Rheumatology/MSK Clues 1-61, no derm ULTIMATE LIST Flashcards
What is primary biliary cirrhosis?
Pruritis, xanthomas, anti-mitochondrial Ab; seen in females
Autoimmune rxn→ lymphocytic infiltrte +/- granulomas→ destruction of lobular vile ducts
Associated with other autoimmune conditions (hashimotos, rheumatoid, celiac, etc)
tx: ursodiol
What is primary sclerosing cholangitis?
P-ANCA Ab, bile duct inflammation, onion skinning, alternation beading of intra-extra hepatic bile ducts
associated with middle aged men, Ulcerative colitis, increased risk of cholangiocarcinoma and gallbladder cancer, MPO-ANCA/P-ANCA.
What is type I autoimmune hepatitis?
Anti-smooth mm. Ab; seen in young women
What is type II autoimmune hepatitis?
Anti-LKM Ab
What is bullous pemphigoid?
Anti-hemidesmosome Ab, subepithelial skin bullae
What is celiac sprue?
Anti-gliaden Ab (can’t eat wheat/ bread products = steatorrhea) assoc. w/ dermatitis herpetifomis
What Ab is assoc. w/ CREST Syndrome?
Anti-centromere Ab
What is dermatitis herpeteformis?
Vesicles on anterior thigh, Anti-BMZ Ab, Anti-endomysial Ab
What is dermatomyositis?
Anti-jo-1 Ab; Myositis with a rash; Assoc. w/ occult malignancy “dermato-jo”
- malar rash (distinguished from SLE by involving nasolabial folds)
- Gottron papules
- heliotrope (violaceous periorbital) rash
- darkening/thickening of finger tips
- perimysial inflammation + atrophy
- CD4+ T cells
What are symptoms of T1DM?
Polyuria, polydipsia, wt. loss, Anti-islet cell Ab, anti-GAD Ab, post-infection, DKA
What is drug Induced lupus(and causative drugs)?
Anti-histone-Ab; Drugs: Hydralazine Isoniazid Phenytoin Penicillamine Procainamide Ethosuximide
HiPPPE’s develop HISTONEs
What is gastritis type A?
Anti-parital cells, atrophic gastritis, adenocarcinoma
What is goodpasture’s?
Anti-GBM Ag, attacks the lungs and kidneys, can progress to RPGN and death
What is Graves’ disease?
Anti-TSHr Ab, hyperthyroid exopthalmos, pretibial myxedema
What is Hashimotos?
Anti-microsomal Ab/anti-TPO, hypothyroid
What is ITP (immune thrombocytopenic purpura)?
Anti-platelet Ab, anti-GP IIB/IIIA Ab
What is MCTD (mixed connective tissue disease)?
Anti-RNP Ab
What is mononucleosis?
Heterophile Ab positive; teenager w/ sore throat, lymphadenopathy, splenomegaly, “kissing disease”
What is MPGN type II?
Anti-C3 convertase Ab= C3 nephritic factor
What is paroxysmal hemolysis?
Donath landsteiner Ab; bleed when cold
Paroxysmal cold hemoglobinuria (PCH, also called Donath-Landsteiner hemolytic anemia or Donath-Landsteiner syndrome) is an uncommon type of autoimmune hemolytic anemia (AIHA) in which autoantibodies to red blood cells bind to the cells in cold temperatures and fix complement, which can cause intravascular hemolysis
What is pemphigus vulgaris?
Anti-desmosome Ab (skin sloughs off when touched) starts in oral mucosa
What is pernicious anemia?
Anti-IF Ab, via. B12 deficiency = megaloblastic anemia
What is polyarteritis nodosa?
P-ANCA Ab; attacks gut, kidneys and nervous system; assoc. w/ Hep. B infection. There is no pulmonary environment
PAN is a multisystem disease that may present with fever, sweats, weight loss, and severe muscle and joint aches/pains.
What is post-strep. GN?
ASO Ab, Nephritic w/ complement deposition; after 3 weeks post-infection
What is RA?
Rheumatoid factor present pain and swelling, worse in the morning, anti-citrullinated peptide protein Ab’s
What is scleroderma?
