Rheumatology I 03 Connective Tissue Diseases

Rheumatic Diseases
Connective Tissue Diseases
With Kartik Rangaraj MD
Wen Jie Lau, j.wenjie93@hotmail.com

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Connective Tissue Diseases
Classification
• Prototype is Systemic Lupus Erythematosus
(SLE)
• Also included is rheumatoid arthritis (RA), SLE

Sjögren’s syndrome (SS), scleroderma (Scl), Sjögren’s
PM/
Raynaud’s phenomenon, DM
polymyositis/dermatomyositis (PM/DM)
RA
Sclero-
derma
Raynaud’s
• Considerable amount of overlap can occur
between all CTDs

Approach to Rheum Labs
Interpretation of autoimmune labs is the most common consult for rheumatologists in the hospital:
• KEY: Labs generally do not make or break a diagnosis of autoimmune disease!

Rheumatologic disease is a clinical, not laboratory, diagnosis.
• ANA: Anti-nuclear antibody

• Multiple antibodies that demonstrate specificity for nucleic acids and nucleoproteins
• Measured by immunofluorescence microscopy; reported as a titer
• Used as screen for CTDs (except RA; i. e., possesses high sensitivity, low specificity)
• (+) ANA also seen with aging, HIV, viral hepatitis, interstitial lung disease, TB,
malignancy (especially B-cell lymphomas)
• If negative, chance of CTDs (except RA) virtually nil

If ANA (+), then order tests to speciate ANA
• dsDNA: anti-double-stranded DNA antibody

• Specific for SLE, especially lupus nephritis
• Titers can be used for monitoring disease activity in some patients.
• SS-A/Ro: seen in Sjögren’s syndrome, SLE; associated with photosensitivity, neonatal lupus,
fetal congenital heart block in mothers with SLE
• SS-B/La: seen in Sjögren’s syndrome
• RNP (ribonucleoprotein): mixed connective tissue disease (combination of lupus,

scleroderma, myositis)

If ANA (+), then order tests to speciate ANA
• Anti Scl-70: seen in systemic sclerosis (scleroderma) with increased risk of

interstitial lung disease
• Anti-centromere: seen in CREST (scleroderma)
• Anti-histone: seen in DIL (drug-induced lupus)
Anti-Jo-1 Ab: seen in anti-synthetase syndrome (polymyositis + interstitial lung disease)

Other labs:
• Rheumatoid factor: seen in RA, Hep C
• IgG anti-IgM
• Seen in RA:
• Anti-CCP Ab: CCP is cyclic citrullinated peptide, a derivative of arginine
CRP: C-reactive protein

• An acute phase reactant
• Varies more acutely with level of inflammation

ESR: Erythrocyte sedimentation rate
• Higher the number, the higher level of acute phase reactants (esp. fibrinogen)
• Acute phase reactants coat RBCs, decreasing repulsive zeta forces, increasing rouleaux
formation of RBCs and thereby packing in ESR tube
• False elevation seen in severe anemia
• False decrease in advanced liver disease, nephrotic syndrome, protein-losing

enteropathies/malnourishment

Rheumatoid Arthritis

Rheumatoid arthritis is a chronic, symmetric, polyarticular,

inflammatory, systemic, autoimmune disease that attacks the
synovium of the joints.
Causes a polyarticular, symmetrical arthritis with extra articular

manifestations:
• Rheumatoid nodules (skin, lungs); rheumatoid lung (interstitial
fibrosis); rheumatoid vasculitis (skin, nerve, internal organs)
Definition
• Episcleritis/scleritis
• Felty’s syndrome
• Pericarditis

• Unclear: Antigen-presenting cells (macrophages, dendritic cells),
T lymphocytes, B lymphocytes, and neutrophils appear to be
involved.
• These cells elaborate cytokines (TNF-α) that are associated
with disease.
• Rheumatoid factor and anti-CCP antibodies confer
more aggressive disease.
• Associated with female gender, HLA-DR4
Pathogenesis
This complex interaction causes the inflamed synovium (synovitis) to
thicken creating a pannus. Eventually, without treatment, the
inflamed synovium can erode bone creating erosions and
permanent disability.
• Current therapy can reverse synovitis and synovial thickening;
erosions and joint disability are irreversible

