Rheum/ Ortho Flashcards
Hyper mobility collagen syndromes , what are the two to be aware of
Ehlers danlos syndrome and marfans syndrome
Presentation of hyper mobile ehlers danlos syndrome
Hyper mobile joints
Joint pain after exercise
Soft and stretchy skin
What are the associations of hyper mobile EDS
High arched palate and dental crowding
Spine scoliosis
Arm span height ration >1.05
What is the Beighton score, and what is significant for it
Used to assess hyper mobility
Palms flat on floor with straight legs=1
Elbows hyper extended (1 for each side)
Knees hyperextended(1 on each side)
Thumb can touch forearm (1 on each side)
Little finger can bend past 90 degrees
Score of more than 6 suggests hyper mobility
What causes marfans syndrome
Autosomal dominant mutation of FBN1 coding for fibrillin
Clinical features of marfans
MSK- long limbs and fingers. Chest wall deformities. Scoliosis and hypermobitlity
CVS- aortic root dilation, aortic regurgitation, mitral and tricuspid prolapse. Most seriously aortic aneurysm and aortic dissection
Pneumothorax and restrictive lung disease
Definition of JIA
Joint pain for more than 6 weeks
Age less than 16
Diagnosis of exclusion following excluding infection and malignant
Investigations for JIA
FBC, CRP, ESR, u and e and lft
Urine dip
To exclude infection- blood culture, throat swab and viral PCR
To exclud malignancy (leukiemia and lymphoma) - bone marrow biopsy and lymph node biopsy
Most common type of JIA
Polyarticular >more than 5 joints affected
What are the features of systemic JIA (Stills disease)
Subtle salmon pink rash
High swinging quotidian fevers
Generalised lymphadenopathy
Weight loss
Joint pain
Hepwtosplenomegaly
Bloods for stills disease
ANA and RF are negative
Raised inflammatory markers, platelets (thrombocytosis) and ferritin
What type of JIA is silent uveitis seen in
Oligoarticular JIA
Features of polyarticular JIA
Rheumatoid arthritis equivalent of adults
Symmetrical in small and large joints
More often seronegative
Features of oligoarticular JIA
Oligoarticular JIA is often asymmetric, affecting the lower limb more
Less than 5 joints
Girls more common than boys
Silent uveitis occurs - must see ophthalmology once every 3 months
Managment of JIA
MDT- rheumatologist, nurse specialist, physio, occupational therapy, camhs, ophthalmology
NSAIDS, DMSRD (methotrexate for st least 3 months, biological like anti tnf adalminub
Specific mx of SJIA
NSAIDS
IV steroids if no improvement after 1 week
Management of methotrexate and steroids in illness
Hold methotrexate, but must keep or increase steroids due to risk of addisonian crisis
Presentation of macrophage activation syndrome or hemophagocytic lymphohistiocytodid (HLH)
Acutely unwell
Hepatosplenomegaly
Lymphadenopathy
Pancytopenia
DIC
HIGH ferritin and low ESR is key!
Needs specialist review - steroids and immunosuppressants
Muscle features of dermstomyositis
Muscle pain and weakness
Occurs bilaterally and affects proximal joints- pelvic and shoulder girdle
Bloods for dermatomyositis
Raised muscle enzymes - CK, LDH, AST and ALT
Cutaneous features of dermatomyositis
Heliotropic rash on face
Gottron lesions on knuckles elbows and knees
Nailfold capillar
Rash on eyelids
Dermatomyositis anti bodies
Anti jo 1
Anti mi 2
ANA
Mx of dermatomyositis
IV methylprenisilone or oral prednisilome
Methotrexate
Sun protection and adequate vit d and calcium
