Spectrum of Rheumatic Disease

Yuliasih
Ahli Penyakit Dalam – Konsultan Rematologi
Penyakit Dalam RSUD dr Soetomo -
FK Unair Surabaya
 Rheumatic Disease
Rheumatism refers to various painful medical
conditions which affect joints, bones, cartilage,
tendons, ligaments and muscles;

Rheumatic diseases, also called musculoskeletal

diseases, are characterized by pain and a consequent
reduction in the range of motion and function in one
or more areas of the musculoskeletal system;

signs of inflammation: swelling, redness, warmth in

the affected areas. Rheumatic diseases can also affect
internal organs;
 Rheumatic Disease
 Some people use the word arthritis to refer to all
rheumatic diseases. Arthritis, which literally
means joint inflammation, is just part of the
rheumatic diseases.

 not a single disorder

 more than 200 different diseases which span from
various types of arthritis to osteoporosis and on
to systemic connective tissue diseases.
 affect all ages and both genders, although women
are more frequently affected than men
 Rheumatic Disease

There are different symptoms to rheumatic diseases.

You might suspect that you have a rheumatic disease if
you have signs and symptoms which include the
following:
 Persistent joint pain
 Tenderness
 Inflammation indicated by joint swelling, stiffness,
redness, and/or warmth
 Joint deformity
 Loss of range of motion or flexibility in a joint
 Extreme fatigue, lack of energy, weakness, or a feeling of
malaise.
 Rheumatic Disease
There are many myths and misconceptions about arthritis.
Here are some examples:
 Arthritis is an old person's disease.
 Fact: Arthritis can occur at any age.
 Arthritis is induced by a cold, wet climate.
 Fact: Climate itself is neither the cause, nor the cure.
 Arthritis is caused by a poor diet.
 Fact: There is little scientific evidence that specific food
prevents or causes arthritis, there are few diseases, such as
gout, where intake of certain types of food or drinks
(alcohol) can precipitate an attack.
 Type of Rheumatic Disease

1. Systemic connective tissue diseases.

2. Vasculitides and related disorders.
3. Seronegative spondyloarthropathies.
4. Arthritis associated with infectious agents.
5. Rheumatic disorders associated with metabolic, endocrine,
and hematologic disease.
6. Bone and cartilage disorders
7. Hereditary, congenital, and inborn errors of metabolism
associated with rheumatic syndromes.
8. Nonarticular and regional musculoskeletal disorders.
9. Neoplasms and tumor-like lesions
10. Miscellaneous rheumatic disorders
Part of The Joints
Healthy Cartilage and
Function
 Healthy Synovial and Function
Synoviocytes are critical in producing the synovial fluid that keeps
the joint lubricated. Without the synovial fluid, articular cartilage
gets really beat up. Hyaluronates are very important parts of the
synovial fluid and keep it viscous. As inflammation happens, and
the fluid gets less viscous, ouch.
Vertebral column Vertebral canal and intervertebral foramen

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 Back pain

• Patients with significant low back pain cannot do a sit-

up.
• Most back pain is nonsurgical.
• Magnetic resonance imaging (MRI)/computed
tomography (CT) scans of lumbar spine are abnormal in
30% of patients with no symptoms. Do not attribute a
patient’s symptoms to an abnormal radiograph.
• Straight leg raise test and electromyography/nerve
conduction velocities (EMG/NCV) are often normal or
nonspecific.
 Tendon

Hand Tendonitis

Tendon Sheath
 Rheumatic Disease Classification

1. Inflammation & non inflammation

2. Articular & non articular
Articular Structure :
synovial membrane, cartilage,
ligament intra articular, capsule,
juxta articular bone surface
Non articular ;
bursa, tendon, muscle, bone, neuro
fiber , skin
3. Acute & chronic
4. Mono, oligo, polyarthritis (Count of
joint )

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 Inflammation & non inflammation

Inflammation Disease Symptoms

 Fever
 Stiff joints (> 1 hour)
 Weight loss
 Fatigue
 sign of inflammation (calor dolor rubor tumor)
 ESR , CRP 
Non Inflammation Disease Symptoms
No systemic or local symptoms of inflammation
exp : Osteoartritis, fibromyialgia

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Articular vs Periarticular

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Articular & periarticular

Periarticular
(tendinitis Achilles)

