RHEUMATOLOGICAL Emergency. Dr.Majeed Shekhany 7 th Jan 2014. CASE 1. History. 56 year old male with HTN presents with painful swollen L ankle. The pain started abruptly yesterday. The edema has been progressive and initially started in the L great toe.
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7th Jan 2014
56 year old male with HTN presents with painful swollen L ankle. The pain started abruptly yesterday. The edema has been progressive and initially started in the L great toe.
Similar episodes have occurred over past 1 year. They involved the R wrist, R elbow and L knee and always resolved after a few days after taking Motrin.
PMH/PSH: htn, angina
MEDS/ALL: nkda; on hctz, metoprolol, aspirin
SH: neg x2, works in real estate and only drinks with his clients, the family, the neighbors, etc; lives with wife and 2 children
FH: htn, cad, renal stones, brother is alcoholic
t 101, bp 160/100, wt 210 lbs
Exam wnl overall
Purple and tender L first MTP joint with erythema and swelling up the foot to the ankle
Enlarged bursa on R elbow
b. Crystal induced arthritis
c. Fungal septic arthritis
d. Bacterial septic arthritis
e. Rheumatoid arthritis
g. Reiter’s syndrome
h. Prodrome of hepatitis B
a. CBC, SMA 12, UA
c. ANA, RF
e. Blood cultures
f. X-ray the feet
g. X-ray the hands
h. Aspirate the MTP joint
WBC 12,200 (85% PMN, 10% Bands), Hct 41%
Ast 60, Alt 73, Alk phos 225
Creatinine 1.2, UA wnl
L 1st MTP joint arthrocentesis 3 drops of fluid with PMN’s on gram stain but no organisms
X ray L foot soft tissue swelling and bony erosion of 1st MTP joint slightly removed from the joint with overhanging edge
Under polarizing microscopy see:
Intra- and extracellular needle shaped crystals
Yellow when aligned parallel to the slow axis of rotation
More specifically, what does your patient have in common with Michelangelo?
Raphael’s fresco at the Vatican “School of Athens” 1509
First described 5th century BC by Hippocrates
Uric acid overproduction OR underexcretionurate deposition in joints (first MTP joint of great toe) and kidneys with primary and secondary causes
Obese alcoholic male with DM, HTN, HL abusing his medications stumbling around like his father with a painful toe
Diagnosis by aspiration of joint or can be made clinically
Terminate acute attack nsaids, steroids, +/- colchicine
Prevent recurrence xanthine oxidase inhibitor (allopurinol), uricosoruic (probenecid), dietary modifications
Prevent complications goal uric acid < 6.4 mg/dl
Reverse associated conditions control DM, HTN, etoh abuse, etc
34 yo male with IDDM presents with 3 hours of pain and swelling in the L knee and R shoulder. The pain started after an episode of chills.
One day prior he had twisted his L knee and scraped his R elbow.
PMH/PSH: juvenile onset DM
MEDS/ALL: nkda, on insulin
SH: neg x 3, is sexually active, works as a lawyer
PE: t 102, p 110, Appears ill overall
1x1cm carbuncle on the neck
R shoulder ROM limited because of pain with focal tenderness
Superficial skin abrasion over R olecranon
L knee swollen, red, warm, tender with decreased ROM due to pain but no instability
Wbc 20, 000 with 70% PMN, 20% bands
UA- glucose 3+
a. Rheumatoid arthritis
b. Ankylosing spondylitis
c. Septic arthritis
d. Hemarthrosis of shoulder and knee
e. Biceps tendon rupture
f. Medial meniscus tear
g. Anterior cruciate ligament rupture
h. Rotator cuff tear
i. Reiter’s syndrome
j. Acute gout
k. Diabetic neuropathic arthropathy
a. MRI of the knee
b. Bone scan
e. X- rays of shoulder and knee
f. Blood culture
g. Skin abscess culture
h. Chest x-ray
i. AP pelvis x-ray
j. Serum uric acid
X-rays of both joints normal articular surfaces with effusion and soft tissue swelling evident on knee films
Arthrocentesis of both joints yellow cloudy appearance
80,000 Wbc with 95% PMNs
Gram + cocci in clumps and chains
What does your patient have in common with the Summoner in the Canterbury Tales?
