Systemic Lupus Erythematosus. Systemic Lupus Erythematosus. Autoimmune disease Organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes 90% are women of child-bearing years In the US: 15-50 per 100,000 highest prevalence in African Americans.
(1) activation of innate immunity (dendritic cells) by CpG DNA, DNA in immune complexes, and RNA in RNA/protein self-antigens;
(2) lowered activation thresholds of adaptive immunity cells (antigen-specific T and B lymphocytes);
(3) ineffective regulatory and inhibitory CD4+ and CD8+ T cells;
(4) reduced clearance of apoptotic cells and of immune complexes
E.G., 21 Female
Came in with a chief complaint of SEIZURES
A diagnosed case of systemic lupus erythematosus since December 2008
Initially presented with
malar rash, photosensitivity, arthralgia, hair loss and oral ulcers, edema of both lower and upper extremities, weakness
-> was ANA (+)
prednisone 10 mg once a day
hydrochloroquine 200 mg once a day
Regular follow up in the OPD (Fammed and Rheuma)
(PU 14 1/7 weeks AOG, G1P0)
on follow-up at the PGH OB HRC, last seen February 17, 2010
2 WEEKS PTA
(+) decreased appetite
(+) gradually increasing generalized body weakness
(-) interventions or consults
4 DAYS PTA
generalized weakness now more prominent on both right upper and lower extremities
(+) behavioral change
(+) general decrease in activity
(+) episodes of staring blankly into space
(+) cough productive of whitish phlegm
(+) febrile episodes (qualitative)
(-) nasal congestion
2 HOURS PTA
(+) seizure - upward rolling of the eyeballs, grinding of teeth and clenched fists (duration ~5 minutes)
-> Promptly rushed to PGH, hence this admission
(-) urinary incontinence, (-) polyuria/nocturia, dysuria (-) dribbling, (-) tea colored urine,
(+) vaginal spotting (Feb 14, 2010)
(+) SLE, 2008
(-) HPN, DM, goiter, BA, PTB, Ca,
(-) heart problems
FAMILY MEDICAL HISTORY
(-) SLE, HPN, DM, goiter, BA, Ca, heart problems
Pt lives with live-in partner, a college graduate (BA HRM), currently unemployed.
Non-smoker, occasional alcoholic beverage drinker.
(-) known exposure to chemicals, and radiation
LMP: October 9, 2009 , PU 14 1/7 weeks AOG by LUTZ
2/28: sinus tachycardia, normal axis, low voltage complexes
3/11: sinus tachycardia, normal axis, low voltage complexes
3/12: sinus tachycardia, normal axis, low voltage complexes
2D ECHO: concentric LVH with good wall motion and contractility, EF=73% minimal pericardial effusion, mild pulmo HPN, incidental finding of pleural effusion, mild TR, PR.
CRANIAL CT – enhanced: unremarkable
Systemic lupus erythematosus in activity
t/c SLE cerebritis, myocarditis
t/c sec APAS
Community Acquired Pneumonia
r/o SOL vs electrolyte imbalance vs CNS infection as cause of seizure
Anemia of chronic disease
Severity varies from mild and intermittent to severe and fulminant
Exacerbations interspersed with periods of relative quiescence
Permanent complete remissions (absence of symptoms with no treatment) – rare
Systemic symptoms, particularly fatigue and myalgias/arthralgias – present most of the time
Severe systemic illness requiring glucocorticoid therapy can occur with fever, prostration, weight loss, and anemia with or without other organ-targeted manifestations.
discoid lupus erythematosus (DLE), systemic rash, subacutecutaneous lupus erythematosus (SCLE), or "other"
Normochromic, normocytic anemia
Lymphopenia, not granulocytopenia
SLE or not?
Diffuse process of vascular occlusive disease?
