Connective Tissue Disorders Flashcards
Connective Tissue Disorders
Sjogren's syndrome Scleroderma Raynaud's phenomenon Myositis/Dermatomyositis CREST syndrome
Define Sjogren’s Syndrome (SS)
Chronic inflammatory disorder characterized by diminished lacrimal & salivary gland function
Primary Sjogren’s Syndrome
Not associated with other disease
Secondary Sjogren’s Syndrome
Complication of other rheumatic conditions (RA, scleroderma, lupus)
Pathogenesis of Sjogren’s Syndrome
Lymphocytic infiltration into lacrimal & salivary glands, where lymphocytes are not normal found
Impairs function
Symptoms of Mild Sjogren’s Syndrome
Dry eyes Dry mouth Fatigue Myalgias Cognitive dysfunction
Symptoms of Severe Sjogren’s Syndrome
Florid salivary gland enlargement
Adenopathy
Antibodies to Ro/SSA & La/SSB antigens
Cryoglobulinemia
Hypocomplementemia
Propensity to develop non-Hodgkin’s lymphoma
Extraglandular Involvement in Sjogren’s Syndrome
Vaginal dryness Dyspareunia Interstitial nephritis Recurrent thirties & sinusitis Bronchial dryness
Define Karatoconjunctivitis Sicca
Deficiency in tear production leading to symptoms of dry eyes which usually presents insidiously overall several years
Complications of Karatoconjunctivitis Sicca
Corneal ulceration
Infection of the eyelids
Clinical Manifestations of Sjogren’s Syndrome
Karatoconjuncitivitis sicca
Xerostomia
Salivary gland enlargement
Complications of Xerostomia
Dental caries Oral candidiasis Laryngotracheal reflux Chronic esophagitis Weight loss Nocturia
Salivary Gland Enlargement in Sjogren’s Syndrome
Firm, diffuse, & non-tender
Occur in parotid & submandibular glands
Criteria for Diagnosis of Sjogren’s Syndrome
Ocular symptoms of inadequate tear production
Ocular signs of corneal damage
Oral symptoms of decreased saliva production
Salivary gland histopathology demonstrating foci of lymphocytes
Tests indicating impaired salivary gland function
Presence of autoantibodies (Anti-Ro/SSA & Anti-La/SSB)
Exclusion Criteria for Sjogren’s Syndrome
Prior head/neck irradiation Infection with hepatitis C AIDS Lymphoma Sarcoidosis Graft-vs-host disease Recent use of anti-cholinergic meds
Primary Sjogren’s Syndrome Diagnosis Criteria
Positive salivary gland biopsy or anti-Ro/SSA and/or anti-La/SSB & satifies 4/6 criteria
Satisfies 3/4 objective items (ocular signs, biopsy, salivary gland involvement, autoantibodies)
Secondary Sjogren’s Syndrome Diagnosis Criteria
CTD present
One item indicative of ocular or oral dryness
2/3 objective items
Diagnosing of Sjogren’s Syndrome
Thorough H&P
Schirmer test
Rose bengal test
Labs: CBC w/diff, CMP, ESR, UA
Specialized Lab Testing for Sjogren’s Syndrome
ANA RF Antibodies to Ro/SSA, La/SSB, Sm, & RNP antigens Antibody to anti-centromere B or topoisomerase ANCA (vasculitis) Serum C3 & C4 Antibodies to dsDNA Serum cryoglobulins SPE Ig levels
Diagnosis of Sjogren’s Syndrome
Salivary gland biopsy
Antibodies to Ro/SSA
Antibodies to La/SSB
Treatment of Sjogren’s Syndrome
Topical therapy for dry eyes & dry mouth
Treatment of systemic manifestations
3 Aspects of Treating Sicca Symptoms
General principles
Xerostomia
Karatoconjunctivities
General Treatment Principles of Sicca Symptoms
Avoidance of irritants Avoidance of exacerbating drugs Avoidance of low humidity atmospheres Consider candidiasis if a painful mouth Consider blepharitis if adequate hydration Excellent oral hygiene Topical moisturizers & lozenges
Treating Xerostomia
Stimulation of existing salivary flow: sugarless candy, dried fruit slices, citrus flavored tablets, gum
Muscarinic agonists
Artificial saliva
Treating Keratoconjunctivities (KCS)
Replacing or supplementing deficient tears
Immunosuppressive Treatment of Systemic Sjogren’s
Hydroxychloroquine
Methotrexate
Define Scleroderma
Thickened, hardened skin
Spectrum of Scleroderma
Limited anatomic extent affecting only the skin & adjacent tissues
Associated with systemic involvement
Pathophysiology of Systemic Sclerosis
Vascular damage
Immune activation