Anti-Scl70 Ab (anti-Topol), fibrosis, tight skin, masked faces
What is Sjogrens?
Anti-SSA Ab/Anti-Ro dry eyes, dry mouth, arthritis Sjogren’s is an ASS
What is SLE?
Anti-dsDNA; Anti-Smith, Anti-cardiolipin Ab. Malar rash, photosensitivity, oral ulcers, RF positive, VDRL positive but not infected, FTA-ABS to confirm
What is SLE Cerebritis?
Anti-neuronal Ab; Anti-ribosomal Ab
What is Vitiligo?
Anti-melanocyte Ab; white patches on skin
What is warm hemolysis?
Anti-Rh Ab; bleeds at body temperature
Rh factor (on Red Blood cells!!)
What is HSP?
IgA Disease - 2 or 3 days post-common cold or GI infection. Leads to Berger’s
What is Berger’s?
IgA disease, 2 weeks post vaccination; serum sickness
What is Alport’s?
IgA disease; 2 weeks after diarrhea, HSP and polio
- bilateral sensorineural hearing loss
- glomerular disease
- ocular disease (lens dislocation)
- x linked dominant
- IgA attacks type IV collagen
Where is CK-MB found?
Heart
Where is CK-MM found?
Muscle
Where is CK-MB found?
Brain
Why should you wait 30 mins after a meal before swimming?
All blood is shunted to GI for digestion, digestion depletes ATP stores; No ATP = no muscle movement
How does neurogenic muscle disease present?
Distal weakness and fasciculations
How does myopathy muscle disease present?
Proximal weakness and pain
What is a light chain composed of?
Actin
What is a heavy chain composed of?
Myosin
What band of sarcomere does not change length?
The A band
Where are T-tubules located?
Cardiac mm: at the z-line Skeletal mm: at the A-I junction
What is duchenne’s muscular dystrophy?
Caused by a dystrophin frame shift; defective Dystrophin patient will have a Gower sign and calf pseudohypertrophy
What is Becker Muscular Dystrophy?
Dystrophin Missense; milder form of DMD Symptoms present after 5yo
What is seen with Myotonic Dystrophy?
Bird’s beak Face; can’t let go when shaking hands
What is Myasthenic Syndrome/Lambert-Eaton?
Patients will tell you that they get stronger as the day goes by; stronger with EMG (electromyography) Assoc. w/ small cell CA
What is Myasthenia Gravis?
Anti-ACh post-synaptic Ab; PT ~20-30yo, weaker as day goes on (dysarthria, ptosis, dysphagia) Periotic exacerbations get stronger w/ Edrophonium Weaker with EMG (electromyography); must rule out thymoma
What is MS?
Production of Anti-myelin Ab MC in 20-40yo women, vision disturbances, spasticity (UMNs), decreased sensation, symptoms wax and wane - each MS attack gets worse
What is Metachromatic Leukodystrophy?
Arylsulfatase A deficiency; a child who presents similar to MS
What is ataxia telangiectasia?
IgA deficiency; PT has spider veins
What do you see in Friedrick’s Ataxia?
Retinitis pigmentosa, scoliosis
What is adrenal leukodystrophy?
Carnitine acyltransferase (CAT-1), defective adrenal failure (XR); long-chain FA stuck in cytoplasm
What is Guillain-Barre?
Ab’s damage multiple peripheral nerves (acute inflammatory polyneuropathy); patients present with ascending paralysis 2 weeks post-UTI
What is ALS?
Descending paralysis seen in middle aged men with fasciculations; no sensory problems
What is Werdnig-Hoffman?
Fasciculations in newborn due to failure of anterior horns to develop
Werdnig Hoffman is the old name for Spinal Muscular Dystrophy, type 1 (SMA-1)
What is polio?
Asymmetric fasciculations in kids; presents 2 weeks post- gastroenteritis
What is choreoathetosis?
Dance-like movements; wringing of hands, quivering/unsteady voice
What is Atonic Cerebral Palsy?
