VGH CTU Noon Rounds

The Rheumatologic Review of Systems: A Crash Course

Athena Huynh, Internal Medicine PGY-3

July 8, 2024

Objectives

By the end of this presentation, you should be able to…

1. Perform a focused rheumatologic review of systems tailored to the condition(s) under suspicion
2. Define the major rheumatologic conditions and their common manifestations
3. Characterize positive symptoms to determine whether it may be in keeping with a rheumatologic condition

Overview of Rheumatologic Diseases

• Rheumatoid Arthritis
• Spondyloarthritis (PEAR)
• Psoriatic arthritis
• Enteropathic arthritis
• Ankylosing spondylitis
• Reactive arthritis
• Connective Tissue Disease
• Systemic lupus erythematosus
• Sjӧgren’s syndrome
• Scleroderma
• Inflammatory myositis
• Mixed connective tissue disease

• Vasculitis
• Large: Giant cell arteritis (Polymyalgia rheumatica), Takayasu arteritis
• Medium: Polyarteritis nodosa, Kawasaki disease
• Small: ANCA-associated, Immune complex-mediated
• Crystal Arthropathy
• Gout
• Pseudogout
• Weird & wonderful: sarcoid, IgG4-related disease, Still’s disease…

Overview of Rheumatologic Diseases

• Rheumatoid Arthritis
• Spondyloarthritis (PEAR)
• Psoriatic arthritis
• Enteropathic arthritis
• Ankylosing spondylitis
• Reactive arthritis
• Connective Tissue Disease
• Systemic lupus erythematosus
• Sjӧgren’s syndrome
• Scleroderma
• Inflammatory myositis
• Mixed connective tissue disease

• Vasculitis
• Large: Giant cell arteritis (Polymyalgia rheumatica), Takayasu arteritis
• Medium: Polyarteritis nodosa, Kawasaki disease
• Small: ANCA-associated, Immune complex-mediated
• Crystal Arthropathy
• Gout
• Pseudogout
• Weird & wonderful: sarcoid, IgG4-related disease, Still’s disease…

Before we begin… ask about constitutional symptoms!

Recurrent fevers, malaise, drenching night sweats, unexplained weight loss, **fatigue** etc. etc.

Outline

• Rheumatoid Arthritis
• Spondyloarthritis (PEAR)
• Psoriatic arthritis
• Enteropathic arthritis
• Ankylosing spondylitis
• Reactive arthritis
• Connective Tissue Disease
• Systemic lupus erythematosus
• Sjӧgren’s syndrome
• Scleroderma
• Inflammatory myositis

• Vasculitis
• Large: Giant cell arteritis (Polymyalgia rheumatica)
• Small: ANCA-associated

Rheumatoid Arthritis

Definition: A symmetric, inflammatory, peripheral polyarthritis often the affecting the wrists, MCPs & PIPs, and MTPs; it does NOT affect the DIPs!

Review of systems: joint pain (see next slide), dry eyes, dry mouth, eye pain or redness, cough, dyspnea, (pleuritic) chest pain, Raynaud’s phenomenon, skin changes (e.g., nodules, pyoderma gangrenosum)

Joint Pain - Inflammatory vs. Non-Inflammatory

• When did the joint pain start?
• How did the joint pain start, and how has it progressed over time?
• Is there (or has there ever been) associated pain, redness, or swelling?
• Does the joint pain get worse or better with rest? What about activity?
• What has been tried for pain management, and was it helpful?
• Is there morning stiffness? If so, how long?
• Are there associated systemic symptoms?

Outline

• Rheumatoid Arthritis
• Spondyloarthritis (PEAR)
• Psoriatic arthritis
• Enteropathic arthritis
• Ankylosing spondylitis
• Reactive arthritis
• Connective Tissue Disease
• Systemic lupus erythematosus
• Sjӧgren’s syndrome
• Scleroderma
• Inflammatory myositis

• Vasculitis
• Large: Giant cell arteritis (Polymyalgia rheumatica)
• Small: ANCA-associated

Spondyloarthritis

Definition: A family of seronegative diseases characterized by inflammation of the spine and joints, along with other extra-articular features

• PEAR = Psoriatic arthritis, Enteropathic arthritis, Ankylosing spondylitis, and Reactive arthritis (formerly Reiter’s)
• Associated with the HLA-B27 gene

Review of systems: joint pain, personal or family history of psoriasis, abdominal pain, diarrhea, urgency, tenesmus, blood or mucus in stools, inflammatory back pain, dactylitis, enthesitis, uveitis, gastrointestinal or genitourinary infection within 1-2 months of symptom onset, dysuria, pain with sex

Back Pain - Inflammatory vs. Non-Inflammatory

• When did the back pain start? Was there any inciting trauma at the time?
• Where is the back pain? Is there alternating buttock pain?
• Does the back pain get worse with rest? What about activity?
• Does the back pain wake you up at night? If so, is it in the first or second half of the night?
• Have NSAIDs been helpful for the back pain?
• Remember to screen for red flag features!

