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Which characteristic feature is associated with Sarcoidosis?
Behcet's disease is known for asymmetrical non-erosive oligoarthritis.
False
What syndrome is characterized by mouth and genital ulcers with inflamed cartilage?
MAGIC Syndrome
_____ is an acute febrile neutrophilic dermatosis seen only in Behcet's.
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Match the following conditions with their associated characteristics:
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Which marker is considered the best for diagnosing preclinical infection in rheumatoid arthritis?
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A high rheumatoid factor suggests the absence of extra articular manifestations.
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What is the predicted risk percentage score that indicates greater than 80% chance to progress to rheumatoid arthritis if the score is 28?
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In chronic inflammatory symmetric peripheral polyarthritis, the most common presentation is _____ (percentage range).
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Match the following markers with their specificity:
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Which genetic factor is associated with an increased risk of developing rheumatoid arthritis?
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Activated T-cells migrate to the synovium and subsequently activate macrophages.
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What is the role of TNF-α in the pathogenesis of rheumatoid arthritis?
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The inflamed tissue that damages cartilage in rheumatoid arthritis is called ___.
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Match the following terms to their descriptions:
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What is the minimum duration of symptoms needed to diagnose rheumatoid arthritis (RA)?
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The distal interphalangeal (DIP) joints are typically involved in rheumatoid arthritis.
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What is the most common extra-articular manifestation in rheumatoid arthritis patients?
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Rheumatoid arthritis is classified as a __________ disease due to its autoimmune nature.
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Match the following types of arthritis with their distinct joint involvement:
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Which environmental factor is considered the strongest risk factor for rheumatoid arthritis?
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Females are affected by rheumatoid arthritis three times more than males.
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What chronic infection is associated with rheumatoid arthritis?
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The age group most commonly affected by rheumatoid arthritis is _____ years old.
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Match the following risk factors with their classification in rheumatoid arthritis:
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What is the primary treatment for severe mucocutaneous manifestations in a patient with Cogan's Syndrome?
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Cogan's Syndrome is typically diagnosed in individuals aged 40-50 years.
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Name one type of skin lesion found in Cogan's Syndrome.
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In the treatment of severe systemic manifestations, __________ is used along with methotrexate or mycophenolate mofetil.
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Match the following aspects of Cogan's Syndrome with their details:
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Which type of arthritis primarily affects the spine and shoulders?
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All types of inflammatory arthritis can cause joint erosions.
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Name one example of a peripheral predominant spondyloarthropathy.
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The predominant joint involvement in rheumatoid arthritis is the ___________ joints.
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Match the following types of arthritis with their characteristics:
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What condition is characterized by acute episodic monoarthritis and often affects young girls?
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Elevated ESR levels are associated with elderly rheumatoid arthritis.
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What is one common deformity observed in rheumatoid arthritis patients involving the wrist and fingers?
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The incidence of palindromic rheumatism is such that _____ of individuals may progress to rheumatoid arthritis.
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Match the following deformities with their descriptions:
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Which of the following conditions is characterized by non-inflammatory arthropathy?
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Acute arthritis symptoms worsen with activity and improve with rest.
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What is the best test to differentiate between inflammatory and non-inflammatory types of arthritis?
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Rheumatoid Arthritis primarily affects the _____, whereas Osteoarthritis primarily affects the _____ cartilage.
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Match the following features with their corresponding types of arthritis:
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Which of the following describes the Swan Neck Deformity?
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The Boutonniere Deformity involves hyperextension of the PIP joint.
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What are the characteristics of the Intrinsic Plus Deformity?
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The ____ deformity is characterized by arthritis mutilans.
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Match each deformity with its main characteristic:
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Study Notes
Rheumatology and Immunology
- Active space: A scoring system used to predict the risk of progression to rheumatoid arthritis (RA). A score of 28 indicates an over 80% chance of progressing to RA.
- Anti-CCP (Cyclic citrullinated polypeptide) antibody: A highly specific marker for pre-clinical rheumatoid arthritis. A high titer suggests extra-articular manifestations.
- Rheumatoid factor: A less specific marker of pre-clinical rheumatoid arthritis. Can be seen in other conditions like Sjogren's syndrome and infections.
- CRP and ESR: Useful for monitoring relapse in rheumatoid arthritis.
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Ophthalmic manifestations:
- Sarcoidosis: Chronic relapsing bilateral panuveitis, retinal vasculitis. Anterior chamber (AC) involvement is more prominent than posterior chamber (PC) involvement.
- Behcet's disease: Retinal vasculitis, hypopyon. Posterior chamber (PC) involvement is more prominent than anterior chamber (AC) involvement.
