- inflammatory autoimmune disorder

- Hallmark: +ANA (very sensitive: almost 100 are +ANA; not specific: ANA also present in other dz)

- multiple organ systems involved

- antigen-Ab complexes trapped in capillaries of organs 

- antibody destruction of host cells (thrombocytopenia)

- waxing and waning pattern

- mild episodes to rapidly life threatening

• scleroderma
• SLE
• polymyositis/dermatomyositis
• Sjogren
• vasculitis (polyarteritis nodosa, takayasu dz, Buerger dz)

+ANA (95-100%)

+LE cell prep (replaced by ANA, NEVER ordered anymore)

Ab to native DNA (60%) usually means lupus, rarely RA

+ Anti-Sm (10-25%) no other dz has this

+ RF (20%)

False + syphilis test

Depressed serum complement: nl in remission

Smith antigen helps keep DNA in its correct shape while it works to communicate with other cells on how to effectively work

- the antibody to the smith antigen test usually means that lupus is present

• Hydroxychloroquine (plaquenil): 200 or 400mg/d po
  • an antimalarial agent
  • must give annual eye exam (to check for retinal changes caused by plaquenil)
  • Drug induced neuropathy and myopathy may occur
• Danazole: (an androgenic steroid)
  • for thrombocytopenia not controlled by steroids
• DHEA is comparable to plaquenil, but has troubling side effects (acne)

• Systemic corticosteroids (40-60 mg qd)
• Indicated for kidney complications, hemolytic anemia, pericarditis or mocarditis, alveolar hemorrhage, CNS problems and thrombotic thrombocytopenic purpura (TTP) 
• If resistant to steroids use Immunosuppressants:  
  • Cyclophosphamide, mycophenolate mofetil, azathioprine

• Consists of Induction and Maintenance phases
• Cyclophosphamide used for both phases
  • improves renal survival, not pt survival
• Gonadotropin releasing hormone analog 
  • to protect against premature ovarian failure

• 10 year survival rate: 85%
• waxing/waning
• Bimodal distribution of mortality:
  • Early: opportunistic infections leading cause, followed by SLE causing kidney and CNS dz
  • Later: accelerated atherosclerosis, due to chronic inflammation, becomes major caues
• MIs 5x over general population (due to hypercoagulation from vasculitis)
• Treat risk factors for MI
• Flu vaccine annually, pneumococcal q 5 years
• Higher rates of CA: preventative cancer screening must be followed
• Avascular necrosis of bone common (often hip)

• Rheumatologist must be involved
• other specialists as different organs become affected

• Symptomatic and supportive
• No tmt for underlying dz
• Severe Raynauds: CCBs
• GERD: PPIs
• eat small, frequent meals
• Hypertensive crisis in renal crisis: ACEi
• Prednisone has NO ROLE

• Keep body warm
• CCBs (i.e nifedipine 30-180mg/d po) are first line therapy
• others: ACEIs, sympatholytic agents, topical nitrates, phosphidiesterase inhibitors, SSRIs, and endothelin-receptor inhibitors

• a group of findings developed in some polymyositis and dermatomyositis pts
• includes: inflammatory arthritis, Raynaud, ILD, and often severe muscle dz associated with certain autoantibodies (eg, anti-Jo1 Ab)

• Muscle enzyme levels (CK and aldolase) important for dx and dz activity
• elevated ALT and AST
• +ANA (80-95%)
• +RF (33%)
• Anti-Jo-1 (20-30%) specific for these dzs
• Anti-Mi-2 (dermatomyositis specific)
• Anti-SRP (severe polymyositis specific)

• a dz that is pathologically distinct from polymyositis, but that has similar a clinical presentation and tmt
• characteristic dusky red rash may appear in a malar distribution, as well on other areas of ht eface, neck, shoulders, and upper chest and back. 
• Periorbital edema and a purplish suffusion over eyelids are typical
• Periungual erythema, dilations of nail bed capillaries, and scaly patches over the dorsum of PIP and MCP joints are highly suggestive. 
• Dz can easily be mistaken for SLE or psoriasis 
• High risk of malignancy associated with this dz

• a systemic autoimmune dz, predominantly in women (90%) 
• Keratoconjunctivitis sicca and xerostomia are predominant symptoms 
• loss of taste and smell
• systemic manifestations (less common): dysphagia, vasculitis, pleuritis, obstructive lung dz, neruophychiatric dz, pancreatitis, renal tubular acidosis
• Tmt is symptomatic and supportive (artificial tears, polocarpine or cevimeline (an acetylcholine derivative) are useful for dry mouth
• Atropinic drugs and decongestants should be avoided as they decrease salivary secretions

