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Questions and Answers
What is the most common cause of death in patients with Systemic Sclerosis?
Which of the following is a characteristic of CREST syndrome, a variant of Systemic Sclerosis?
What is a common feature of Dermatomyositis/Polymyositis (DM-PM)?
What is the characteristic rash of Dermatomyositis?
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What is the main difference between Primary, Idiopathic DM and Primary, Idiopathic PM?
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What is a common complication of Systemic Sclerosis?
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What is the etiology of Dermatomyositis/Polymyositis (DM-PM)?
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Which of the following is a subgroup of Dermatomyositis/Polymyositis (DM-PM)?
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What is the 2nd form of joint disease seen in SLE?
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Which of the following is a systemic manifestation of SLE?
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What is the epidemiology of SLE in terms of age of onset?
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What is the characteristic skin rash seen in SLE?
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What is the diagnostic criteria for Sjogren's disease?
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What is the primary symptom of Sjogren's disease?
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What is the characteristic extraglandular manifestation of Sjogren's disease?
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What is the type of connective tissue disorder that Sjogren's disease is classified as?
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Which of the following autoimmune diseases is an organ-specific disease?
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What is a characteristic of systemic autoimmune diseases?
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Which of the following is a systemic autoimmune disease?
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What is a characteristic of Systemic Sclerosis?
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What is a common feature of autoimmune diseases?
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What is a risk factor for developing Systemic Sclerosis?
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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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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.