Articular

Trigger
Finger

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 Soft tissue rheumatism

• Most shoulder pain is periarticular (i.e., a bursitis or tendinitis). Rule out

impingement in patients with recurrent shoulder tendinitis.
• Causes of olecranon or prepatellar bursitis: trauma, infection, gout,
rheumatoid arthritis (RA).
• Recalcitrant trochanteric bursitis: rule out leg length discrepancy, hallux
rigidus with an abnormal gait, and lumbar radiculopathy.
• Recalcitrant medial knee pain: rule out anserine bursitis.
• Recalcitrant patellofemoral syndrome: rule out pes planus/hypermobility
causing patellar maltracking.
• Due to risk of rupture, do not inject corticosteroids for therapy of Achilles
tendinitis/enthesitis. Use iontophoresis
 Involved of The Joints

• Mono

• Oligo < 4 joints

• polyarthritis 24
 Site of hand or wrist Involvement and
their potential disease asscociations

DIP: OA, PsA, Reiter’s

PIP: OA, SLE,RA. PsA

MCP: RA, Pseudogout,

Hemochromatosis

1st CMC: Wrist: RA, Pseudogout,

OA Gonococcal arthritis,
De Quervain’s JRA,
tenosynovitis Carpal tunnel syndrome

Cush and Lipsky (2005). Harrison’s Principles of Internal Medicine.16th Edition, 2029- 25
2035
Distribution of Joints
Athritis
Arthritis
 Ankle arthritis

bunion
GALS
Source of Pain
Type of Pain
 Clue of Arthritis : RA
• Polyarthritis symetrics, more common small joints,
woman, chronic arthritis

• Synovial disease • High grade inflammation

 Rheumatoid Arthritis
Patterns
• Bilateral symmetric
• Involves small joints of
hands and feet
• DIPs & 1st CMC are
commonly spared
• Can also involve larger
joints in addition
Hand Deformities
Erosion Joint of Rheumatoid Arthritis

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 Clue of Arthritis : OA
• Cartilage disease, mechanical disease

• Woman or trauma • Low grade inflammation

Degradation Cartilage of OA
 Osteoarthritis
Patterns
• Bilateral Symmetric
• Spine, hands, feet &
weight bearing joints
(knees & hips)
• DIPs & 1st CMC
commonly involved
(differentiates from
RA)
• Can affect other joint
areas that are subject
to overdue
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Impact of OA

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 Clue of Arthritis : Spondyloarthritis
• Tendon disease, spine arthritis

• Extra articular manifestation • Low grade inflammation

 Axial Patterns
Spondyloarthropathy
• Spondylitis prominent
• SI joint involvement
prominent
• Another clue is enthesitis
• Can affect other joints as well
and tends to be an
oligoarthritis
• Four types of seronegative
spondyloarthropathies
– Ankylosing Spondylitis
– Psoriatic
– Reactive
– Inflammatory Bowel Disease
 Perifer Patterns
• Billateral asymmetric
polyarthritis
• Prominent DIP involvement
• Other clues are enthesitis,
dactylitis, tenosynovitis,
psoriatic skin changes, psoriatic
nail changes
• The 5 patterns of psoriatic
arthritis are :
– Classic
– RA like
– Oligoarthritis
– Arthritis mutilans
– spyndyloarthropathy
Radiographic

Sacroiilitis

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Impact of Axial Spondylitis

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 Clue of Arthritis : Gout
• Crystal disease, podagra, tophus, common in man

• Mono/Polyarthritis unsymmetrical • High grade inflammation

 Gout Patterns

• 1st MTP is a classic

presentation
• Can also affect knees, MTPs,
PIPs, and DIPs prominently
• Commonly monoarthritis
• Can also present as bilateral
asymmetrical polyarthritis
• Red, hot and prominent tophi
are clues
Radiographic

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Impact of Chronic Gout Arthritis

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 Clue of Arthritis : Connective tissue disease
• Woman, child bearing, rash, pigmentation, vasculitis

polyarthritis nodosa rash

• Non specific arthritis • Internal organ manifestation

Fibrosis Skin : The trunk is never involved. In limited scleroderma
These sclerodermatous changes typically affect the face, neck
and extremities distal to the elbows and knees.
Skin thickening typically peaks in the first 3 to 5 years
 Clinical manifestation of face
Patients frequently complain of darkening of complexion. Peri-oral fibrosis leads to
a “puckered mouth”. This, along with pinched nose and mask like face,
constitutes the typical scleroderma facies.

history of Raynaud’s phenomenon for 5-10 years before development of other

features of scleroderma.
Connective Tissue Disease

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Pericardial effusion Pleural effusion

CVA infark

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 Laboratorium test
• Laboratory tests should be used to confirm your clinical diagnosis not make it.
• All patients with a positive rheumatoid factor do not have RA, and all patients
with a positive antinuclear antibody do not have systemic lupus erythematosus
(SLE).
• Low complement (C3, C4) levels in a patient with systemic symptoms suggest an
immune complex-mediated disease and narrows your diagnosis: SLE,
cryoglobulinemia (types II and III), urticarial vasculitis (HepB and C1q
autoantibodies), subacute bacterial endocarditis (SBE), poststreptococcal or
membranoproliferative glomerulonephritis.
• An undetectable (not just low) CH50 activity may indicate a disease associated
with a hereditary complement component deficiency: autoimmune (C1, C4, C2),
infection (C3), Neisseria infection (C5 to C8).Separating iron deficiency from
anemia of chronic disease is best done by measuring the ferritin level. In a
patient with elevated C-reactive protein, a ferritin level of >100 ng/mL rules out
iron deficiency.
 Laboratory ..
• Hematologic parameters
– Anaemia
– Thrombocytosis
– ↓ Serum iron & IBC
– ↑ Serum globuline
– ↑ ALP
– ↑ Acute phase reactant
• Immunological parameters :
ANA,dsDNA.RF
 American College of Rheumatology (ACR)
recommended the following five tests/treatments not
be done in adult rheumatology patients:
• Do not test antinuclear antibody (ANA) subserologies (anti-dsDNA,
anti-Sm, anti-RNP, anti-SS-B, anti-Scl-70) without a positive ANA and
clinical suspicion of immune-mediated disease. Anti-SS-A may be an
exception to this recommendation
• Do not test for Lyme disease as a cause of musculoskeletal
symptoms without an exposure history and appropriate
examination findings.
• Do not perform an MRI of the peripheral joints to routinely monitor
inflammatory arthritis.
• Do not prescribe biologics for RA before a trial of methotrexate (or
other conventional nonbiologic disease-modifying antirheumatic
drugs [DMARDs]).
• Do not routinely repeat dual-energy X-ray absorptiometry (DXA)
scans more often than once every 2 years.
Pattern ANA Test
ANA Profile

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70
 The synovial fluid analysis is a liquid
biopsy of the joint.
• One can estimate the synovial fluid white blood cell
(WBC) count by using the equation that one WBC per
high powered field (HPF; 40×) equals 500 cells/μL.
Thus, 6 WBCs/HPF estimates a synovial fluid WBC
count of 3000 cells/μL, which is inflammatory.
• Crystal mnemonic: ABC = Alignment Blue Calcium. If
the long axis of the crystal is aligned with the first
order red compensator and is blue then it is a calcium
pyrophosphate crystal. Uric acid crystals are yellow
when aligned.
• If you cannot find uric acid crystals initially, let the
slide dry for 3 hours and reexamine it.
A few other “do nots” in rheumatology
• Except for anti-dsDNA, do not repeat ANA subserologies in patients with
an established connective tissue disease (CTD) diagnosis.
• Do not perform serial measurements of rheumatoid factor and anti-cyclic
citrullinated peptide (CCP) in patients with documented seropositive RA or
serial ANAs in patients with a documented positive ANA and a CTD
diagnosis (e.g., SLE).
• Do not order a human leukocyte antigen (HLA)-B27 unless you suspect an
undifferentiated spondyloarthropathy based on history and examination
but have nondiagnostic radiographs.
• Do not check CH50 to follow lupus disease activity.
• Do not order an MRI before ordering plain films in a patient presenting
with joint or back pain.
• Do not use intraarticular hyaluronic acid injections for advanced knee OA
(i.e., bone on bone).
• Do not treat low bone mass in patients at low risk for fracture (T score > –
2.5, no history of fragility fracture, no steroids, low FRAX).
 Important information
• The innate immune system is critical to the
activation of the adaptive immune system.
• Joint effusion and limited range of motion are the
most specific signs for arthritis.
• True hip joint pain is in the groin. In a young
patient who cannot flex their hip greater than
90°, rule out femoroacetabular impingement
syndrome.
• Feel both knees with the back of your hand for
temperature differences and compare it to the
lower extremity.
• Extraarticular manifestations are often the most
important findings to make a diagnosis in a
patient with polyarthritis.
• Myopathies tend to cause proximal and
symmetric weakness, whereas neuropathies
cause distal and asymmetric weakness and
atrophy of muscles. Cardiac disease occurs 10
years earlier in patients with inflammatory
rheumatic disease compared to normal
individuals with the same cardiac risk factors.
• This must be considered during the preoperative
• VAST MAJORITY OF
MUSCULOSKELETAL
DISORDERS CAN BE
EASILY DIAGNOSED BY A
COMPLETE HISTORY AND
PHYSICAL.