Misc; amy, ray, rsp3, palin, sarcoid pbc rf Flashcards
a. Amyloidosis: primary, secondary, hereditary b. Raynaud’s disease c. Charcot joint d. Remitting seronegative symmetrical synovitis with pitting edema e. Multicentric reticulohistiocytosis f. Plant thorn synovitis g. Intermittent arthritides: palindromic rheumatism, intermittent hydrarthrosis h. Arthritic and rheumatic syndromes associated with: sarcoidosis, scurvy, pancreatic disease, chronic active hepatitis, primary biliary cirrhosis, drugs, and environmental agents i. Rheumatic dise
What are the common causes of angioedema?
Drug reaction to Ace inhibitors are the most common cause, but NSAID’s, calcium channel blockers, estrogens, fibrinolytic agents, any new medication or significant increase in dosage should also be suspect. Complement factor 1 inhibitor (C1-INH) deficiency either inherited (Types 1, 2 or 3) or acquired are not as common. Inherited forms are due to absent protein, defective protein, or perhaps inherited problems with factor XII causing bradykinin release. Aquired C1-INH deficiency can occur with malignancies where autoantibodies develop to C1-INH.
What common diseases cause arthritis?
Likely causes include: rheumatoid arthritiis, gout, Crohn’s disease, ulcerative colitis, Lyme disease, psoriatic arthritis, Reiters syndrome, ankylosis spondylitis, Systemic lupus erythematosus, reactive arthritis, acute serum sickness.
Less common causes include sarcoidosis, spondyloarthritis, gonococcal arthritis, viral arthritis-parvovirus b19, post traumatic arthritis, Intraarticular hemorrhage, loose body, torn meniscus, post traumatic arthritis, and osteochondritis. Degenerative joint disease, and degenerative disc disease are more likely to cause pain and noninflammatory swelling.
What are the diagnostic criteria for Behcet’s disease?
Behcet’s disease is characterized by the presence of recurrent aphthous ulcers with at least 3 episodes in any 12 month period. There must be at least 2 additional features such as:
recurrent genital ulceration,
eye lesions, Anterior or posterior uveitis on slit lamp examination or retinal vasculitis documented by an ophthalmologist,
skin lesions, erythema nodosum or papulopustular skin lesions or pseudo-folliculitis with characteristic achneiform nodules observed by a physician
positive pathergy test. A positive pathergy test is defined by a papule 2 mm or more mm in size developing within 48 hours after the insertion of a 20gauge needle 5 mm into the forearm skin.
Other features commonly explained by Behcet’s disease include arterial aneurysms in the heart, kidney, or pulmonary vasculature, oligo or polyarticular non deforming arthritis, renal amyloidosis or glomerulonephritis, pericarditis or myocarditis. Aseptic meningoencephalitis, cerebral vasculitis, recurrent phlebitis, arteritis, synovitis, or focal bowel ulceration might substitute for pathergy in European or North American populations.
What life-threatening complications occur in Behcet’s disease?
The greatest morbidity and mortality comes from neurologic, ocular, and large vessel arterial or venous disease.
What commonly causes bilateral knee pain in an adolescent with normal x-rays?
Billateral anterior knee pain is a descriptive diagnosis for a condition formally called chondromalacia patella. The descriptive term is more honest as chondromalacia is difficult to demonstrate as a specific cause of knee pain and is often asymptomatic. Bilateral anterior knee pain seems to occur in late adolescence during vigorous physical activity and then subsides over years. It is not a precursor to osteoarthritis or rheumatoid arthritis. Mechanical irritation in the soft tissues guiding the quadriceps/patellar tendon from overuse and/or poor biomechanical stress tolerance is the probable cause.
What treatments are commonly used for acute gouty arthritis?
NSAID, PO glucocorticoids, cochicine, and intraarticular glucocorticoids In various combinations are commonly used. Comorbid conditions affect the choice as many patients have associated cardiovascular disease or gastric irritation that contraindicates NSAID use. 5 mg of prednisone twice daily is usually adequate however much higher doses are commonly used. Colchicine doses used in the past were commonly excessive and 0.6 mg twice daily initially is adequate. Education about gout causes including hyperuricemia, obesity, hypertension, and diuretic use may also need attention.
Canakinumab (Ilaris) an IL-1 beta inhibitor, or anakinra (Kineret) an anti IL1 receptor antagonist, may be used in complicated situations.
With systemic diseases can cause muscle enzyme elevation?
