Connective Tissue Diseases

Question 1

CTDs (general)

Answer 1

A group of systemic autoimmune rheumatic diseases May be associated with arthropathy, but the hallmarks of these diseases are multiorgan and systemic involvement, and autoimmunity

Question 2

Types of CTDs (5)

Answer 2

Systemic Lupus Erythematosus (SLE) Sjogren’s Syndrome Scleroderma - Progressive Systemic Sclerosis - Limited Scleroderma/CREST Inflammatory Myopathies - Polymyositis - Dermatomyositis Undifferentiated CTD

Question 3

SLE

• Patho
• Labs

Answer 3

Systemic Lupus Erythematosus

Patho:

• • unknown etiology
• • MC in women
• • genetic predisposition
• • antigen-antibody complexes with with complement binding and immune complex deposition in skin and glomeruli

Labs:

• Antibody Antinuclear Antibodies (ANA)
• antibodies that bind to nuclear antigens; seen in most CTDs; sensitive but not specific
• Anti-ds-DNA (ds-DNA):
• correlates with renal disease and disease activity; specific to SLE
• Complements:
• serum proteins involved with immune response that are abnormally low
• Extractable Nuclear Antigens (ENA):
• complete panel that allows delineation of which CTD

*Use theses tests to help establish dx, determine dz activity, assess organ involvement, monitor therapeutic response. Pt must have PE significant for dz too.

Question 4

SLE Classification Criteria (11)

Answer 4

• Malar rash
• discoid rash
• photosensitivity
• oral ulcers
• arthritis
  
  • arthralgias, myalgias, inflammatory polyarthritis (non-erosive, reversible joint deformity - Jaccoud’s arthropathy)
• serositis (pleuritis or pericarditis)
• Nephritis
  
  • can be variable: persistent proteinuria and cellular casts, dysmorphic RBC, dec GFR
• Neuro disorder
  
  • seizures, psychosis, cognitive deficits, depression, strokes, HA, aspetic meningitis
• Hematologic disorder
  
  • cytopenia, coagulopathy (may lead to thrombosis, antiphospholipid antibodies indicate this state)
• Positive ANA
• Other positive antibodies

Must have 4/11

Question 5

SLE Treatment (4)

Answer 5

based on manifestations and organ involvement

NSAIDs for m/s

Sun protection

Glucocorticoids–bridge therapy

Steroid sparing agents like Methotrexate or Hydroxychlorquine

Question 6

Sjogren’s Syndrome

• Patho
• Labs
• Signs/Sxs
• Dx
• Tx

Answer 6

Patho:

• lymphocytic infiltration of lacrimal and salivary glands
• primary SS - no other autoimmune disorders
• secondary SS - presence of other autoimmune disorders
• MC in females

Labs:

• +/- ANA
• • Rh

Signs/Sxs:

• xeropthalmia (Schirmer’s test)
• xerostomia (–>dental carries)
• parotid enlargement
• nonerosive systemic arthralgias
• fibromyalgia
• cardiopulmonary, neuro, renal, lymphadenopathy

Dx

• usually clinically
• salivary gland/parotid bx

Tx

• depends on systems involved
• eye drops, saliva substitutes (mostly here)
• NSAIDs
• hydroxychlorquine

Question 7

Scleroderma

• patho
• classification (2)
• clinical manifestations (6)
• treatment

Answer 7

Patho

• collagen deposition in skin, vessels, GI tract, renal, cardiopulmonary organs

Classification

• Limited Scleroderma/CREST
  
  • Calcinosis
  • Raynaud’s
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasias
  • Characterized by distal sclerosis
• Diffuse/Progressive Systemic Sclerosis (PSS)
  
  • proximal and distal sclerosis

Clinical Manifestations Common to both Classifications

• Raynaud’s
• Interstitial lung disease
• Pulmonary HTN
• Renal
• GI (reflux, dysphagia, decreased peristalsis, bacerial overgrowth
• M/S (sclerodactyly, acrosclerosis)

Treatment:

• Not effective
• PT/OT
• DMARDs

Question 8

Inflammatory Myopathies

• Patho
• Labs
• Diagnostic Criteria (5)
• Clinical Signs (other things you may see)
• Treatment

Answer 8

Patho

• Inflammatory immune mediated proximal muscle weakness of unknown etiology

Labs

• elevated muscle enzymes (CPK, aldolase, AST, ALT, LDH)

Diagnostic Criteria

• proximal muscle weakness
• elevated muscle enzymes
• myopathic changes on EMG
• muscle bx evidence of myositis
• skin rash

Clinical Signs

• Dysphagia
• Interstitial lung disease
• Arthralgias
• Raynaud’s phenomenon
• Skin manifestations of DM
  
  • heliotrope rash
  • **V or shawl sign **
  • Gottron’s papules

Treatment

• prednisone, MTX, PT
• follow muscle enzymes and strength

Question 9

Mixed CTD

Undifferentiated CTD

Overlap CTD

Answer 9

Mixed: has features of multiple CTDs

Undifferentiated CTD: lack a single unifying diagnosis but rather have features of several diseases

Overlap CTD: meet the criteria for 2 or more