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Joint manifestations:
- Rheumatoid arthritis: Chronic inflammatory symmetric peripheral polyarthritis, typically involving small joints of the upper limb.
- Behcet's disease: Asymmetrical non-erosive oligoarthritis.
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Warning signs in rheumatoid arthritis:
- Vascular thrombosis (venous > arterial)
- Gastrointestinal involvement
- Central nervous system involvement, including vascular, parenchymal, and brainstem encephalitis.
- Sweet syndrome: An acute febrile neutrophilic dermatosis, only seen in Behcet's disease.
- MAGIC syndrome: A combination of Behcet's disease and relapsing polychondritis, characterized by mouth and genital ulcers with inflamed cartilage.
Etiopathogenesis of Rheumatoid Arthritis
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Genetic and environmental factors:
- Genetic factors lead to abnormal protein modifications (e.g., citrullination, carbamylation).
- These modifications are detected as antigens.
- Dendritic cells (DC) and T-cell interaction: DCs process antigens and present them to T-cells, activating them.
- T-cell activation: Activated T-cells migrate to the synovium and activate macrophages.
- TNF-α production: Activated macrophages produce TNF-α, which causes bone resorption by activating osteoclasts and inhibiting osteoblasts.
Etiology of Rheumatoid Arthritis
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HLA factors:
- DRB1-04: Associated with a 5-fold increased risk of rheumatoid arthritis.
- DR-13: Associated with protection.
- DR-1: Unknown association.
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Non-HLA factors:
- PADI-4: Post-translational modification of arginine to citrulline, associated with rheumatoid arthritis in Asian populations.
- PTPN22: Not associated with rheumatoid arthritis in Asian populations.
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Environmental factors:
- Smoking: Strongest environmental risk factor for rheumatoid arthritis. Increases PADI-4 expression in the airway.
- Silica: Primarily associated with systemic sclerosis.
- Alcohol and OCP (Oral Contraceptive Pills): Protective.
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Other factors:
- Gender: Females are affected 3 times more than males.
- Age: 40-60 years old.
- Pregnancy: Increases IL-10, inducing remission.
- Infection: Porphyromonas gingivalis (chronic periodontitis).
Clinical Manifestations of Rheumatoid Arthritis
- Synovial hypertrophy: Increase in size of the synovial membrane.
- Pannus: Inflamed tissue that forms, damaging cartilage.
- Periarticular erosion/osteopenia: Breakdown of bone surrounding the joint.
Rheumatoid Arthritis
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Types based on duration of symptoms:
- ≥6 weeks: Rheumatoid arthritis (RA).
- 0-3 months: Very early rheumatoid arthritis (VERA).
- 3 months - 1 year: Early established rheumatoid arthritis.
- 1-2 years: Late established rheumatoid arthritis.
- >2 years: Chronic stabilized rheumatoid arthritis.
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Features:
- Chronic inflammatory symmetric peripheral polyarthritis (CISPP).
- Involvement of small joints of the upper limb.
Rheumatoid Arthritis (Specific Joint Involvement)
- Joints involved: Proximal interphalangeal (PIP), metacarpophalangeal (MCP), elbow, G-Ca spine, 5th metatarsophalangeal (MTP), knee, temporomandibular joint (TMJ), cricoarytenoid joint.
- Joints spared: Distal interphalangeal (DIP), 1st carpometacarpal (cmc), 1st metatarsophalangeal (MTP).
Rheumatoid Arthritis (Non-HLA factors and Environmental factors)
- PADI-4: Involved in post-translational modification of arginine to citrulline, seen in Asian populations.
- PTPN22: Not seen in Asian populations.
- Smoking: Strongest environmental risk factor. Increases PADI-4 expression in the airway and risk of ILD (interstitial lung disease), associated with a worse prognosis.
- Silica: Primarily associated with systemic sclerosis.
- Alcohol and OCP: Protective.
Rheumatoid Arthritis (Other factors and Risk Prediction)
- Gender: Females affected 3 times more than males.
- Presentation in males: Most common presentations in males are ILD (interstitial lung disease) and vasculitis.
- Age: 40-60 years old.
- Pregnancy: Increases IL-10, inducing remission.
- Infection: Porphyromonas gingivalis (chronic periodontitis)
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Risk prediction:
- 1/3rd of patients achieve remission.
- 1/3rd persist with symptoms or progress to other diseases.
- 1/3rd evolve to RA.
Rheumatoid Arthritis (518)
- Diagnosis: Clinical diagnosis based on presentation, skin lesions, and immunoglobulins.
- Skin lesions: Predominantly neutrophilic, cutaneous leukocytoclastic vasculitis, mixed infiltrate (IgG/IgM/C3).