Uveitis (usually anterior)

• Inflammation of the uvea, the middle portion of the eye
• Usually anterior as opposed to intermediate or posterior
• Symptoms include eye pain or redness, blurry vision, photophobia, and excess tearing
• Have you ever had inflammation of the eyes for which you needed to see an eye doctor and get treated with steroid eye drops?

Dactylitis

• Inflammation of an entire finger (flexor tenosynovitis) causing generalized swelling and a “sausage digit” appearance
• Preference for 3rd and 4th toes
• Does not resolve without treatment*
• Have your fingers or toes ever swollen up to the point where they looked like sausages?

Enthesitis

• Inflammation where a tendon, ligament, or joint capsule inserts into bone
• Have you had issues with your tendons and ligaments such as Achilles tendonitis, plantar fasciitis, patellar tendonitis, golfer’s elbow, or tennis elbow?

Outline

• Rheumatoid Arthritis
• Spondyloarthritis (PEAR)
• Psoriatic arthritis
• Enteropathic arthritis
• Ankylosing spondylitis
• Reactive arthritis
• Connective Tissue Disease
• Systemic lupus erythematosus
• Sjӧgren’s syndrome
• Scleroderma
• Inflammatory myositis

• Vasculitis
• Large: Giant cell arteritis (Polymyalgia rheumatica)
• Small: ANCA-associated

Connective Tissue Disease (Acquired)

There are technically 5, but you just need to remember the core 4…

1. Systemic lupus erythematosus
2. Sjögren’s syndrome
3. Systemic sclerosis
4. Dermato/polymyositis
5. Mixed connective tissue disease

There is also undifferentiated connective tissue disease and overlap syndromes.

Systemic Lupus Erythematosus

Definition: A chronic autoimmune disease characterized by production of autoantibodies to components of the cell nucleus, resulting in diverse clinical manifestations

Review of systems: headaches, excessive hair loss, oral/nasal ulcers, photosensitivity, rash, myalgia, arthralgia, Raynaud's phenomenon, (pleuritic) chest pain, dyspnea, cough, abdominal pain, hematuria, frothy urine, pedal edema, paresthesias, previous seizures or psychosis; history of pregnancy loss or thromboembolism

Mucocutaneous Manifestations in Lupus

Excessive hair loss:

• When tying your hair, do you have to do an extra turn on the elastic now?
• When you wake up in the morning, is there hair on your pillow?

Oral/nasal ulcers:

• Have you had any oral or nasal ulcers? If so, how many?
• Have any of your oral ulcers occurred on the roof of the mouth?

Photosensitivity:

• Do you develop itchy eruptions or areas of redness and inflammation after sun exposure?

Mucocutaneous Manifestations in Lupus - Rash

Sjögren’s Syndrome

Definition: Autoimmune lymphocyte-driven attack against exocrine glands, causing glandular and extraglandular manifestations

Review of systems: dry eyes, dry mouth, dry skin, vaginal dryness, Raynaud's phenomenon, joint pain, facial swelling, lymphadenopathy, cough, chest pain, dyspnea, or paresthesias

*Note: chronic fatigue and chronic pain are common in Sjögren’s syndrome, so it is not uncommon for people to present with comorbid fibromyalgia and psychiatric diagnoses

Sicca Symptoms (dry eyes, dry mouth)

Dry Eyes:

• Do you use lubricating eye drops or artificial tears? If so, how often?
• Is there any redness, stinging or burning, foreign body sensation, photophobia, or sensitivity to light?
• Have you ever had any corneal ulcers?

Dry Mouth:

• Do you have to wake up in the middle of the night to drink water?
• Do you need to take sips of water to swallow dry food?
• Do you have burning in your mouth or changes in taste?
• Do you have frequent dental caries?

Systemic Sclerosis

Definition: A chronic multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis of the skin and internal organs

• Scleroderma = thick tight skin
• Systemic sclerosis = scleroderma PLUS internal organ involvement
• Limited systemic sclerosis = skin involvement only below elbows and knees (plus face)
• Diffuse systemic sclerosis = skin involvement extending above elbows and knees

Review of systems: Raynaud's phenomenon, skin thickening or tightening, digital ischemia or ulcers, gastroesophageal reflux, dysphagia, cough, dyspnea, chest pain, history of gastrointestinal bleeding, or paresthesias

Raynaud’s phenomenon

• Definition: Reversible vasospasms of digital arteries/arterioles with pallor and rubor or cyanosis (i.e., exaggerated reaction to cold)
• Can be primary or secondary
• Often associated with systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, and vasculitis
• Do your fingers change colour in the cold? If yes, is the colour change well demarcated?
• What colours do your fingers and toes turn? (white, purple/blue, red)
• How long have you had it for? Has it changed over time?
• Does it bother you a lot?
• Is there any history of digital ulcers or digital ischemia?