Autoimmune peritonitis and/or intestinal vasculitis
Inc in AST and ALT
Retinal vasculitis and optic neuritis – threaten vision
(1) to establish or rule out the diagnosis;
(2) to follow the course of disease, particularly to suggest that a flare is occurring or organ damage is developing; and
(3) to identify adverse effects of therapies
Activated complement products
Urinary adiponectin or monocytechemotactic protein 1
(1) whether disease manifestations are life-threatening or likely to cause organ damage, justifying aggressive therapies;
(2) whether manifestations are potentially reversible;
(3) the best approaches to preventing complications of disease and its treatments
venous or arterial clotting, and/or repeated fetal losses, and at least two positive tests for aPL have APS
Target INR of 2-2.5 (1 epi of venous clotting)
INR 3-3.5 (recurring clots or arterial clotting)
Thrombotic Thrombocytopenic Purpura
Hemolytic Uremic Syndrome
High mortality rate
Young, with lupus nephritis
Labs: identification of schistocytes on PBS, elevated LDH
Tx: plasma exchange or extensive plasmapheresis
minimize exposure to ultraviolet light
sunscreens with a sun protection factor of at least 15
Topical glucocorticoids and antimalarials
Systemic retinoic acid
In therapy resistant: topical tacrolimus, systemic dapsone, or thalidomide
Suppressing recurrent UTI
Prevention of osteoporosis
Control of hypertension
Management of hyperglycemia, dyslipidemia, and obesity
Disability is common
25% may experience remissions
Leading cause of death in 1st decade – systemic disease activity, renal failure, infections, thromboembolic
received GCS 5 E2V1M2
BP 100/70, HR 88, RR 18; CBG 163
given Diazepam 5 mg IV
intubated with note of yellowish secretions per ET tube
GCS 10, E4V1M5 bilateral rhonchi, (+) Babinski, left and decorticate posturing, right.
A> PU 14 1/7 weeks AOG, G1P0; SLE not in activity, t/c SLE cerebritis. Referred to POD.
benefits of MPPT treatment was deemed to supercede risks
MPPT treatment allowed to start
S> awake but with (-) verbal output and regard
BP 120/80, HR 118, RR 20,
(+) crackles BLF,
tachycardic with S3 gallop,
no lateralizing signs.
A> SLE in activity, (cerebritis, myocarditis),
P> Recommended MPPT regimen:
500mg methylprednisolone + 250cc D5W x 6 D1 500mg methylprednisolone + 250cc D5W x 4 D2 500mg methylprednisolone + 250cc D5W x 4, D3
received awake with no verbal output
BP 100/70, HR 132, RR 24
rhonchi on bilateral lung fields.
put on O2 support via nasal cannula
1. Pip-tazo 4.5 gm IV q8
2. Hydrocortisone 100 mg IV q8
3. Omeprazole 40 mg tab 1 tab OD
4. CaCO3 1 tab OD
5. NAC 200 mg sachet, 1 sachet + 50 cc water TID 6. Salbutamol neb Q6.
Feeding started via NGT.
2D echo results: concentric LVH with good wall motion and contractility, EF=73% minimal pericardial effusion, mild pulmo HPN, incidental finding of pleural effusion, mild TR, PR.
Cardio service no longer highly considering myocarditis.
Neuro: diazepam 5mg IV for frank seizures, leviteracetam 500mg tab 1 tab BID.
Rheuma: SLE activity in the patient are cerebritis, nephritis and serositis (due to pericardial and pleural effusion seen on 2D echo). Plan for MPPT therapy, to hold hydroscortisone while on MPPT.
dyspneic episodes not relieved by nebulization but not associated with desaturation.
Breath sounds were noted to be harsher.
She was given Furosemide 40mg and there was noted improvement in dyspnea.
Patient awake, conversant, comfortable in bed, still with dyspneic episodes and harsh breath sounds on chest PE.
new labs: Urine CS – No growth after 2 days;
ET CS – moderately heavy growth of S. aureus (sensitive to Clindamycin, Erythromycin, Gentamicin, Ofloxacin resistant to penicillin.
S> blurring of vision, OS>OD.
Still with no recurrence of seizure,
(-) headache, vomiting, LOC.
O> Oriented to time and place, neurologic PE: normal.
Motor strength: from 4/5 to 5/5 on all extremities.
P> Patient referred to Ophtha for the BOV.
Clindamycin discontinued on the basis of sufficient coverage by Pip-tazo as recommended by IDS,
S> (-) seizures, vomiting, headache, LOC (+) mild DOB
O> BP 100-120/70-80, HR 130-140, RR 24-40 38.2C
HBS, and occasional crackles and rhonchi on bilateral lung fields. fever
Tachycardic, normal rhythm, no murmurs.