No mm. Tone - floppy
Fatigue, malar rash, inflammatory and symmetric joint pain, photosensitivity
SLE (may also see discoid lesions, Reynaud’s, myalgias, pericarditis/endocarditis, pleuritis, hemolytic anemia, proteinuria/GN, impaired immune response, nausea/vomiting/dyspepsia, seizures/psychosis) Treatment: NSAIDS for mild symptoms, corticosteroids for exacerbations and severe manifestations, hydroxychloroquine (constitutional/cutaneous/articular), and cyclophosphamide (glomerulonephritis)
SLE syndrome without CNS or kidney involvement
Drug-induced lupus Treatment: remove offending drug (hydrazine, procainamide, isoniazid, chlorpromazine, methyldopa, quinidine)
Raynaud’s, sclerodactyly, dysphagia, pulmonary HTN
Diffuse scleroderma (can have renal malignant HTN, pulmonary HTN/interstitial fibrosis, or pericardial/myocardial involvement) Treatment: symptomatic, treat complications
Raynaud’s, sclerodactyly, dysphagia, calcium deposits, telangiectases under nails
CREST (cutaneous limited–no renal/pulmonary/cardiac findings) Treatment: symptomatic
Dry eyes/mouth, arthalgias, interstitial nephritis, vasculitis, positive Schirmer test
Sjogren’s (Dx: Schirmer test for lacrimal gland output, salivary gland biopsy) Treatment: pilocarpine/cevimeline (increase secretions), artificial tears, NSAIDS
Pulmonary manifestations, esophageal dysfunction, polyarthritis, sclerodactyly, cutaneous findings, myopathy, Raynaud’s
Mixed connective tissue disease (SLE, RA, systemic sclerosis, polymyositis) Treatment: varies depending on which disease predominates
Fatigue and symmetrical joint pain in PIP/MCP/wrist/knees, morning stiffness which improves throughout day, subcutaneous nodules over extensor surfaces
RA Treatment: NSAIDS/low dose corticosteroids and DMARDS (methotrexate with folate, leflunomide, or hydroxychloroquine)
Anemia, neutropenia, splenomegaly, RA
Felty’s syndrome
50 year old man with podagra and fever
Gouty arthritis (Dx: needle shaped negatively birefringent urate crystals in synovial fluid) Treatment: bed rest, indomethacin, colchicine, corticosteroids (oral or intra-articular injection) Prophylactic: allopurinol (xanthine oxidase inhibitor decreases uric acid synthesis), probenecid (uricosuric drugs increase renal excretion of uric acid)
Acute onset of severe unilateral knee or wrist pain with erythema, swelling, and warmth
Pseudogout, also called calcium pyrophosphate deposition disease (Dx: weakly positively birefringent rod-shaped and rhomboidal crystals in synovial fluid) Treatment: treat underlying disease (hemochromatosis, hyperparathyroidism, hypothyroidism, Bartter’s syndrome) and manage symptoms with NSAIDS, colchicine, and intra-articular corticosteroid injections
Subacute onset of weakness of neck flexors, shoulder girdle, and pelvic girdle muscles with myalgias and rash around eyes, bridge of nose, and cheeks Elevated CK, AST, ALT, and LDH
Dermatomyositis (can be associated with vasculitis) Types of rashes: heliotrope, Gottron’s papules (scaly lesions over knuckles), V sign (rash on face, neck, anterior chest), shawl sign (rash on shoulders, upper back, and elbows), periungual erythema with telangiectases Treatment: corticosteroids and immunosuppression (methotrexate, cyclophosphamide, chlorambucil)
Weakness of shoulders and pelvic muscles, dysphagia, and myalgias
Polymyositis Treatment: corticosteroids and immunosuppression (methotrexate, cyclophosphamide, chlorambucil)
Older man with progressive asymmetrical weakness of quadriceps, forearm flexors, tibialis anterior, and facial weakness with loss of deep tendon reflexes and dysphagia
Inclusion body myositis Treatment: poor response
Weakness of shoulders and pelvic muscles, dysphagia, and myalgias
Polymyositis Treatment: corticosteroids and immunosuppression (methotrexate, cyclophosphamide, chlorambucil)
Older man with progressive asymmetrical weakness of quadriceps, forearm flexors, tibialis anterior, and facial weakness with loss of deep tendon reflexes and dysphagia
Inclusion body myositis Treatment: poor response
Older man with progressive asymmetrical weakness of quadriceps, forearm flexors, tibialis anterior, and facial weakness with loss of deep tendon reflexes and dysphagia
Inclusion body myositis Treatment: poor response
Elderly patient with abrupt stiffness, especially in morning, in shoulder/hip after a period of inactivity. Pain with movement and muscle strength 5/5.
