SYSTEMIC LUPUS ERYTHEMATOUS
Epi:
-most commonly in girls >8yo
Sx:
-fever (usually low grade), fatigue, malaise, anorexia, wt loss
-jt pn/stiff +/-edema (usually at smaller jts)
-skin rash (malar facial rash most common, but only in 1/2 of pts at Dx)
-chest pain - bc of pleritispericarditis
-84% have renal disease at time of Dx
-also get htn, Raynaud's, oral/nasal mucosal sores, alopecia, photoSn, heme xx- thrombocytopenia/anemia/neutropenia, GI Sx, CNS/PNS xx
-chorea- might be the only initial Sx
Dx:
-ANA - 95% Sn, but Sp may be low if low titers
-DDx of +ANA - PRA, dermatomyositis, Sjogren, scleroderma, mixed CT dz, transiently high w viral infection, or certain Rx exposure
-if +ANA, thencheck dsDNA, Sm Ag, ribonucleoprtn (RNP, SS-A or SS-B (Anti Sm Ab is Sp to SLE but low Sn)...
Cardiac Sx:
-31% have cardiac sx, 10% serious xx
Pericardium
-Pericarditis- #1 xx - often ASx
-1/4 SLE kids get it
-see mild effusion on echo
-ant. chest pain, dyspnea, incr Sx while supine, big cardiac silhouette, ST-T elevation, decr QRS amp
-usually no HD xx, but sometimes it can be large effusion, tamponade rare
-Fluid: WBC 10-50k, mainly neutros, glucose > 1/2 bld, so must ddx bacterial pericarditis
-Pericarditis DDx
-Rh dz- JRA, demratomyositis, mixed CT dz, acute Rh Fever, PAN, Wegener's, TA, IBD, spondylo's.
-viral pericaridits
-bacterial/TB
-ideo a/i pericarditis
Myocardial Involvement
-Myocardiits- 2-25% of SLE pts
-often more that are subclinical
-occlusion of small intramyocardial arts --> vascullitis, excessive fibrin accum and plt agg, accelerated atherosclerotic plaque formation--> ischemia and then CM
-hemodynamic strain assoc w htn, lupus related anemia, lupus valvulopathy--> myocardium xx
-12% have LVH, 5% LV global dysfx, ...
Valvular Involvement
-35% of pts get valve xx
-3 types of valve xx
-verrucous lesions - Libman-Sacks lesion- benign, on MV and sometimes AoV, no murmur, irregular vegetations 2-4mm diam on vlv & apparatus seen best on TEE, rarely does it embolize to cor/cereb art
-diffuse valve thickening assoc w valve dysfx- more w Anti Phospholipid, and anticardiolipin
-valve regurg w/o thickening/verrucous lesions
Coronary Artery Involvement
-Extramural/Intramural cor arts can have arteritis, accel'd athero, emboli/thrombi, spasm...
-can --> MI at young age
Conduction System Involvement
-arrhythmias less common, AV block, BBB , SSS, sinus tachy, PACs, a-fib all reported
Pulmonary Hypertension
-rare, but well documented w SLE
-? bc of active corticosteroid responsive pulm arteritis, nonvasculitic pulm vasc occlusion, etc
Neonatal Lupus
-congen complete heart block, transient lupus rash
-imm mediated AV ndal tissue damate fr transplacental maternal Ab- SS-A or SS-B
-most of the mothers were ASx despite having the Ab
-fetal brady fr AV block seen - may have CHF/hydrops and pericardial effusion
-postnatally must assess for ongoing active myocardiits- and c/s steroid Tx
-most pts need a pacemaker
-at least 20%of pts w CHB die, most in early infancy, usually fr CHF
-only 1/2 of infants w NLE have CHB
-aside fr the CHB, the rest of the sx resolve in a few months
Tx:
steroids for pericarditis...
JUVENILE RHEUMATOID ARTHERITIS
=a/i dz w immune attack on synovium
Dx:
-arthritis (decr ROM, heat, pain, tender) (not just arthralgia)
-x>6 weeks
3 types:
-Systemic Onset JRA = daily intermittent f to 103F +/- JRA rash or other organ xx +arthritis
-Pauciarticular Onset JRA = 4 or fewer jts
-Polyarticular Onset JRA = 5 or more jts
(aka systemic JIA, oligoarticular JIA, and polyarticular JIA for ea of the above)
Path:
-reports of JRA and pericarditis - with heorrhages on pericardial surfaces and fibrous adhesions bn the surfaces
-One pt w JRA and recurrent myocarditis had hypertrophy, mononuclear and polymorphonuc inflmy infiltrate in myocardial interstitium, and thickened arteriole/venule walls
-In pts w valve leaflet xx- irregular nodular thickening, valve cusp retraction, nonspecific mixed cellular infiltrate w necrosis/fibrosis, fibroblastic prolif, ... +/- vegetations/thrombi
Sx/Si:
Systemic JRA
-20% of pts get systemic JRA
-greatest CV risk
-h/o high fevers for weeks to months, with abrupt temp >103 then decr w/o Tx; looks great when afebrile
-may not get synovitis for weeks or more after fever onset
-some only get polyarthralgia or myalgia before getting synovitis sx
-Rash = multiple small (2-10mm) pale pink macular lesions at the volar aspect of forearms and and/med thighs (but can be anywhere), round usually, but can be linear and clustured where skin is scratched/rubbed. not usually pruritic. evanescent- more w fevers
-Other Sx- pericarditis/myocarditis, pleruitis, fatigue, malaise, LAN, HSM, abd pn, pneumitis on CXR
-WBC >20, pltlt >1.2million, incr ESR, anemia fr ch inflmy dz can be sev (to 7)
-ANA and RF NEGATIVE
Pauciarticular JRA
-usually prschool girl w ch arthritis at a knee with weeks/months of morning stiffness/limp assoc w knee swelling
-no fever, rash or other sx
-some get uveitis
-rare to have CV xx
Polyarticular JRA
-1/3 pts get polyarticular JRA
-many large and small jts, often symmetric
-1/3 are ANA+ and about 1/10 RF+ at Dx
-no uveitis
-often tired/malaise
-no rash usually
-low grade fever only
-cardiac xx do occur but less than systemic JRA
Cardiac Sx
Pericarditis-
-usually w systemic JRA, w onset or during exacerbation
-usually benign and ASx, with only about 7% having Sx
-so many systemic JRA pts have mod to large effusions (20%)
Myocarditis
-uncommon, but can --> CHF and arrhythmias
-11% of JRA deaths were fr myocarditis in one cumulative study
Endocarditis
-vavlar dz is rare
-most get AI, other MR
-most common in RF+ polyarticular JRA
Tx:
-Tx ASx pericarditis w NSAIDs; if mild to mod pericarditis w Sx usually needs steroids
-Sx/Recurrences usually decrease gradually over 1-15 weeks (mean of 7.3weeks), so prednisone course for 8-16 weeks is reasonable..., w a taper...