From the Ellesmere manuscript (15th century) of the Canterbury Tales by Geoffrey Chaucer (14th century)
Arthritis due to seeding of a joint by a bacteria, mycobacterium or fungus usually via hematogenous spread.
Classify as gonococcal and non-gonococcal
Adult G (-) Cocci 50% N. Gon
G (+) Cocci 45% Staph A or Strep Pneumo
Prosthetic joints Staph epi or Staph aureus
cancer, DM, liver disease, SLE, sickle cell disease, complement deficiency
RA, gout, pseudogout, OA, hemarthrosis
Regular introduction of organism
IVDA, pregnancy, menses, etc
Choose 3 courses of action before the synovial fluid culture and sensitivity test results are available
a. Admit to hospital
b. Oral penicillin V with bed rest at home
c. IV penicillin
d. IV vancomycin alone
e. Aminoglycoside and 3rd Gen cephalosporin
f. IV nafcillin plus aminoglycoside in hospital
g. Daily needle aspiration
h. Joint lavage
i. Surgical debridement and drainage
j. IV colchicine
k. Intraarticular steroids
l. High dose prednisone for 1 week
72 yo male complains of headaches and muscle pains.
The headache started 3 months ago when he was involved in a MVA. He has had daily R sided headaches since that are now worsening and most severe in the evening but are present upon awakening.
Tylenol does not help. He also has some general malaise, 5 pound weight loss, aching around the hips and shoulders and morning stiffness in the legs.
MEDS/ALL: nkda; on hctz, asa
SH: smokes a pipe, occasional etoh use
a. Hypertensive encephalopathy
b. Subdural hematoma
c. Brain tumor
e. Giant cell arteritis
h. Rheumatoid arthritis
i. Cerebral artery aneurysm
Low grade temperature (99-100ºF)
He did have an episode of decreased vision in the L eye and pain on the R side of his face when he chews his food. He also states his scalp hurts when he combs his hair.
t 37.6, p 100, bp 150/95 R, 145/90 L
Fundoscopic exam: arteriolar narrowing without hemorrhage or exudate, optic discs well demarcated.
Slight R ptosis with tenderness to palpation along temporal area. Tenderness around the shoulder girdle with no true muscle weakness.
b. CBC with diff
d. Serum creatinine
h. Rheumatoid factor
i. Spinal fluid analysis
j. Chest x ray
k. Brain MRI
l. Cerebral angiogram
What does your patient have in common with this Bishop?
Virgin with Canon Van der Paele by Jan Van Eyck 1436
Chronic, patchy vasculitis of medium and large sized vessels mostly in patients over the age of 50
Most commonly presents with polymyalgia rheumatica in 40% of cases
PMR= symmetrical pain/stiffness in shoulder and pelvic girdle muscles for more than 1 month in a patient age > 50 with elevated ESR with rapid response to steroids
Cranial and peripheral neuropathies
Rare organ involvement
Diagnose with 3/5 of the following:
Age > 50 years
Temporal artery abnormality
ESR > 50mm/hr
Abnormal artery biopsy with mononuclear cell infiltrate, granulomatous inflammation, usually with multinucleated giant cells
b. Oral analgesics
c. IV steroids
d. Temporal artery biopsy now
e. Temporal artery biopsy at later date
h. Referral to neurology
i. Oral steroids
Oral prednisone 1mg/kg/day
IV steroids if visual loss, etc
Obtain temporal artery biopsy within 7-14 days
Follow symptoms and ESR
Cytotoxic agents if steroid resistant
Monitor for late thoracic aortic aneurysms
Relapses in 60%
36 yo female with 6 month history of Raynaud’s phenomenon. She also complains of pain in the wrists, fingers and ankles.
She has daily morning stiffness x 45 minutes and has bilateral hand swelling.
PMH/PSH: G1P1001, nsvd x 1, regular menses, h/o appy
MEDS/ALL: nkda, on mvi daily
SH: smoker, neg x 3, no blood transfusions
FH: cousin with SLE
bp 110/70, t 37.8, p 88, overall in nad.
Edema of hands, fingers, legs from the knees down.