A> SLE in activity (nephritis, serositis, hematologic, t/c cerebritis, NPSLE); pneumonia in the ICH, r/o APAS, UTI, PU 15 4/7 wks AOG by EUTZ, NIL.
OB-GYN cleared pt for CT scan.
Fetal biometry and viability recommended
VA 20/25 (near vision chart), eoms full and equal, tonometry soft OU. ; Slit beam: AC formed, cornea looks clear, lens look clear, (-) dye uptake. ; On indirect fundoscopy, clear media, distinct disc borders, CD ratio 0.3, AV 2:3, (+) exudates on vessels, (-) hemorrhages, retina attached.
t/c SLE retinopathy, OU.
Patient’s serum K found to be 2.9, correction started for a K deficit of 222. Oral KCl 30cc q8 x 5.
O> dyspneic, tachycardic, tachypneic. (+) harsh breath sounds, crackles and rhonchi all over both lung fields. Hemoglobin decreased from 81 to 78. Crea 100 Mg 0.89 Na 144 K 2.9. P> for BT with 2 units pRBC.
3:00 PM, the pt was referred for DOB -> nebulized with 1 neb salbutaol, given 40 mg furosemide IV.
3:35 PM, the pt was persistently dyspneic with 02 saturation decreasing from 95 -> 87 -> 84%. Pt was intubated ET size 7.5, level 21. Subsequently hooked to mech vent with the following settings: AC mode, FiO2 100%, BUR 16, IFR 60, PEEP 5, TV 450cc. (+) copious whitish to yellowish secretion per ET. : BP 100-120/70-80, HR 160s, RR 20 CAB, O2 sat 100%.
Pt was also sent to radio for CT scan after intubation. Cranial CT scan results: Unremarkable.
Patient was referred for desaturation, noted to be dyspneic. Suction was done, found to have mucus plug, and ET out. Patient was reintubated with ET size 7.5, level 20, tolerated very well, hooked to MV with settings: AC mode FiO2 40%, BUR 16, IFR 60 PEEP 5, TV 450 cc (+) copious whitish secretions per ET.
Patient became persistently tachypneic (30s): MV settings were adjusted as follows: SIMV, FiO2 35%, TV 400, BUR 12, PEEP 5, I:E 1:2.
Patient’s K was low at 2.7. This was corrected with 60 mEqs K x 1L pNSS x 10H x 3 cycles.
There was also note of harsh breath sounds despite regular suctioning, along with a spike in temperature, on ave 39C, which remained constant throughout the day, sepsis was a strong consideration so a shift of antibiotic medication from Pip-Tazo to Meropenem was ordered to provide wider and stronger coverage of nosocomial pathogens
Patient arrested and was revived after 7 minutes, 2 epinephrine ampoules were given. Post ACLS: BP 110/70, HR 136, O2 sat 100%, ECE, CBS. Suctioning was done with no resistance and minimal brownish secretions per ET.
Possible cause of code: NFA secondary to Hypokalemia, r/o PE. MICU ROD then changed MV settings to: AC mode FIO2 100%, BUR 16, IFR 60, PEEP 5, TV 400cc. She was also given Heparin 3600 ‘u’ as IV bolus then started on Heparin drip: 10000 ‘u’ heparin + enough pNSS to make 100cc in a soluset to run for 7cc/hr.
Post-code, she was E1V1M1. Also on PE, there was note of an S3 gallop.
Post-code ECG showed ST, NA, low voltage complexes.
Her ECG at 9am had similar findings: ST, NA, low voltage complexes.
Electrolytes were obtained revealing high K at 7.7 so KCl drip was discontinued.
She had a complete abortion at 1pm and was immediately seen by the OB service, there was no uterine atony. Stat Hgb and Hct were 61 and 0.148 respectively, and she had hypotensive episodes (lowest 70/50) and was immediately given 500cc fast drip of pNSS and so 2’u’ pRBC were processed as quickly as possible. She also had Dopamine on standby, 400mg in 250cc D5W, to run at 38cc/hr max, to be down titrated by 2cc/hr until it reaches 10cc/hr if BP remains above 90/60.
Her PWI was revised to: