Reu 6 - Systemic Disorders Flashcards
What are the diagnostic criteria for Systemic Lupus Erythematosus?
There has to be 4 of the 11 diagnostic criteria.
1.Skin disorders (malar rash,discoid rash,photosensitivity,painless oral ulcer).2,Inflammatory disorder(Arthritis[non-erosive, 2 joints], Serositis[pleuritis, pericarditis], (+)ANA). 3. Organ system Disorder (Renal[proteinuria, cellular casts],Neuro[seizures, psychosis],Heme[hemolytic anemia, leukopenia, lymphopenia,thrombocytopenia],Immune[antiphsopholipid Ab(+), anti-dsDNA, anti-Smith, false (+) VDRL]).
What type of lesions do we see in Lupus nephritis?
[Wire lupus] Wire loop lesions.
What labs are ordered for Lupus?
(+)ANA (antinuclear antibodies), Anti-dsDNA Ab (more specific for lupus), Anti-Smith Ab, Anti-histone Ab (Shows up more on drug induced lupus).
What diseases can have (+) ANA?
SLE, Sjogren syndrome, Scleroderma, Polymyositis and dermatomyositis, Rheumatoid arthritis, Juvenile idiopathic arthritis, Mixed connective tissue disease.
What drugs are associated with drug-induced Lupus?
[SHIPP]
Sulfonamides, Hydralazine, Isoniazid, Phenytoin, Procainamide.
What are the treatments for Lupus?
Steroids, NSAIDs, Hydroxychloroquine, Cyclophosphamide.
What is Scleroderma?
AKA Progressive systemic sclerosis, it causes fibrosis and excess collagen deposition. Skin (tight, shiny (no wrinkles). There are two main types: Diffused Scleroderma, CREST syndrome.
Describe Diffuse Scleroderma.
Diffuse Scleroderma: Diffuse, widespread sclerosis, it progresses rapidly, involves the visceral organs. It has Anti-DNA topoisomerase I (Anti-Scl-70) antibodies
Describe CREST Scleroderma.
Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias. There is limited skin involvement, less visceral involvement, Progresses less rapidly. It has anti-centromere antibodies.
What are the symptoms of Sjogren Syndrome?
Dry eyes (dryness, conjunctivitis, sensation of sand in the eyes), Dry mouth (dysphagia, difficulty swallowing), Arthritis. Can cause Parotid enlargement, increased risk of cavities, Increased risk of B cell lymphoma, autoantibodies (Anti-SSA [Anti-Ro], Anti-SSB [Anti-La]), (+) rheumatoid factor., Women over the age of 40.
What are Sicca symptoms?
Dry eyes, Dry mouth, Nasal dryness, Vaginal dryness, Chronic bronchitis, Reflux esophagitis.
What are the symptoms of Duchenne muscular dystrophy?
X-linked frameshift mutation causing a deletion of the dystrophin gene. Accelerated muscle breakdown causing elevated CK. Presents very early in life, starting with weakness in pelvic girdle muscles, progressing superiorly. They get pseudohypertrophy of the calf muscles due to replacement of muscle with fibrofatty tissue. They use Gower’s maneuver to stand up.
What are the symptoms of Becker muscular dystrophy?
Caused by a mutated dystrophin gene, it is less severe than DMD. It has a later onset: adolescence, early adulthood.
What are the symptoms of Polymyalgia Rheumatica?
Pain and stiffness in the shoulders and hips, malaise, weight loss, fever, but no muscle weakness. Average age of onset is 70, women and associated with temporal arteritis.
What are the lab findings and treatment for Polymyalgia Rheumatica?
Lab is going to find elevated ESR and normal CK. Treatment is steroids.