Rheumatology Quiz: Systemic Sclerosis and Related Disorders
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Questions and Answers

What is the most common cause of death in patients with Systemic Sclerosis?

  • Vasculitis and resultant disease (correct)
  • Pulmonary fibrosis
  • Renal crisis
  • Cardiac failure

    • Which of the following is a characteristic of CREST syndrome, a variant of Systemic Sclerosis?

  • Subcutaneous calcinosis (correct)
  • Raynaud's phenomenon
  • Heliotrope rash
  • Esophageal dysmotility

    • What is a common feature of Dermatomyositis/Polymyositis (DM-PM)?

  • Muscle hypertrophy
  • Proximal limb weakness (correct)
  • Distal limb weakness
  • Skeletal muscle atrophy

    • What is the characteristic rash of Dermatomyositis?

    <p>Heliotrope rash</p> Signup and view all the answers

    What is the main difference between Primary, Idiopathic DM and Primary, Idiopathic PM?

    <p>Presence of rash</p> Signup and view all the answers

    What is a common complication of Systemic Sclerosis?

    <p>All of the above</p> Signup and view all the answers

    What is the etiology of Dermatomyositis/Polymyositis (DM-PM)?

    <p>Unknown, but suspected to be related to abnormal immunoregulation and viral causes</p> Signup and view all the answers

    Which of the following is a subgroup of Dermatomyositis/Polymyositis (DM-PM)?

    <p>DM/PM associated with malignancy</p> Signup and view all the answers

    What is the 2nd form of joint disease seen in SLE?

    <p>Avascular necrosis</p> Signup and view all the answers

    Which of the following is a systemic manifestation of SLE?

    <p>Renal failure</p> Signup and view all the answers

    What is the epidemiology of SLE in terms of age of onset?

    <p>Onset at any age, but often between 15-25 years old</p> Signup and view all the answers

    What is the characteristic skin rash seen in SLE?

    <p>Malar rash</p> Signup and view all the answers

    What is the diagnostic criteria for Sjogren's disease?

    <p>Ocular symptoms, oral symptoms, ocular signs, and histopathology</p> Signup and view all the answers

    What is the primary symptom of Sjogren's disease?

    <p>Xerostomia</p> Signup and view all the answers

    What is the characteristic extraglandular manifestation of Sjogren's disease?

    <p>All of the above</p> Signup and view all the answers

    What is the type of connective tissue disorder that Sjogren's disease is classified as?

    <p>Connective tissue disorder affecting the exocrine glands</p> Signup and view all the answers

    Which of the following autoimmune diseases is an organ-specific disease?

    <p>Grave's disease</p> Signup and view all the answers

    What is a characteristic of systemic autoimmune diseases?

    <p>Female predisposition</p> Signup and view all the answers

    Which of the following is a systemic autoimmune disease?

    <p>Sjogren's Syndrome</p> Signup and view all the answers

    What is a characteristic of Systemic Sclerosis?

    <p>Rapid progression and symmetric skin thickening in proximal and distal limbs, face and trunk</p> Signup and view all the answers

    What is a common feature of autoimmune diseases?

    <p>Presence of autoantibodies</p> Signup and view all the answers

    What is a risk factor for developing Systemic Sclerosis?

    <p>Pregnancy</p> Signup and view all the answers

    Study Notes

    Progressive Systemic Scleroderma (PSS)

    • Infiltration of muscle leads to weakness
    • Kidneys affected by vasculitis, resulting in high mortality rate
    • May also have Raynaud's phenomenon
    • CREST syndrome variant: subcutaneous calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia

    Clinical Features of PSS

    • Raynaud's phenomenon and esophageal dysmotility
    • Skin thickening and pulmonary fibrosis
    • Subcutaneous calcinosis and isolated pulmonary arterial hypertension
    • Telangiectasia and congestive heart failure
    • Arthralgia and arthritis, and renal crisis
    • Myopathy

    Dermatomyositis/Polymyositis (DM-PM)

