Rheumatology Basics Quiz

Rheumatology Overview

• Batterjee Medical College, a center for science and technology, provides a rheumatology course taught by Dr. Mohamed Roshdi, MD, Assistant Professor of Internal Medicine.

Learning Objectives

• Students will learn the pathophysiology, etiology, epidemiology, manifestations, complications, and management of rheumatological and collagen disorders.
• Students will develop skills to interpret clinical manifestations and investigations of rheumatological and collagen disorders to formulate reasonable diagnoses.
• Students will apply evidence-based management plans for rheumatological and collagen disorders, and distinguish patients in emergency situations to formulate comprehensive management plans, maintaining homeostasis.

Rheumatoid Arthritis: Definition and Etiology

• Rheumatoid arthritis is a connective tissue disorder.
• It's characterized by articular and extra-articular manifestations, suspected to be autoimmune related.
• The precise etiology is uncertain, but it's likely autoimmune.
• Synovial membrane infiltration with lymphocytes and plasma cells is suggested.
• Environmental and genetic factors may contribute, including a positive family history and HLA association (HLADr4).

Rheumatoid Arthritis: Pathology

• I. Articular:
  • Synovitis: Thickening, inflammation, and infiltration of the synovium with lymphocytes and plasma cells.
  • Pannus formation: Erodes and destroys articular cartilage.
  • Cartilage erosion and destruction lead to subluxation and deformity.
• II. Extra-articular:
  • Rheumatoid nodules (in 20% of cases): Fibrinoid degeneration with inflammatory cells surrounded by a fibrous capsule.
  • Exclusive to seropositive patients.

Rheumatoid Arthritis: Stages

• The presentation of rheumatoid arthritis progresses through stages, with synovitis being the first stage, followed by pannus formation, fibrosis, and ultimately bony ankylosis.

Rheumatoid Arthritis: Mode of Onset and Clinical Picture

• Mode of Onset:
  • Gradual (months): 70%
  • Intermediate (weeks): 20%
  • Acute (days): 10% -Typically begins as gradual polyarthritis but can start as monoarthritis.
• Clinical Picture:
  • 1-3% of the population affected.
  • Predominantly affects females (3:1 ratio).
  • Includes general symptoms like fever, fatigue, and morning stiffness.

Rheumatoid Arthritis: Articular Manifestations

• Affects multiple joints; primarily peripheral joints (upper and lower limbs) more than central joints.
• Focuses on smaller joints (e.g., hands, feet, wrist).
• Distal interphalangeal joints are usually spared.
• Characterized by redness, hotness, swelling, tenderness, and limited movement.

Rheumatoid Arthritis: Deformities

• Hand deformities (boutonniere, swan-neck, ulnar deviation, Z-shaped thumb).
• Carpal tunnel syndrome.
• Cricoarytenoid (stridor and hoarseness of voice).
• Atlanto-axial subluxation (spinal cord compression potentially leading to paraplegia).
• Tendon rupture (extensors more frequently than flexors, as seen in the hip and knee).
• Baker cyst (popliteal cyst).

Rheumatoid Arthritis: Diagnostic Criteria

• Patients are diagnosed with RA if they exhibit at least 4 of the 7 criteria.
• Symptoms must persist for at least 6 weeks, including morning stiffness (over 1 hour), arthritis in at least 3+ joints and hand joints, symmetrical arthritis, rheumatoid nodules, characteristic X-ray findings, and a positive rheumatoid factor.

Rheumatoid Arthritis: Investigations

• X-ray: Osteoporosis, joint space narrowing, and deformity.
• Chest X-ray: Pleural effusions.
• Synovial fluid aspiration: Antibody detection.
• Blood tests: Anemia (normochromic and hypochromic), elevated white blood cell count (potentially decreased in Felty's syndrome), elevated ESR (potentially over 100 in severe cases), and elevated CRP
• Serological tests: positive rheumatoid factor in approximately 80% of cases. Less frequently positive for anti-nuclear antibodies (ANA) and lupus erythematosus (LE) cells at diagnosis. Positive for Anti CCP.

Rheumatoid Arthritis: Treatment

• General Treatment:
  • Rest, especially during acute stages.
  • Splinting for pain management and to prevent deformities.
  • Physiotherapy to maintain joint function once the acute phase subsides.
• Medical Treatment:
  • NSAIDs: Aspirin, diclofenac, paracetamol, indomethacin, and ketoprofen to relieve pain and inflammation. Mechanisms and Side Effects are presented.
• Cortisone: Small doses for RA, potentially high doses for extra-articular manifestations. Side effects of cortisone are presented.
• DMARDs: Slow-acting medications used to modify RA and reduce disease progression: Gold salts, Penicillamine, Chloroquine. Side effects are presented.
• Immunosuppressive Drugs (MAC): Methotrexate, azathioprine, cyclophosphamide for severe/resistant cases.
• Intra-articular injections of cortisone: To treat inflammation of tendons.
• Surgical treatment: Synovectomy, arthrodesis, and joint replacement for severe cases.