Destruction of cartilage
Bone
Inflamed joint
capsule
Inflamed
synovium
Synovial fluid
Joint pain occuring
(Enlarged view of a joint)
in various joints
© by Lecturio
• RA is a polyarticular, symmetric, inflammatory, arthritis.
• Active synovitis: on examination the joints will be warm, swollen,
red, and tender.
• Patients complain of morning stiffness that improves with use.
• Patients have involvement of multiple joints.
• The wrists, MCPs, and PIPs are most commonly involved.
• Joints are involved in a symmetric fashion.
Signs & symptoms
• Patients may develop rheumatoid nodules along extensor
tendons.
• Only in RF+ patients
• Synovium also line tendons (called tenosynovium), causing carpal
tunnel syndrome (palmar flexor tendonitis) or rotator cuff tendonitis

As the disease progresses, joint destruction occurs resulting in

deformity:
• Ulnar deviation of the fingers
• Swan-neck deformity of the fingers
• Boutonniere deformity of the fingers
• Atlanto-axial (C1–C2) subluxation may cause spinal cord injury

in patients who have involvement of their vertebral joints.
Signs & symptoms AM stiffness

Zarei-Ghanavati S, Javadi MA, Yazdani S, Bilateral Terrien's Marginal Degeneration and Posterior Polymorphous Dystrophy (…) https://openi.nlm.nih.gov/
detailedresult.php?img=PMC3381109_jovr-07-60f1&query=Rheumatoid+Arthritis&it=xg&lic=by&req=4&npos=2, no changes, CC BY 2.5
F.l.t.r.: E. Goljan, Rapid Review Pathology E-Book, 4th Edition, 2013, p. 642, Fig. 24-8 B, Mosby (Elsevier), Agrawal RV, Murthy S, Sangwan V et al., Current approach in diagnosis and management
of anterior uveitis., https://openi.nlm.nih.gov/detailedresult.php?img=PMC2841369_IJO-58-11-g010&query=rheumatoid+arthritis&it=xg&lic=by&req=4&npos=242, CC BY 2.0, resized and cropped
Extra articular manifestations

• Heme: anemia of chronic disease, lymphoproliferative
malignancies (lymphoma)
• Pulmonary: pulmonary nodule, diffuse interstitial fibrosis,

pleural effusion (exudate with low glucose)
• Cardiac: pericardial effusion, pericarditis, aortitis, increased

incidence of coronary artery disease (~8-fold increase)
Signs & symptoms
• Felty’s syndrome: splenomegaly, neutropenia, and RA
• Rheumatoid vasculitis (skin, nerve, internal organs)
• Episcleritis/scleritis
• Overlap with SLE (lupus), Sjögren’s syndrome (eye sicca)

Diagnostic criteria: (need 4 of 7 criteria: 90 % sensitivity and
specificity)
1. Morning stiffness > 1 hr for 6 weeks
2. Arthritis > 3 joints simultaneously for 6 weeks
3. Hand joint arthritis (MCPs, PIPs) for 6 weeks
4. Symmetric joint involvement for 6 weeks

Diagnosis
5. Rheumatoid nodules
6. Positive rheumatoid factor
7. Radiographic changes of joint erosions
Remember differences between RA and OA

• NSAIDs are the first line treatment for patients with RA

(for exam purposes).
• In reality, DMARDs (disease modifying anti-rheumatic
drugs) are first line therapy.
• Glucocorticoids are necessary for many patients with

symptomatic disease.
• Their use should be minimized, if possible , to avoid the side-

effects of chronic steroid use.
Treatment

• Goal is to prevent flares with DMARDs or biologic agents and

control flares with steroids.
• Biologic agents reserved for severe disease given
increased risk of life-threatening infections
• Examples of DMARDs include: methotrexate, sulfasalazine,

azathioprine, and leflunomide
• Examples of biologic agents include: etanercept (Enbrel),

adalimumab (Humira), infliximab (Remicade), rituximab
Treatment (Rituxan), and abatacept (Orencia)

This document is a property of: Wen Jie Lau
Note: This document is copyright protected. It may not be copied, reproduced, used, or
distributed in any way without the written authorization of Lecturio GmbH.


