Vasculitis and Conective Tissue Dz Flashcards
Necrotizing Granulomatosis inflammation with eosinophila and asthma. Dx? Test? Rx?
Churg Strauss. Check for P-ANCA. Give steroids.
Treatment SLE?
Avoid sun. NSAIDs. Local or systemic steroids for acute exacerbation. Long term therapy is hydroxychloroquine ( watch out for retinal toxicity) and cyclophosphamide for glomerulonephritis.
Test for scleroderma? Rx?
Anticentromere and antitopoisomerase. Can also do a barium swallow esaohogeal dysmotility ( CREST). No effective treatment. NSAIDs for muskoloskeletal pain. Raynauds -avoid cold. H2 and PPIs for esophagus.
Dry eyes dry mouth. Dx? Test? Rx? What two dz is this associated with. Explain 1 of the diseases.
Sjogren syndrome. SS- A rho SS-B la. Pilocarpine or cevimeline. Can give artificial tears and maintain good oral hygiene. RA and B cell lymphoma- late in the dz course pt will have unilateral enlargement of the parotid gland.
What does CREST stand for ?
Calcinosis Raynaud’s phenomenon esophageal dysmotility sclerdactly, telengectasia.
Anti U 1 RNP Abs. Dx? What dz consist of this dz?
SLE, RA, Systemic Sclerosis, Polymyositis
Ulnar deviation, spearing of the DIP joints, swan neck deformity, boutineres deformity. Morning stiffness. Dx? Test? What does X-ray show? Rx?
RA. Rf and ACPA. High RF means severe dz start DMARD right away. If not X-day shows narrowing of the joints and erosion of the bones. Exercise, NSAIDs, steroids. Dmards methotrexate, leflunomide, hydroxychloroquine. Surgery includes synovectomy or joint replacement.
Build up of monosodium urate crystals. Name 3 etiologies for this disease? Dx? Test? Rx? Complications
This is Gout. Hemolysis, Chemotherapy, Lesch Nyan Syndrome, Renal Dz. Aspiration of joint will show crystals which are negatively bifrigent. Xray may reveal punched out lesions and erosions. Start with 1. NSAID 2. Colchicine 3. Steroids 4. Prpebenacid and Allopurinol ( more for prophylactic treatment having 2 attacks a year). Gout can lead to degnerative arthritis. - prevent topheaceous gout with effective treatment.
Build up of calcium pyrophosphate crystals. Dx? Etiology? Test? Rx?
This is pseudogout. Occurs in old age and ppl with osteoathritis Hyperparathyroidsm, Barrters Syndrome. Aspiration of the joint will show postively bifrigent crystals. Xrays may show calcified cartilage. Need to rx the underlying disorfer if aidentified
Heliotrope Rash, Grottons papules symmetrical proximal muscle weakness. Dx? Test? which antibodies have the worse prognosis which has the better prognosis. Rx?
Dematomyositis. CK levels. Check for Anti-Jo 1 antibodies , Antisignal recognition particle (worse prognosis) Anti Mi 2 antibodies - better prognosis. Rx? 1.Steroids dont respond then methotrexate.
proximal and distal muscle weakness with Muscle atrophy? Dx? Test? Rx?
Inclusion Body Myositis, Diagnosis of exlcusion. Clincal diagnosis. Poor response to therapy.
Old lady age 70 with fever, malaise, hip and shoulder stiffness + pain and joint swelling. Dx? Test? Rx? What other dz is this associated with?
Polymyalgia rheumatic, clinical diagnosis, will have elevted ESR. Corticosteroids will stop inflammation may see response within 2 years.
Female with aches and pains everyhwere that waxes and wanes. Wakes her up from sleep. Also is depressed. Dx? Test? Rx?
Fibromyalgia. Need at least 11 of the 18 possible tender points. rx with SSRI and (amitryptiline) TCA.
Low back pain and stiffness that is worse in the morning and better with use. Occurs in boys. Dx? Which genetic number is this associated with? Which other dz is this associated with? Test? Rx? Worse Complications ?
Ankylosing spondylitis occurs in young males HLA B27. Associated with UC. Elevated ESR. Xray shows bamboo spine. Indomethacin (NSAIDs) Anti- TNF meds (infliximab adn Entercaept). Can lead to restrictive lung dz with inability to expand chest secondary to pain. Can lead to easy fracture of spine and inability to stand up straight (loss of lumbarlordosis)
After genitourinary infection or GI infection, pat Cant pee, Cant see, Cant Climb a tree. Bugs? Dx? Test? Rx?
This is Reactive artthritis specifically reitiers syndrome. Urethritis, Uveitis, Athritis. Shigella, Salmonella, Yersinia, Camplybacter, Chlyamida. Aspirate fluid and analysis. Give NSAIDs.
Old lady age 50 with polymyalgia rheumatica. Develops HA, tenderness upon palpation of the temporal region of the head. Jaw pain with chewing. Dx? Test? Rx? what is important about this management?
this is Giant Cell Arteritis. Important to give steroids before doing the biopsy to prevent eye loss. ESR is also elevated. Give High dose prednisone for 4 weeks.
Young asian women develops limb ischemia, absent pulses. Etiology? Dx? Test? Rx? Name 3 complications?
Takayasu’s Arteritis. Vasculitis of the aortic arch causes absent pules and limb ischemia. Can lead to aortic regurgitiation and aneurysm. Need to do arteriogram. Rx with steroids. Rx HTN.
Asthma, palpable purpura. Dx? Test? Rx?
Churg Strauss. Check P-anca. Poor prognosis.
Rx of wegners?
Cyclophosphamide and Corticosteroids. prognosis is poor.
Etiology of PAN. Test? Dx? Rx?
Hep B or HIV. biopsy of medium vessels. P-anca.
Prognosis is poor can try steroids.
Vasculitis that causes genital and oral ulcers
Bechets Syndrome.
Vasculitis associated with smoking cessation as a form of treatment? Complication?
Buergers Disease (Thromboangiitis Obliterans) Veseels of the arms and legs can lead to gangrene.
Pt has stiffness of MCP and PIP joint. Pt has morning stiffness. There is no swelling and no other symptoms. ESR is 12 Dx.
Viral Athritis likely due to parovirus 19.
Acute Rx of Gout? Preventive Rx?
Steroids, NSAIDs, Colchicine. Prevention - Allopurinol and Probenacid.
GERD, Pulmonary HTN, R Heart Failure, Renal failure Skin thickening Raynouds. Dx? Marrker?
Systemic Sclerosis (CREST). ANA and Anti topoisomeraes (anti scl-70)/ anticentromere andtibodies.
Inflammation of the medial patella tibia? Inflammation directly above the patella?
Anserine Bursitis with negative valgus sress test. Prepatella bursitis
Peripattellar pain worsened with activity and sitting and creptius with motion of the patella.
Patellofemoral syndrome. Due to overuse
hypcalcemia and alot of infections - viral and fungal Dx?
Digerorge Syndrome 22q1 deletion - failure of the 3rd and 4th pharyangeal puch. No parathyroid no thymus - t cells cant mature.
Name the 3 types of SCID ( Both humoral and cell mediatied deficiencies)
1.ADA defiecincy (Adenosine is toxic to T cells 2. Cytokine receptor defects (proliferation and maturation of B and T cells does not occur) 3. MHC class 2 ( no activation of CD4 T cells)
Infections with bacteria, enterovirus (coxsackie and polio) and Giardia.
X-linked Agamaglobinemia. Mutation in Brutin tyrosine kinase. Naive B cells can mature in to plasma cells. - avoid live vaccines
Thrombocytopenia, Eczema and Infections
Wiskott-aldrich syndrome. X-linked WASP gene mutation.
Angiodema
C1 inhibitor deficiency ( over activation of complement)
Mutation in CD40 L
Hyper IgM Syndrome second signal defect IgM cannnot be turned into IgG or IgE or IgA.
35 yo with pain to palpation of the shoulder, back and neck. C/o poor sleep, easily faitgue Dx? Rx?
Fribomyalgia. TCA.
50 year pt c/o of morning stiffness in the shoulder and pelvic gridle. Dx? What will be elevated?
Polymyagia rheumatica. ESR is elevted.
Pt complains of weakness with rising from chairs and trying to lift elbows over her arms. No skin changes. Dx?
Polymyosisits.
Pain/hyperparastheisa in the 3rd and 4th meterasal. Gets better when shoes are off. Dx?
Morton Nueroma
In pagets dz of the bone what would be the levels:
1. Calcium 2. Phosphate 3. Alk phos 4. Urinary Hydroxyproline ?
- Normal 2. Normal 3. Elevated 4. Elevated