Systemic Lupus Erythematosus (SLE): Definition and Etiology

• Systemic Lupus Erythematosus (SLE) is a chronic systemic autoimmune disease with systemic and articular manifestations.
• It involves multiple organs and systems in the body.
• The cause remains questionable, but autoimmune mechanisms potentially play a key role, causing a T-suppressor lymphocyte dysfunction, an increase in autoantibodies and immune complexes, in turn affecting multiple tissues/organs.
• Genetic factors, environmental elements (sunlight exposure, infections), and drug-induced lupus (e.g., hydralazine, INH, penicillamine, phenytoin) are possible contributing factors.
• Hormonal factors (e.g., estrogen) may contribute, particularly in the childbearing years.

SLE: Pathology

• I. Articular: Synovitis is typically non-erosive, with no cartilage loss.
• II. Extra-articular: Immune complex deposition in various tissues and organs: skin, Kidney (glomeruli, heart, lungs, CNS.

SLE: Clinical Presentation

• I. General: Fever, fatigue, and sweating.
• II. Skin:
  • Malar rash (across cheeks).
  • Butterfly rash (on cheeks and bridge of nose).
  • Discoid rash (scaling, scarring on face).
  • Photosensitivity.
  • Alopecia.
  • Vasculitis (purpura, leg ulcers).
  • Raynaud's phenomenon (poor circulation, cold fingers).
• Other systems affected: Renal, Cardiac, Chest, CNS, Eye, GIT, Blood are presented

SLE: Diagnostic Criteria

• The American Rheumatism Association (ARA) has defined 11 criteria for diagnosing SLE, although the presence of four or more symptoms strongly suggests the condition.
• These criteria include the presence of a malar rash, discoid rash, photosensitivity, oral ulcers, non-erosive arthritis, proteinuria, pleurisy or pericarditis, neurological symptoms, Peripheral arthritis, presence of Anti-nuclear antibodies (ANA) and anti-DNA antibodies (anti-smith).

SLE: Treatment

• There is no cure.
• General prophylactic measures: Rest, protective clothing/sunglasses/sunscreen avoidance of drugs that trigger SLE. Psychological treatment.
• Medication: NSAIDs, Cortisone, Topical cortisone for skin rash, Hydroxychloroquine, Immunosuppressive drugs.
• Plasmapheresis: For severe cases involving brain or kidney.
• Treatment of complications: e.g., spleen removal for thrombocytopenia; dialysis or transplant for end-stage kidney disease.

Progressive Systemic Sclerosis (Scleroderma): Definition and Pathology

• Progressive Systemic Sclerosis (Scleroderma) is a connective tissue disorder characterized by fibrosis and thickening of the skin and related damage to blood vessels and internal organs.
• Pathology: Characterized by collagen deposition throughout the body and vasculitis (inflammation of blood vessels and potentially associated organ dysfunction)

Scleroderma: Clinical Picture

• Skin: Early edema, followed by progressive thickening and fibrosis/induration; the skin loses its elasticity. Skin discoloration (hyperpigmentation/hypopigmentation), ulceration, and calcification.
• GIT: Can lead to dysphagia/reflux/malabsorption. Constipation also commonly impacted.
• Renal: Vasculitis and renal crisis can be life-threatening.
• Other systems: Cardiac, Chest, CNS are presented

Scleroderma: Treatment

• Similar to SLE. No cure exists, and treatment is symptom-focused and treatment of associated organ problems. Treatment is mostly supportive.

Osteoarthritis: Definition and Classification

• Osteoarthritis is a degenerative joint disease, characterized by cartilage breakdown and formation of osteophytes.
• It is not an inflammatory disease unlike rheumatoid arthritis.
• Classification:
  • Primary: Unknown etiology. Genetic factors may play a role.
  • Secondary: Related to mechanical stress (occupational or sport related injuries), existing joint problems (RA, septic arthritis, Gout), and endocrine conditions (hyperparathyroidism, diabetes, acromegaly).

Osteoarthritis: Risk Factors and Clinical Picture

• Risk factors: Aging, positive family history, sex (more common in females), obesity, joint overuse.
• Clinical picture:
  • Pain (worsening with activity, better with rest).
  • Stiffness.
  • Crepitus (grating sensation on movement).
  • Minimal joint deformity and muscle wasting.
  • No systemic manifestations.
• Common joint involvement: knee, hip, spinal (cervical and lumbar), distal and proximal interphalangeal joints, first carpometacarpal joint, first metatarsophalangeal joint.

Osteoarthritis: Investigations and Treatment

• Investigations: Normal blood tests, joint space narrowing, osteophytes, subchondral sclerosis in X-rays.
• Treatment: Exercise, physiotherapy, short course of analgesics (NSAIDs), intra-articular steroid injections, and surgery (in severe cases).

References

• The provided slides include references to Step-up to Medicine and Davidson's Principles and Practice of Medicine.

Test your knowledge on essential skills and management strategies for rheumatological and collagen disorders. This quiz covers key aspects such as emergency management, understanding pathophysiology, and evidence-based practices. Perfect for healthcare professionals looking to enhance their expertise in rheumatology.