead of rituximab)

- Renal transplant

EPGA vs GPA

Both:

- Granulomatous, necrotizing

- Affecting respiratory = opacities in CXR/CT

- ANKA associated

- Dx: skin or lung biopsy

- Tx (steroids, cyclophosphamide, rituximab)

EPGA: more involved with other organ systems / systemic

- eosinophilia

- p-ANKA

- Nasal polyps, sinus opacification

GPA: + renal only

- c-ANKA

- Nasal ulcers, more upper and lower resp sx

- Older adults

- Anemia, thrombocytopenia, leukocytosis

Thromboangiitis Obliterans (TAO)

AKA Buerger's disease

- Young male

- Heavy smoker (tobacco and/or dat green)

Upper and lower extremity ischemia, arthralgia

- Knee, wrist MC for arthralgia

- Ischemia --> ulcers --> gangrene

Gangrene: fingers and toes MC, painful, red, cyanotic

= "autoamputation"

Highly cellular and occlusive with thrombus (sparing vessel wall)

- migratory superficial throbophlebitis

- Raynauds

TAO dx and tx

Dx: positive allen test

- < 50 y/o

- Ankle-brachial index (ABI)

- Biopsy: definitive, not usually needed

- Ddx: repetitive trauma (jackhammer operator for example)

Tx: Smoking cessation only option that is curative

- ulcer debridement and dressing changes

- Vasodilators, CCB