TAKAYASU ARTERITIS
=ch vasculitis of aorta, proximal part of its branches and of the PA
-mainly in women 15-45yo, but has occurred in infants/children
-rare in caucasians, more in Asians/Mexicans
Phases:
-Acute, active inflmy prepulseless phase
-Chronic pulseless end stage phase
Path:
-Acute phase- granulomatous arteritis at all layers, espec the media w medial lympho infiltrate, giant cells, etc
-Ch Phase- progressive intimal/adventitial fibrosis, scarred/degenerated media which is assoc w segmental stenosis, occlusion, dilation, aneurysm
Sx:
-consitutional: n/v, abd pn, arthralgia/arthritis, myalgia, cough, hemomptysis, pleuritis, skin nodules, episcleritis, HA, neuro xx, LAN, anemia, incr ESR
-Early- Nl periph pulses/BP, aortography may be Nl except show mural thickening at arch
-Then, w ch pulseless phase, see extremity claudication espec at arm, bruits at cervical/supraclavicular, abd regions, absent/decr brachial and radial pulse, >10mmHg SBP diff bn ea arm, decr fem pulse, AI, renovascular htn, CHF, angina if coronary involvement (Rare), hemiplegia, sz, syncope, fundoscopic xx...
-CXR- CM, decr pulm vasc markings, irreg desc Ao, dilated Ao arch, linear calcifications at Ao wall,
-US shows turbulent Q thru carotid arts, abd Ao, subclavians..., dampened distal monophasic flow at brachial arts. Does not show Tx Ao, PAs, sup mesenteric and celiac vssls well and can miss stenotic lesions at branches...,
-Echo- dialted Ao root, cor art xx, AR/MR,
-MRI- good to see mural thickening at Ao/PA, and narrowed abd Ao, occluded caotid arts, narrow subclavian/brachiocephalic arts
-CT- good to screen for Ao wall thickening
-Angiography --> most completeaccurate assessment of Ao and branches
Tx:
-steroids 1-2mg/kg/day of prednisone for 1 month, then a taper
-+/- cyclophosphamide not well established
-at end stage it might be too late to get a steroid response, espec if ESR is Nl, but pt will warrant a trial bc the acute and ch phases overlap...
-must Tx htn and CHF!!
-many pts w bad xx ned surgery for renovasc htn, cerebral hypoperfusion, sev caludication, aneurysm repair
SYSTEMIC JUVENILE POLYARTERITIS NODOSA
-necrotizing vasculatis affecting small/medium sized arteries - kidney, skin, GI, muscle, nervous system
-+/- cor art xx, but not the aorta...
-rare
Path:
-focal, segmental necrotizing vasculitis w fibrinoid necrosis, transmural pleomorphic inflmy infiltrate, mainly at sm-med sized arts
-Thrombosis/microaneurysm formation at site of vessel injury
-healing phase w fibrous tissue/endothelial cell prolif
-"nodosa" bc they initially coulld palpate the inflmy exudate along the arteries, but this only is seen rarely today
-Cardiac xx - arteritis, MI, pericarditis, ventric hypertrophy
Sx/Si:
-usually onset at 1-13yo (pk at 9-11yrs)
-consitutional sx- fever, cutaneous changes w palpable purpuric lesions, red tender nodules at feet/legs, petechiae, livedo reticularis, ulcerations, distal digit ischemic xx; myalgia (often at calves), arthralgia/arthritis, renal xx- proteinuria, hematuria, nephrotic syndrome, RPGN, renal failure; central/periph nerve sys xx (sz, encephalopathy, hemiparesis, cranial n palsy, periph neuropathy), plm xx- infiltrate, effusion, hemoptysis
-Cardiac Sx- pericarditis, MI, tachy, CM, arrhythmia, valvulitis, CHF
-CHF fr MI or renovascular htn
Dx:
-no Sp lab test; often see incrW BC, anemia, incr ESR, protenuria, hematuria, renal failure, elevated LFTs, negative ANA/RF
-need tissue Bx or angio of renal/celiac/mesenteric/recebral/cor art to confirm Dx
Tx:
-steroids for several weeks...