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