Systemic disorders such as inflammatory myopathy (polymyositis/dermatomyositis, systemic lupus erythematosus, and mixed connective tissue disease with antibodies to RNP), infectious myopathy, drug induced myopathies (alcohol, lipid lowering drugs, cocaine, antimalarial drugs, antipsychotic drugs, colchicine, antiretroviral drugs, ipecac, Interferon alpha and penicillamine), dystrophic myopathies, neuroleptic malignant syndrome, metabolic myopathies, hyperthermia, motor neuron disease, endocrine myopathy, and periodic paralysis should be considered. Presymptomatic myopathies as in the early stages of polymyositis, distal myopathy, mitochondrial myopathies, inclusion body myopathy, muscular dystrophy, myotonic dystrophy, and predisposition to malignant hyperthermia.
What is the PFAPA syndrome?
PFAPA syndrome refers to a Periodic Fever, Apthous Pharyngitis, cervidal Adenitis syndrome usually presenting before age 5, without correlation to ethnicity, geographical distribution, or gender or genetic defect.
What is diagnostic of inclusion body myositis?
Biopsy shows basophilic rimmed vesicles on frozen section and permanent sections show variation fiber size. On electron microscopy the inclusions resemble myxovirus particles. CD8 + T cells predominate in the inflammatory infiltrate. Inclusion body myositis generally occurs in older men causing slowly progressive mostly distal muscle weakness and lower than expected increases in muscle enzymes. EMG tends to have more fibrillation and polyphasic potentials and MRI changes involve distal anterior muscle groups. Rare genetic enzymatic abnormalities
What are the diagnostic criteria for chronic fatigue syndrome?
Chronic fatigue syndrome criteria include documented fatigue for six months or more, and at least four of the eight following symptoms:
post exertion malaise,
impaired memory/concentration,
unrefreshing sleep,
muscle pain,
polyarticular-joint pain without redness or swelling,
tender cervical or axillary lymph nodes,
sore throat,
headache.
Fatigue must be severe enough to interfere with work or activities.
Exclusionary medical conditions include COPD, congestive heart failure, cirrhosis, hepatitis B or C, insulin-dependent diabetes, rheumatoid arthritis, specific lupus erythematosus, sickle cell anemia, stroke without full recovery, multiple sclerosis, Parkinson’s disease, dementia, epilepsy, schizophrenia, bipolar disorder, depression with psychotic or melancholic features, anorexia nervosa or bulimia, drug/alcohol/narcotic abuse within two years before fatigue diagnosis, narcolepsy, obstructive sleep apnea, sleep disordered breathing, restless leg syndrome, and periodic limb movement disorder.
What are the usual signs and symptoms of granulomatosis with polyangiitis (GPA) ?
Granulomatosis with polyangiitis (GPA , formerly Wegener’s granulomatosis), was initially recognized due to nasal or oral inflammation with ulceration and crusting, pulmonary infiltrates or nodules, abnormal urinary sediment with red cell casts with acute glomerulonephritis, and granulomatous inflammation with capillaritis on biopsy. ANCA Testing typically shows antibodies to proteinase 3 (C-ANCA). Antibodies to myeloperoxidase are more typical of microscopic polyangiitis wherein biopsies usually show capillaritis without the granulomas. Both patterns are pauci-immune.
What is Erdheim-Chester disease?
Erdheim-Chester disease, a.k.a. polyostotic sclerosing histiocytosis is characterized by lipid laden macrophages, multinucleate giant cells, and inflammations in the bone marrow with generalized sclerosis of long bones. Presents with periarticular knee and ankle bone pain, + exophthalomos. Biopsy shows CD68+ CD1a (MHC) cells.
What is the role of abdominal CT examination in inflammatory bowel disease?
On CT scanning of the abdomen, ulcerative colitis usually shows less wall thickness and little evidence of right-sided colonic involvement, mesenteric fibrofatty proliferation, and abscess formation. Barium studies are useful for diagnostic purposes, and CT scanning is best for discovering extra intestinal complications.
What antibody tests are helpful in diagnosing celiac disease?
IgA anti-tTG (tissue transglutaminase) antibodies occur in over 95%, IgG in over 90% similar to IgG antibodies to deamminated gliadin peptides (IgG DGP). All tests are very specific.
What are some common amyloid types?
Aβ, Beta amyloid, forms in Alzheimer’s disease, AA from SAA in Rheumatoid Arthritis, ACal-calcitonin in medullary cell carcinoma, Aβ2M Beta-2 microglobulin in dialysis related amyloidosis, and alpha-synuclein in Parkinson’s disease.