Excessive synthesis of extracellular matrix with deposition of structurally normal collagen results
Vascular Damage in Systemic Sclerosis
Vasoconstriction
Endothelia’s are fibrinogenic
Sera found to be cytotoxic to endothelial cells
Immune Activation in Systemic Sclerosis
Activation of endothelial cells
Up regulation of adhesion molecules
Leukocyte migration out of the vasculature
Diagnosis of Systemic Sclerosis
Skin induration
Heartburn or dysphagia
Acute onset HTN & renal insufficiency
Dyspnea on exertion associated with interstitial pulmonary changes
Pulmonary HTN
Diarrhea with malabsorption
Facial, lip, or hand telangiectasis
Digital infarctions and/or digital tip pitting
Heart issues
Specific Organ Manifestations Treatment Directed at
Cutaneous manifestations Raynaud's phenomenon Musculoskeletal manifestations GI manifestations Pulmonary manifestations Cardiac manifestations Renal manifestations ED & dyspaurenia
Cutaneous Manifestations of Systemic Scleroderma
Edema & erythema of skin Pruritus Digital ulcers Pitting at the finger tips Telangiectasia
Treatment for Cutaneous Manifestations
Emollient creams: soften skin & decrease pruritus Antihistamines & doxepin: pruritus UVA therapy Highly potent topical steroids Methotrexate
Define Raynaud’s Phenomenon
Exaggerated vascular response to cold temperature or emotional stress
Manifestation of Raynaud’s Phenomenon
Sharply demarcated color changes of the skin of the digits due to abnormal vasoconstriction of digital arteries & cutaneous arterioles
In Systemic Sclerosis, Raynaud Phenomenon can Lead to
Ulcerations
Digital infarctions
Gangrene
Treatment of Raynaud’s Phenomenon
Prevention CCBs: nifedipine or amlodipine Alpha blockers Low dose ASA ED pills?? Treat associated complications
Musculoskeletal Manifestations of Systemic Sclerosis
Erosive, inflammatory arthritis similar to rheumatoid arthritis
Treatment of Musculoskeletal Manifestations of Systemic Sclerosis
Methotrexate
GI Manifestations of Systemic Sclerosis
GI tract involved in all smooth muscle areas
GERD
Treatment of GI Manifestations in Systemic Sclerosis
Preventative measures
PPIs
Promotility agent: erythromycin
Pulmonary Manifestations of Systemic Sclerosis
Lung fibrosis
Pulmonary HTN
Increased risk of lung cancer
CT: ground glass appearance
Treatment of Pulmonary Manifestations of Systemic Steroids
Cyclophosphamide
Steroids
Cardiac Manifestations of Systemic Sclerosis
Conduction abnormalities or dysrhythmias
Treatment of Cardiac Manifestations of Systemic Sclerosis
Anti-arrhythmics
Cardiac pacing
Renal Manifestations of Systemic Sclerosis
Acute renal crisis
Treatment of Renal Manifestations of Systemic Sclerosis
ACE inhibitors
Define CREST
Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangectiasia
Pathophysiology of CREST
Production of anti-centromere & anti-nuclear antibodies
Development of pulmonary HTN & CHF
Define Polymyositis/Dermatomyositis
Inflammatory muscle disease that can occur with or without a cutaneous eruption
Clinical Manifestations of Polymyositis/Dermatomyositis
Proximal muscle weakness
Rash over PIP & MCP (Gottron’s sign)
Heliotrope over eyelids
Shawl Sign
Presentation of Proximal Muscle Weakness in Polymyositis/Dermatomyositis
Insidious onset
Symmetric
Mild myalgias
Diagnosis of Polymyositis/Dermatomyositis
Clinically Med Rec Muscle enzyme evaluation: CK, LDH, AST, ALT Elevated ANA Muscle biopsy
Pathogenesis of Dermatomyositis
Humorally-mediated (B-cells)
Cellular infiltrate focused around blood vessels
Abnormal muscle fibers grouped in one portion of fascicle
Pathogenesis of Polymyositis
Cellular infiltrate within the fascicle
Abnormal muscle cells scattered throughout the fascicle
Treatment of Cutaneous Manifestations of Polymyositis/Dermatomyositis
Mild to moderate topical steroid therapies
Hydroxychloroquine or methotrexate
Treatment of Muscular Manifestations of Polymyositis/Dermatomyositis
High-dose systemic steroids
PT program
Immunosuppressant medication: methotrexate, azathioprine
