Connective Tissue Disorders Flashcards
Sjogrens Syndrome (SS)
Chronic inflammatory disorder of the lacrimal and salivary glands.
Dry mouth, dry eyes
Mostly primary
Sicca complex
Dry eyes
Dry mouth
Sjogrens Epidemiology
All age groups
Women (9:1) in 4th and 5th decade
Sjogrens patho
Lymphocytic infiltrate into glands and impair function
Sjogrens extraglandular involvement
Vaginal dryness
Interstitial nephritis
Rhinitis, sinusitis
Dry cough
Antibodies for SS
Anti-Ro/SSA (best) and
Anti-La/SSB
Scleroderma
Thickened, hardened skin, commonly associated w/ systemic involvement
Cutaneous manifestations tx
Nothing can reverse the sclerosis.
Antihistamines for pruritis
Most common initial manifestation of scleroderma?`
Raynauds Phenomenon
Raynauds Phenomenon (RP)
Vasoconstriction from cold or emotional stress.
Can be white or blue
Tx for raynauds?
CCB
Prevention
Alpha blockers
low dose ASA
Systemic Sclerosis GI
GERD like sx
Tx with PPI
Systemic Sclerosis Musculoskeletal
Erosive, inflammatory arthritis
Methotrex to tx
Pulm manifestations of systemic sclerosis
Lung fibrosis common
Ground glass pattern on CT
Pulm HTN, lung CA
Cyclophosphamide to tx
Which meds should not be used for renal sx in systemic sclerosis pts?
DONT use diuretics
Use ACE
CREST
Calcinosis Raynauds Esophageal dysmotility Sclerodactyly Telangiectasia
CREST syndrome
Refers to limited cutaneous form of scleroderma
Pts may go on to develop pulm HTN and CHF
Polymyositis
Inflammatory muscle dz without a cutaneous eruption
Dermatomyocytis
Inflammatory muscle dx WITH cutaneous eruption.
Poly/Dermatomyositis
Inflammatory damage of myocytes leads to proximal muscle weakness (most common presenting feature)
Gottrons Sign
Rash over PIP and MCP joints
Poly/Dermatomyositis Eyelid rash
Purple heliotrope eruption over eyelids.
Shawl sign
Rash over upper anterior and around shoulders
Poly/Dermatomyositis S/S
Insidious onset of symmetric, proximal muscle weakness.
Myalgias and muscle tenderness