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Rheumatic Diseases

Connective Tissue Diseases

With Kartik Rangaraj MD

Wen Jie Lau, j.wenjie93@hotmail.com

• Also included is rheumatoid arthritis (RA), SLE

Wen Jie Lau, j.wenjie93@hotmail.com

• KEY: Labs generally do not make or break a diagnosis of autoimmune disease!

• ANA: Anti-nuclear antibody

• Measured by immunofluorescence microscopy; reported as a titer

• If negative, chance of CTDs (except RA) virtually nil

Wen Jie Lau, j.wenjie93@hotmail.com

• dsDNA: anti-double-stranded DNA antibody

• Titers can be used for monitoring disease activity in some patients.

• SS-B/La: seen in Sjögren’s syndrome

• RNP (ribonucleoprotein): mixed connective tissue disease (combination of lupus,

Wen Jie Lau, j.wenjie93@hotmail.com

• Anti Scl-70: seen in systemic sclerosis (scleroderma) with increased risk of

• Anti-centromere: seen in CREST (scleroderma)

• Anti-histone: seen in DIL (drug-induced lupus)

Anti-Jo-1 Ab: seen in anti-synthetase syndrome (polymyositis + interstitial lung disease)

Wen Jie Lau, j.wenjie93@hotmail.com

• Anti-CCP Ab: CCP is cyclic citrullinated peptide, a derivative of arginine

CRP: C-reactive protein

• Varies more acutely with level of inflammation

Wen Jie Lau, j.wenjie93@hotmail.com

ESR: Erythrocyte sedimentation rate

• False elevation seen in severe anemia

• False decrease in advanced liver disease, nephrotic syndrome, protein-losing

Wen Jie Lau, j.wenjie93@hotmail.com

Wen Jie Lau, j.wenjie93@hotmail.com

Rheumatoid arthritis is a chronic, symmetric, polyarticular,

Causes a polyarticular, symmetrical arthritis with extra articular

Wen Jie Lau, j.wenjie93@hotmail.com

Wen Jie Lau, j.wenjie93@hotmail.com

Wen Jie Lau, j.wenjie93@hotmail.com

As the disease progresses, joint destruction occurs resulting in

• Swan-neck deformity of the fingers

• Boutonniere deformity of the fingers

• Atlanto-axial (C1–C2) subluxation may cause spinal cord injury

Signs & symptoms AM stiffness

Wen Jie Lau, j.wenjie93@hotmail.com

Extra articular manifestations

• Pulmonary: pulmonary nodule, diffuse interstitial fibrosis,

• Cardiac: pericardial effusion, pericarditis, aortitis, increased

• Rheumatoid vasculitis (skin, nerve, internal organs)

• Overlap with SLE (lupus), Sjögren’s syndrome (eye sicca)

Wen Jie Lau, j.wenjie93@hotmail.com

2. Arthritis > 3 joints simultaneously for 6 weeks

3. Hand joint arthritis (MCPs, PIPs) for 6 weeks

4. Symmetric joint involvement for 6 weeks

6. Positive rheumatoid factor

7. Radiographic changes of joint erosions

Remember differences between RA and OA

Wen Jie Lau, j.wenjie93@hotmail.com

• NSAIDs are the first line treatment for patients with RA

• Glucocorticoids are necessary for many patients with

• Their use should be minimized, if possible , to avoid the side-

Wen Jie Lau, j.wenjie93@hotmail.com

• Goal is to prevent flares with DMARDs or biologic agents and

• Examples of DMARDs include: methotrexate, sulfasalazine,

• Examples of biologic agents include: etanercept (Enbrel),

Wen Jie Lau, j.wenjie93@hotmail.com

Wen Jie Lau, j.wenjie93@hotmail.com

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