• Necrosis of skeletal muscle associated with crush injuries to muscle, prolonged immobility, drug toxicity's (ie statins), hypothermia, and other causes
• Massive acute elevations of muscle enzymes 
• can result in acute renal injury, which is almost always associated with hypovolemia
• Tmt: vigorous fluid resuscitation, mannitol and urine alkalinization 

• a heterogenous group of disorders characterized by inflammation within the walls of the affected blood vessels. 
• each predominantly affects either small, medium or large vessels. This is the 1st consideration when narrowing down which dz is present
• From there, symptoms and physical findings are used to determine the dz
• all present with constitutional sxs: fever, wt loss, malaise, arthalgias/arthritis
• Bx can be diagnosits

•  Limb claudication
• Asymmetric blood pressures
• Absence of pulses
• Bruits
• Aortic dilation

• Cutaneous nodules
• ulcers
• livedo reticularis (mottled reticulated vascular pattern, appears as lace-like purple discoloration on the legs)
• Digital gangrene
• Mononeuritis mulitplex
• Microaneurysms

• Purpura (red/purple skin discolorations)
• vesiculobullous lesions (fluid filled lesions)
• uriticaria
• Glumerulonephritis
• Alveolar hemorrhage
• cutaneous extravascular necrotizing granulomas
• splinter hemorrhages
• uveitis
• episcleritis
• scleritis

• Vasculitis always affecting medium arteries, and sometimes small arteries
• pain in extremities is often a prominent early symptom. 
• mononeuritis multiplex (most common finding = drop foot) + systemic illness is suggestive 
• kidney is often affected, causing renin-mediated HTN
• common features include fever, abd pain, livedo reticularis mononeruitis multiples, anemia, and elevated acute phase rxns (ESR or CRP)
• Associated with Hep B (10%)
• Prognosis: without tmt 5yr survival = 10%, with tmt = 60-90%

• Corticosteroids: Prednisone 40-60 mg/d po 
  • control fever, constitutional sxs and heal vascular lesions
• Pulse Methylprednisolone (ex: 1g IV daily for 3 days) for pts who are critically ill at presentation
• Immunosuppressive agents, esp cyclopsosphamide, lower risk of dz related death for those with severe dz

For isolated dz (w/o giant cell arteritis)

• Prednisone 10-20 mg/d po x 2-4 weeks then slowly taper 
• most pts must remain on for 1 year
• addition of weekly methotrexate may help taper off prednisone

• Immediate high dose steroid and bx
  • to prevent blindness, bx to dx
  • Prednisone 60mg qd po x 1 month then try to taper (monitor ESR)
• If visual loss has occurred: IV pulse methylprednisolone 
• No steroid sparing tmt has been identified yet

• Prednisone (1 mg/kg daily) and cyclophosphamide
• Then, azathioprine or methotrexate to maintain remission

• most common in asians, turkish or middle eastern background
• all manifestations believed to be due to vasculitis of small, medium, and large venous and arterial vessels
• Recurrent, painful aphthous ulcers of mouth & genitals
• Additional cutaneous findings include erythema nodosum-like lesions, a follicular rash, and the "pathergy" phenomenon (formation of a sterile pustule at the site of a needle stick)
• Either anterior or posterior uveitis
• Variety of necrologic lesions that can mimic MS, esp involvement of white matter of brainstem

• Corticosteroids are mainstay
• Azathioprine effective steroid sparing agent 
• Cyclophosphamide or chlorambucil for severe dz
• Colchicine and thalidomide for mucocutaneous findings

• inflammatory joint diseases of the vertebral column, associated with HLA-B27. 
• Seronegative is used b/c they may mimic rheumatoid diseases, but these pts are Rh factor -
• Ex: ankylosing spondylitis, Psoriatic arthritis, Reactive arthritis (Reiter syndrome) 

Elevated EST (85%)

Rh -

HLA-B27 (90% of whites, and 50% of blacks, but not specific)

• earliest changes at sacroiliac joints (1st 2 years only detectable by MRI (b/l and symmetric)
• Bamboo spine describes late radiographic appearance of the spinal column: vertebral bodies are fused by vertically oriented bridging syndesmophytes formed by ossification of the annulus fibrous and calcification of the anterior and lateral spinal ligaments

• NSAIDs
• TNF inhibitors for NSAID resistant axial dz
• Etanercept, adalimumab or infliximab for pts whose sxs are refractory to NSAIDs
• sulfasalazine for peripheral arhtritis

• NSAIDs alone for mild cases
• Methotrexate if no response to NSAIDs (improves both cutaneous and arthritic manifestations)
• TNF inhibitors added if refractory to methotreate for both arthritis and psoriatic dz
• Alefacetp may also help
• Steroids not useful

• NSAIDs are mainstay
• Antibiotics given at time of nongonococcal STI reduce chance of development of this disorder, but once developed, antibiotics do not alleviate sxs