Connective tissue disorders Flashcards
(22 cards)
What is Sjogrens
Relatively benign autoimmun condition affecting the functions of tear and saliva production
Female > male 9:1
How does sjogrens present
Dry eyes and mouth
Bilateral Parotid swelling
any systemic features of sjogrens
1/3 of patients will experience systemic features
Fever, fatigue, myalgia and arthralgia
Investigations of sjogrens
Schimer test - Tear production
Salivary scintigraphy - Gland function
Parotid Bx - looking for Leuckocytes in the gland
Antibodies present (and therefore tested for) in sjogrens
Anti-RO and anti-LA
Rx of Sjogrens
Artificial Tears and Saliva Replacement Solutions
For conjunctivitis - Chloramphenicol
For Oral Candida - Fluconazol
Complications of Sjogrens
NH lymphoma risk increased by 50% Purpura Neuropathy interstitial lung disease Renal tubular Acidosis
What is SLE
systemic Lupus Erthythematosus
Autoimmune condition of Type III hypersensitivity in which Immune complexes are deposited throughout the body - Mainly Skin, Joints and kidneys
Diagnosis of SLE
Require 1 Positive Lab test along with 4 Clinical criteria
Laboratory tests for SLE
ANA +ve (95%) - though positive in several things Anti-dsDNA Anti-SMITH Anti-phospholipid Ab Low Compliment Direct Coombs test
Clinical features
A RASH POINts MD to lupus
A - Arthitis R - Renal - Blood and protein A - ANA S - Serositis H - Haematology - low platelets and WCC and AIHA P - photosensitivity O - Oral Ulcers I - Immunology - Anti-dsDNA etc N - Neurological - Seizures & psychosis M - Malar Rash D - Discoid Rash
Treatment of SLE
Steroids and Immunosupressants (hydroxychloroquine)
What should you test in SLE before pregnancy
Anti-phospholipid Ab
Anti-cardiolipin ab
What is Scleroderma
Autoimmune thickening of the skin commonly affecting those in their 30-50s - Systemic Scleroderma is split into Diffuse and limited
Diffuse Scleroderma
Anti-Scl-70 internal Organs Affected Fatigue Wt loss Joint pain Skin affected throughout entire body
Complications of Diffuse Scleroderma
Renal Crisis
Pulmonary Fibrosis
Small Bowel bacterial overgrowth
Limited Scleroderma
Anti-centromere + CREST Syndrome
C - Calcinosis
R - Raynaulds (often presenting feature)
E - Eosophageal Dysfunction ( leads to GORD)
S - Sclerodactyly
T - Telangiectasis
Skin only affected distal to Knees and Elbows
Gradually worsens but not as severe as diffuse
Complications of Limited Scleroderma
Pulmonary Hypertension
Treatment of Scleroderma
Treat symptomatically
What is Osteogenesis Imperfecta
Type 1 Collagen disorder categorised by brittle bones easily damaged by mild trauma and even daily activities
What are the 4 most common types of Ostegenesis imperfecta
Type I - Milder form - presents on when starting to walk or as an adult
Type II - lethal by age 1
Type III - Progressively deforming with joint dyplasia and abnormal growth
Type IV - Similar to Type I but more severe
What are the Features of osteogenesis imperfecta
Abnormla rowth - Small children small adults Dentigenesis imperfecta Hearing loss Blue Sclera Barrel chest hypermobility Easy bruising Scoliosis
