Rheum Disorders
SLE
SJOGRENS
INFLAMMATORY MYOPATHIES
SYSTEMIC SCLEROSIS
MCTD
Polymyositis
Dermatomyositis
Antisynthetase syndrome
"NonRIM"
Nonerosive arthritis
Raynaud's phenomenon
ILD
Mechanic's Hands
Dx: aminoacyl-tRNA synthetase... most commonly anti Jo1 antibodies
Immune mediated necrotizing myopathy
Inclusion Body Myositis
Dx: 50% with Anti-cytosolic 5′-nucleotidase 1A antibodies
Dx: Prominent myonecrosis on biopsy
SRP antibodies or HMGCOA Reductase
Very high serum CK -- can be triggered with statins
Rx: Steroids
Dx: At least 4 criteria (1 clinical, 1 immunologic) and biopsy proven lupus nephritis in presence of ANA or antidsDNA
(s)erositis
(o)ral ulcers
(a)rthritis (2+ nonerosive peripheral joints
(p)hotosensitvity
(b)lood disorder (leukopenia <4k, lymphopenia <1.5k, thrombocytopenia <100k
(r) enal involvement
(a)NA
(i)mmunologic phenomenon
antidsDNA
anti smith
antiphospholipid ab
(n)euro
(m)alar rash
(d)iscod rash
acute
subacute (antiro/SSA ab)
chronic (usually ANA negative if isolated DLE)
look at dsDNA
Markers
ANA= initial screen
antidsDNA = correlates to severity, esp in kidney disease
anti Ro /SSA - photosensitive rash, DLE. Also in secondary sjogrens
anti U1RNP= raynaud, esophageal dysmotility, MCTD
anti smith - specific, more severe disease, but DOES NOT correlate with disease activity
anti-LA/SSB = Sjogrens
antiribosomal P = CNS lupus, lupus hepatitis
Autoantibody markers
Anticentromere
AntiSCL70 (antitopoisomerase-1)
Anti RNA polymerase III
Subtypes (based on skin involvement)
Ltd cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Systemic sclerosis sine scleroderma
organ only
less internal fibrosis -- most often PAH and CREST
ANA, anticentromere, antiTh
GAVE more common. ILD more common too.
ANA, antiscl70 (higher risk of ILD)
anti RNA polymerase III (scleroderma renal crisis)
antiU3RNP
rx: captopril
Rx: mycophenolate / rituximab / nintedanib
steroids increase risk for renal crisis
Rheumatoid Arthritis
Autoantibodies
10-20% seronegative
RF
AntiCCP
more predictive for erosive disease
DMARD: Nonbiologic
DMARD: Biologic
Methotrexate = anchor drug
Sulfasalazine
Leflunomide
Hydroxychloroquine
Not potent-- but can be used in early disease
Triple therapy = MTX + Sulfasalazine + Hydroxychloroquine
Supposedly comparable to TNF except for radiographic progression
Synergy w/ methotrexate
MTX + TNF alpha
Rituximab/Tocilizumab + MTX
Abatacept
avoid in resp disease
Tofacitinib
Renal crisis = HTN emergency, headache, microangiopathic hemolytic anemia, schistocytes, thrombocytopenia, proteinuria
side effect: peripheral neuropathy
Rx:
Belimumab
Moderate to severe: best for mucocutaneous and joint manifestations