Polymyalgia rheumatica (10% develop temporal arteritis) Treatment: resolves within 1-2 years (self limited) but corticosteroids reduce inflammation until this happens
Woman with anxiety/depression and aching pain in many joints exacerbated by weather changes and stress
Fibromyalgia Treatment: some effect with SSRIs and TCAs, otherwise cognitive/psych
Man with low back pain/stiffness worse in morning, acute anterior uveitis, enthesitis
Ankylosing spondylitis Treatment: indomethacin, anti-TNF (etanercept, infliximab), PT
Acute asymmetric arthritis and effusions of joints of lower extremities that progresses from one joint to another. History of recent GI infection
Reactive arthritis (preceded by salmonella, shigella, campylobacter, chlamydia, yersinia) Treatment: NSAIDS, if no response give sulfasalazine and immunosuppressive agent (azathioprine)
Arthritis, uveitis, urethritis
Reiter’s syndrome Treatment: NSAIDS, if no response give sulfasalazine and immunosuppressive agent (azathioprine)
Sausage digits, psoriasis, arthalgias of small joints
Psoriatic arthritis Treatment: NSAIDS, if no response methotrexate/leflunomide
Severe headache with jaw claudication and visual impairment
Temporal arteritis (40% also have polymyalgia rheumatica) Treatment: high dose prednisone
Constitutional symptoms, absent pulses in carotid/radial/ulnar arteries with tenderness and signs of ischemia
Takayasu’s arteritis (of aortic arch/branches) Treatment: steroids, anti-HTN, surgery/angioplasty
Oral and genital ulcers, arthritis of knee/ankle, uveitis, intracranial HTN, fever
Behcet’s syndrome Eye findings: uveitis, optic neuritis, iritis, conjunctivitis CNS findings: intracranial HTN, meningoencephalitis Treatment: steroids
Glomerulonephritis, sinusitis, and hemoptysis
Wegener’s granulomatosis Treatment: cyclophosphamide and corticosteroids can induce remission but most die within 1 year of diagnosis
Painful palpable purpura, with fever, weight loss, and fatigue after infection
Hypersensitivity vasculitis (reaction to penicillin, sulfa drug, infection, or other stimulus) Treatment: steroids and removal of offending agent
Oral and genital ulcers, arthritis of knee/ankle, uveitis, intracranial HTN, fever
Behcet’s syndrome Eye findings: uveitis, optic neuritis, iritis, conjunctivitis CNS findings: intracranial HTN, meningoencephalitis Treatment: steroids
30 year old male smoker with leg pain on exertion, paresthesias, and ulcerations on digits
Buerger’s disease (thromboangiitis obliterans) Treatment: smoking cessation
Painful palpable purpura, with fever, weight loss, and fatigue after infection
Hypersensitivity vasculitis (reaction to penicillin, sulfa drug, infection, or other stimulus) Treatment: steroids and removal of offending agent
Glomerulonephritis, hemoptysis, and IgG anti-glomerular basement membrane antibody
Goodpasture’s syndrome
1) progressive symmetric proximal m weakness, anti-Jo1+ (histidyl tRNA synthetate),+anti-Mi2, +ANCA,
2) progressive symmetric proximal m weakness, anti-Jo1+ (histidyl tRNA synthetate),+anti-Mi2, +ANCA + malar rash, gottron papules, heliotropic rash
1) polymyositis
2) dermatomyositis
pain and stiffness in proximal muscles of shoulders and hips but does not cause muscle weakness
polymyalgia rheumatica
