rheumatology Flashcards
What do you expect the ANA, RF and ESR to be in patients with ankylosing spondylitis ?
Normal ANA and RF
Normal or mildly elevated ESR
What diagnosis presents with leg and back pain which is worse in the mornings and bending over but is relieved by exercise ? What are two other conditions associated with this disease?
Ankylosing spondylitis - uveitis and IBD
How should you treat a patient with ankylosing spondylitis ?
NSAIDs . Sulfadiazine and methotrexate
What diagnosis presents with ulcers in the mouth and genitals as well as uveitis and arthritis? How would you treat?
Behcet syndrome - steroids
What would you expect the following lab results to be in a patient with behcet syndrome: ANA, RF, ESR and CRP?
ANA and RF normal
Elevated ESR and CRP
What diagnosis would you suspect in a patient with clumsiness, shiny scaly skin on extensor surfaces, periungal lesions and difficulty swallowing ?
Dermatomyositis
What is the most appropriate initial step in the diagnosis of dermatomyositis? How would you treat?
Creatine kinase level
High dose steroids, immunosuppressives and IVIG
Avoid sunlight
Antimalarials sometimes used
Other than hypermobility of joints, what are 2 other findings associated with ehlers danlos syndrome ?
Poor wound healing and easy bruising
How can you distinguish Lowe’s syndrome from ehlers danlos syndrome?
Lowe’s also has hypermobile joints but also has blindness, intellectual disability and hypotonia
What diagnosis presents as blanching purpura on legs, crampy abdominal pain and blood in urine and stool? What lab findings do u expect to be abnormal?
Henoch schonlein purpura - elevated BUN/creatinine, urinalysis with protein and blood and normal platelet count!
What should be suspected when patients with HSP present with abdominal pain?
Ileoileal intussusception
What is the underlying cause/pathology of HSP?
Vasculitis of skin, GI Tract, joints and kidneys - usually after bacterial or viral infection
How can you distinguish the joint manifestations present in HSP from those seen in rheumatic fever ?
HSP involves periarticular disease with the soft tissues around the joints involved (vs articular disease in rheumatic fever)
What are the 8 main causes of polyarticular arthritis ?
Fabry disease Infection Reactive arthritis Rheumatic fever Serum sickness Connective tissue disease iBD Malignancy
What two criteria must be met in order to diagnose JIA?
Age of onset <16
Symptoms >6 weeks
What diagnosis should you suspect in a female with pain and swelling in >5 joints?
Polyarthritis JIA (type 1)
What diagnosis should you suspect in a child who presents with pain and swelling of 3 joints and uveitis?
Oligoarthritis JIA
What can an ANA and RF indicate in JIA?
Most patients are ANA positive and those with positive RF typically have worse disease
Both ANA and RF are usually negative in systemic JIA
What is the term used to describe systemic JIA which presents with extra-articular involvement?
Stills disease
What are 6 main extra-articular manifestations of systemic JIA?
High fever Leukocytosis Rash (red macules with central clearing that coalesce) Hepatosplenomegaly Lymphadenopathy Pleuritis/pericarditis
What is the first line treatment for a patient with morning stiffness & joint pain x 6 weeks, fevers and lymphadenopathy with symptoms that wax and wane? Second line treatment?
NSAIDs (1st line)
steroids and immunosuppressives (2nd line)
What are the abnormal lab findings common in Kawasaki?
Thrombocytosis Leukocytosis Normocytic anemia Elevated CRP/ESR Negative ANA/RF
When should cardiac ECHO be performed in patients suspected of having Kawasaki disease?
At time of diagnosis
2-3 weeks later
6-8 weeks after onset of illness
What is the appropriate treatment for Kawasaki disease ?
IVIG 2g/kg
Aspirin 80mg/kg for 48hr then 5mg/kg for 2 months
What is the difference between measles and Kawasaki disease?
Measles has exudative conjunctivitis and rash typically is descending
What is the cause of Lyme disease?
Immunologic response to borrelia burgdorferi
What are the common symptoms in the first two weeks of Lyme disease?
Erythema chronicum migrans rash (in 75% of cases) and vague flu like symptoms with arthralgia
What is the appropriate method of testing and confirmation of Lyme disease?
Lyme antibody titer (takes 4-6 weeks to become positive) then western blot to confirm
What diseases cause false positive Lyme testing?
Lupus
Dermatomyositis
ricketsia
What is the treatment for Lyme disease ?
Doxycycline 14-21 days (amoxicillin or cefuroxime if younger than 8 y/o)
What causes chills, fever and hypotension in a patient treated for Lyme disease?
Jarisch Herxheimer reaction due to lysis and endotoxin release
What diagnosis should you consider in a tall patient with high arched palate, hyperextensible joints and pectus? What could a murmur be due to in these patients?
Marfan - aortic or mitral valve regurgitation
What are the major criteria for diagnosis of Marfan syndrome ?