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Treatment: Varies based on severity:
- Mucocutaneous: Topical steroid + PGE₂ gel.
- Severe mucocutaneous: Thalidomide + methotrexate + topical steroid.
- Systemic: Oral steroid (prednisolone) + methotrexate/mycophenolate mofetil (MMF).
Cogans Syndrome
- Age group: 20-30 years old.
- Characteristic triad:
- Aortitis
- Interstitial keratitis
- Audiovestibular syndrome
Irreversible Deformities in Rheumatoid Arthritis
- Swan neck deformity: Hyperextended PIP, flexion of DIP, rupture of flexor digitalis superficialis (FDS) tendon.
- Boutonniere deformity: Flexion of PIP, hyperextension of DIP, synovitis of PIP, subluxation of lateral band, intact extensor index tendon.
- Opera glass hand deformity: Arthritis mutilans.
- Vaughan-Jackson syndrome: Impaired medial extensor tendons.
- Intrinsic plus deformity: Tightness of intrinsic muscles, hyperflexion of MCP, extension of interphalangeal joints.
Rheumatoid Arthritis in the Elderly
- Acute destructive polyarthritis: Painful erosions, mimicking polymyalgia rheumatica (PMR).
- Pain and stiffness: In the shoulder and pelvic girdle.
- Elevated ESR: Increased erythrocyte sedimentation rate.
- Periarticular involvement: Enthesitis, bursitis.
- Arthritis robustus: Minimal symptoms.
- X-ray findings: Erosions.
Palindromic Rheumatism
- Primarily affects young girls.
- Acute episodic monoarthritis: Pain and swelling of a single joint followed by complete resolution and involvement of other joints.
- Anti-CARP (carbamylated peptide) antibody positive.
- 30% progress to RA.
Deformities in Rheumatoid Arthritis
- Synovitis: Inflammatory process affecting the synovium.
- Tendinitis: Inflammation of tendons.
- Bursitis: Inflammation of bursae.
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Reversible deformities:
- 2 zig-zag deformity: Wrist radial deviation, MCP ulnar deviation, subluxation, interphalangeal joints extended.
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Specific deformities:
- Synovitis of MCP joint: Redness and inflammation of the MCP joint.
- Rheumatoid hand: Ulnar deviation.
- Piano key deformity: Rupture of the ulnar collateral ligament (UCL), and deformity of the ulnar styloid.
- Hitchhiker thumb: Two-shaped deformity, abduction, and hyperextension of the thumb.
- Trigger finger: Tenosynovitis.
Types of Arthritis Based on Joints Involved
- Axial predominant: Ankylosing spondylitis → Spine + shoulder, hip (root joints).
- Peripheral predominant: RA → Peripheral joints + C-spine.
- Peripheral predominant spondyloarthropathies: Reactive arthritis, psoriatic arthritis.
Approach to Inflammatory Arthritis
- Acute: Post viral (parvo B19), undifferentiated arthritis.
- Chronic: Undifferentiated arthritis, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) & SLE-like arthritis/arthralgia, psoriatic arthritis, osteoarthritis (OA) hand (inflammatory), sarcoidosis.
- Septic arthritis.
- Monoarthritis: Acute crystal arthropathy (Gout, Pseudogout).
Erosive vs Non-erosive Arthritis
- Erosive arthritis: Rheumatoid arthritis (RA), psoriatic arthritis, chronic crystal arthropathy, connective tissue disorders (CTDs) (Mixed CTDs, sarcoidosis, multicentric reticulohistiocytosis, Rhupus arthritis).
- Non-erosive arthritis: SLE (Systemic Lupus Erythematosus) & SLE-like arthritis, acute rheumatic fever (Jaccoud's arthropathy), relapsing polychondritis, Behcet's disease, acute crystal arthropathies.
Differentiating Features in Arthritis
- Articular vs. periarticular: Involves the joint or surrounding tissues (e.g., tendinitis, bursitis).
- Inflammatory vs. non-inflammatory: RA is inflammatory, osteoarthritis is non-inflammatory.
- Axial vs. peripheral predominant arthropathy: Primarily affects the spine or peripheral joints respectively.
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Acute vs. chronic: Acute arthritis is typically characterized by intense pain that worsens with rest and improves with activity.
- Lab findings: ↑ESR, ↑CRP.
- X-ray: May show erosions or deformities.
- Synovial fluid study: White blood cell count >2000 cells/μL helps differentiate inflammatory from non-inflammatory arthritis.
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Description
Test your knowledge on key concepts in Rheumatology and Immunology, including disease markers, scoring systems, and ophthalmic manifestations associated with autoimmune conditions. This quiz covers important aspects like rheumatoid arthritis and related diseases.