    • Inflammation of muscles with or without rashes
    • Etiology unknown, but abnormal immunoregulation and viral causes suspected
    • Autoimmune disease
    • Polymyositis: skeletal muscle damaged by non-suppurative inflammatory process characterized by lymphocyte infiltration
    • Dermatomyositis: polymyositis plus skin rash (diffuse erythema, maculopapular eruption, dermatitis)
    • Insidious onset
    • Findings: proximal limb weakness, pain in buttocks and thigh, heliotrope rash, itching, periorbital edema, and subcutaneous calcification

    5 Subtypes of DM-PM

    • I: Primary, idiopathic PM
    • II: Primary, idiopathic DM
    • III: DM/PM associated with malignancy or neoplasia
    • IV: DM/PM of childhood associated with vasculitis
    • V: DM/PM associated with other collagen vascular disease (SLE, RA, PSS)

    Group I: Primary, Idiopathic PM

    • Insidious onset
    • Begins with pelvic girdle weakness, later involving shoulder girdle
    • Moderate arthritis, Raynaud's disease, dysphagia, and dysphonia may be present
    • Remission and exacerbations common

    Group II: Primary, Idiopathic DM

    • Acute onset with proximal shoulder and pelvic girdle involvement
    • Erythematous heliotropic rash on the skin of the eyelids and dorsum of the hands

    Autoimmune Diseases

    • Condition in which host immune response against self-components contributes to pathogenesis
    • Two types: organ-specific (antibodies attack only cells of parts of particular organ) and systemic (various organ cells can be attacked)
    • Examples: Multiple sclerosis, Insulin dependent DM, Myasthenia Gravis, Grave's disease, Addison's disease, Rheumatoid arthritis, SLE, Sjogren's, Scleroderma, Dermatomyositis, Polymyositis, Mixed CTD

    Common Characteristics of Autoimmune Diseases

    • Autoantibodies-markers (ANA, rheumatoid factor)
    • Often associated with genetic and viral and environmental factors
    • Female predisposition

    Systemic Sclerosis

    • Diffuse cutaneous scleroderma: rapid progression, symmetric skin thickening in proximal and distal limbs, face, and trunk, visceral involvement likely
    • Localized cutaneous scleroderma: skin thickening limited to distal limbs and face, often has features of CREST syndrome

    Description

    • Epidemiology: rare, 30-40 y/o, F>M (3:1), increased risk: blacks, pregnancy, stone masonry, coal/gold/uranium exposure
    • In PSS, excessive amounts of abnormal collagen cause thickening of the skin and difficulty in moving joints
    • Fibrosis affects GIT motility, lung air exchange, pericardial expansion, and motion, resulting in associated systemic manifestations

    Other Connective Tissue Diseases

    • SLE: 2nd form of joint disease seen in SLE, avascular necrosis frequently in knees, hips, and shoulders
    • Pain relief in avascular necrosis mainly by reducing weightbearing
    • Patellar and Achilles tendon ruptures may occur
    • Systemic manifestations include: wolf-like skin rash, renal failure, central & peripheral nervous system abnormalities, hematologic problems, systemic cutaneous vasculitis, pleurisy

    SLE

    • Epidemiology: onset any age, but often 15-25 y/o, F>M (3:1 to 8:1), blacks, Asians
    • Manifestations: systemic, musculoskeletal, cutaneous, hematologic, neurologic, cardiopulmonary, renal, GIT, ocular, and fetal loss

    1982 Criteria for SLE

    • Malar rash, renal disorder, discoid rash, neurologic disorder, photosensitivity, hematologic disorder, oral ulcers, immunologic disorder, arthritis, serositis, antinuclear antibodies

    Sjogren's Disease

    • Manifestations: xerostomia, dry eyes, dry nose, throat, and trachea, esophageal mucosal atrophy, gastritis, pancreatitis, dyspareunia, and extraglandular manifestations (rare)
    • Diagnostic criteria: ocular symptoms, oral symptoms, ocular signs, histopathology, and salivary gland
    • Criteria definitions: dry eyes, oral symptoms, Schirmer's test, rose Bengal score, and focus score

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    GENERAL-MEDICAL-CONDITIONS-1.docx

    Description

    Test your knowledge of Systemic Sclerosis, CREST syndrome, Dermatomyositis, and Polymyositis. This quiz covers the causes of death, characteristic features, and common complications of these rheumatological disorders.

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