Raynaud’s Phenomenon - Mimickers

Inflammatory Myositis

Definition: A group of autoimmune diseases characterized by inflammation of the skeletal muscles +/- skin, often with systemic manifestations

• Multiple subtypes: dermatomyositis, anti-synthetase syndrome, inclusion body myositis, immune-mediated necrotising myopathy, etc…

Review of systems: proximal muscle weakness, Raynaud's phenomenon, digital ischemia or ulcers, characteristic rashes in sun-exposed areas, cough, chest pain, dyspnea, or dysphagia

Characteristic Dermatomyositis Rashes

1. Gottron’s papules
2. Nailfold telangiectasias, ragged cuticles
3. Mechanic’s hands
4. Gottron’s sign
5. Facial erythema, heliotrope rash
6. V-neck sign

Outline

• Rheumatoid Arthritis
• Spondyloarthritis (PEAR)
• Psoriatic arthritis
• Enteropathic arthritis
• Ankylosing spondylitis
• Reactive arthritis
• Connective Tissue Disease
• Systemic lupus erythematosus
• Sjӧgren’s syndrome
• Scleroderma
• Inflammatory myositis

• Vasculitis
• Large: Giant cell arteritis (Polymyalgia rheumatica)
• Small: ANCA-associated

Vasculitis

Definition: Inflammation of blood vessel(s), with clinical manifestations related to LOCAL and SYSTEMIC inflammation

• Consequences of LOCAL inflammation:
• Lumen narrowing/stenosis = ISCHEMIA/CLAUDICATION
• Vessel wall damage = ANEURYSMS, DISSECTIONS
• Consequences of SYSTEMIC inflammation:
• Constitutional symptoms (fevers, weight loss, night sweats)
• Arthralgias or arthritis, myalgias
• Clots (i.e. venous thromboembolism)

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Giant Cell Arteritis (a.k.a. Temporal Arteritis)

Definition: Large vessel vasculitis affecting people >50 years of age

• 40-60% of patients w/ GCA have PMR

Review of systems: headache, **vision changes**, jaw/tongue claudication, scalp tenderness, limb claudication, dry cough, chest pain, constitutional symptoms, PMR symptoms

Polymyalgia Rheumatica (PMR)

Definition: An autoimmune disorder characterized by pain and stiffness around the neck, shoulder, and hip area without associated muscle weakness

• If there is objective proximal muscle weakness, think about inflammatory myositis

Review of Systems: inflammatory pain and stiffness in the shoulders, neck, hips, glutes; morning stiffness, malaise, fatigue, depression, anorexia, weight loss, low-grade fever, GCA symptoms

• Also ask about peripheral joint pain, as elderly-onset RA often gets misdiagnosed as PMR
• PMR pain should NOT be responsive to acetaminophen or NSAIDs

ANCA-Associated Vasculitis

1. Microscopic polyangiitis (MPA) = pulmonary renal syndrome + mononeuritis multiplex
2. Granulomatosis with polyangiitis (GPA) = granulomas affecting the upper respiratory tract + MPA
3. Eosinophilic granulomatosis with polyangiitis (EGPA) = adult-onset asthma and/or chronic rhinosinusitis + peripheral eosinophilia and eosinophilic granulomas + MPA

ANCA-Associated Vasculitis

Review of systems: hearing loss, tinnitus, eye inflammation, nasal polyps/crusting/ulcers, epistaxis, adult-onset asthma, chronic rhinosinusitis, chest pain, dyspnea, cough, hemoptysis, stridor, abdominal/pelvic pain, hematuria, paresthesias, sensory and/or motor deficits in extremities, skin changes (i.e., purpura, ulcers, reticular changes, Raynaud's phenomenon, nodules)

Putting it Together - General Rheumatology ROS

• Constitutional: fevers, drenching night sweats, weight loss
• HEENT: headache, scalp tenderness, changes in vision, jaw claudication, excessive hair loss, dry eyes or mouth, oral/nasal ulcers, eye pain/redness, photophobia, sinus or nasal pain, epistaxis
• MSK: joint pain, swelling, or morning stiffness >30-60 minutes; proximal muscle weakness; inflammatory back pain
• Skin: tightness or thickening in fingers, toes, or face; rash (e.g. psoriasis, purpura, malar, discoid, reticular); Raynaud's phenomenon; sun sensitivity; nodules; ulcers
• CVS/Resp: pleuritic chest pain, dyspnea, cough, hemoptysis
• GI: bloody diarrhea, intestinal IBD symptoms, GERD, dysphagia, abdominal pain
• GU: hematuria, frothy urine
• Neuro: paresthesias, loss of sensation or motor function in limbs; history of seizures or psychosis
• Heme: previous DVT/PE, pregnancy losses, or complications related to placental insufficiency
• ID: Preceding GI or GU infection, dysuria

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Thank you for listening!