Dilatation/dissection of ascending aorta or aorta root
Ectopia lensis
Lumbosacral dural ectasia
Skeletal manifestations (4)
What is the inheritance of Marfan syndrome and where is the mutation located?
Autosomal dominant - chromosome 15 (fibrillin gene)
What two types of specialists should closely follow patients with Marfan?
Cardiology
Ophthalmology (serial slit lamp exams)
What is the likely diagnosis in a patient with vague joint pains who recently received 4 y/o vaccinations?
Post infectious arthritis from live MMR vaccine
What diagnosis should you consider in a patient who presents with swollen tender joints and conjunctivitis after an episode of diarrhea? What other symptom should you expect?
Reactive arthritis following salmonella, shigella or yersinia
Pt would also present with nongonococcal urethritis
What is a common cause of reactive arthritis in a sexually active teen?
Chlamydia
What would you expect the ANA and HLA-B27 results to be in a patient with reactive arthritis?
Normal ANA
Positive HLA-B27
How would you treat a patient suspected of having reactive arthritis?
NSAIDs and antibiotics
What heart problem is likely in a patient with rheumatic fever with persistent new murmur and mild CHF?
Aortic regurgitation
What heart problem is likely in a patient with rheumatic fever and a murmur at the apex?
Mitral valve regurgitation
What is the criteria for diagnosis of rheumatic fever?
Recent Group A strep infection plus:
2 major symptoms
or
1 major and 2 minor criteria
What are the 5 major jones criteria to diagnosis rheumatic fever ?
Polyarthritis (not arthralgia) Carditis Subcutaneous nodules (painless) Erythema marginatum Sydenham chorea
What are the 4 minor criteria to diagnose rheumatic fever?
Fever
Arthralgia (not arthritis)
Elevated acute phase reactants
Prolonged PR interval
What treatment would you use for the following rheumatic fever symptoms: arthritis, carditis, chorea and heart failure ?
Arthritis - aspirin
Carditis - steroids
Chorea - haldol
Heart failure - digoxin
What are two methods of definitive documentation of recent strep infection ?
ASO titers
Positive streptozyme
What diagnosis should you consider in a patient with chronic cough and easy fatigue as well as weight loss…chest X-ray shows hilar adenopathy?
Sarcoidosis
What is secreted from the non-caseating granulomas found in sarcoidosis ? What end organs are affected by this?
Vitamin D - this leads to hypercalcemia and hypercalciuria which causes renal and eye disease
What diagnosis presents as a linear hyperpigmented patch of skin that is painful and becomes more fibrotic over time? What are 5 treatment options ?
Scleroderma - lubricants, photochemotherapy, steroids, anti malarials and immunosuppressives
What are 4 symptoms of systemic scleroderma and how can you prevent progression from local scleroderma?
Raynauds Sclerodactyly Pulmonary fibrosis LES incompetence *there is no treatment to prevent "progression" to systemic scleroderma
What diagnosis should you consider in a patient who presents with fever, urticaria and arthralgia a few weeks after exposure to an antigen? What is the treatment?
Serum sickness - antihistamines and steroids
What happens to complement levels in patients with lupus?
C3/C4 levels go down during more active disease states
What are the diagnostic criteria for lupus?
4 or more of the following features:
- malar rash of discoid lesions
- photosensitivity
- oral ulcers
- arthritis
- low WBC/platelets /hemolytic anemia
- anti dsDNA or anti-SM antibodies
- cellular casts in urine
- pleuritis/pericarditis
- neurologic problems
What is the possible diagnosis In an adolescent female who presents with fever, joint pain and oral ulcers who has a problem with chronic ITP?
Lupus
What should you be concerned about in a patient with lupus who presents with respiratory distress and lower leg pain?
Thromboembolus/PE
What 6 drugs can cause drug induced lupus?
Procainamide Hydralazine Quinidine Sulfonamides Lithium Anticonvulsants
What diagnosis should you consider in a newborn with bradycardia, thrombocytopenia and red scaly rash on face? What is the most serious potential complication in these patients?
Neonatal lupus - 3rd degree heart block or hydrops fetalis
What antibody is found to be high in patients with neonatal lupus?
Anti SSA
What are side effects with use of hydroxychloroquine?
Retinal damage and ototoxicity
What is the likely cause of high blood pressure and visual changes in patients with lupus?
Chronic steroid use!
What is the likely diagnosis in a patient with chronic sinusitis, joint pain and fever with a positive c-ANCA?
Wegener granulomatosis
What is the appropriate treatment for wegener?
Cyclophosphamide and steroids
What diagnosis should you consider in a synovial fluid analysis with decreased viscosity and 15-20,000 WBCs?
JIA or reactive arthritis
What diagnosis should you suspect when synovial fluid shows WBC 5,000 and normal viscosity ? What if the viscosity is decreased?
Lupus – Rheumatic fever