Skin thickening of hands extending to the elbows with periungal erythema present
a. Giant cell arteritis
b. Rheumatoid arthritis
e. Sjögren’s syndrome
g. Hepatitis b prodrome
h. Ankylosing spondylitis
i. Polyarteritis nodosa
j. Systemic lupus erythematosus
c. Liver enzymes
j. Anti-centromere Ab
l. Hand films
m. Nailfold capillary microscopy
ANA 1:2560 nucleolar
Anti dsDNA neg
Anti centromere neg
Anti SCL 70 positive
What does your patient have in common with the artist of this painting?
Embrace 1939 by Paul Klee (1879-1940) Pioneer of modern art
Endothelial disruption and fibroblast proliferation with unknown etiology
Localized - cutaneous only
Systemic - internal organ involvement
Limited/CREST pulmonary htn, GI issues
Diffuse ILD, cv disease, renal disease,joint deformities, myopathy
Diagnosis = clinical sclerosis +/- organ involvement + auto antibodies
(older method outdated)
Treatment really symptomatic
Calcium channel blockers
Proton pump inhibitors
Manage pulmonary, CV issues
Patient’s skin thickening has progressed to mid upper arm
Hct 30%, Plt 85,000
UA 1+ protein, 5-10 RBCs
Patient now comes back with sob and malaise
Admitted to hospital
Microangiopathic changes noted on peripheral smear
Intubated for congestive heart failure
What is your diagnosis at this time?
Acute renal crisis after prolonged htn
Lead to retinopathy, rapid renal failure
More in the fall/winter months ~ Raynaud’s
Early on in disease course
a. High dose steroids
c. Ace inhibitor
Ace inhibitor was started
BP remained around 130/80
Renal function deteriorated further
WHAT WOULD YOU DO NEXT?
Renal function impairment felt to be due to ACE inhibitor
ACE inhibitor stopped
BP increased dramatically
Renal function deteriorated and HD was started
WHAT WOULD YOU DO NOW DOCTOR?
ACE inhibitor was restarted
Over the next 9 months:
Continued ACE inhibitor kept BP normal
Began to produce urine
Renal function improved
HD stopped 1 year after initial crisis
Accelerated arterial htn
rapidly progressive renal failure
increased plasma renin
CHF, asymptomatic pericardial effusions
headache, fundoscopic changes, seizures
Rapid progression of skin thickening
New cardiac events (CHF/pericardial effusions)
Possibly high dose steroids
Early diagnosis and treatment
If renal dysfunction progresses
Continue ACE Inhibitors
Steroids/plasmapheresis NOT indicated
35 yo female with R sided weakness x 12 hours.
She denies fevers, head trauma, headaches, stiff neck, visual disturbance, Raynaud’s, sicca symptoms, joint pains, skin rash with ROS otherwise negative.
PMH/PSH: G4P2022, nsvd x 2, sab x 2
First pregnancy c/b postpartum bleeding and diagnosed with autoimmune ITP that was treated with high dose prednisone and splenectomy
Second pregnancy c/b DVT
MEDS/ALL: nkda, currently on no meds
SH: + tobacco, 1 ppd x 5 yrs, neg x 2
t 98, bp 125/80
Mild facial droop with slight dysarthria
Flaccid weakness of R upper and lower extremities, no DTR’s, no Babinski
Rest of exam wnl
a. Bacterial endocarditis
b. Cerebral hemorrhage
c. Primary CNS vasculitis
e. Antiphosphlipid antibody syndrome
f. Subdural hematoma
g. CNS infection
a. CBC with diff
c. CXR and EKG
e. MRI brain
f. Blood cultures
Wbc 12,500, normal diff
PTT 45 (H)
Blood cx neg
Echo no vegetations, mass, pericardial effusion
MRI shows acute cva on L
a. CSF analysis
c. Tests for coagulation abnormalities
e. MR angiogram
f. Anti-cardiolipin antibody
g. Lupus anticoagulant
h. Beta 2 glycoprotein I
ACL IgG 100 (nl < 15)
Lupus anticoagulant positive
Coag panel negative
MR angio occlusion of the pericallosal artery with avascular areas in the L fronto-parietal region with no evidence of vasculitis/atherosclerosis
What does your patient’s disease have in common with Budapest, Hungary?
Fisherman’s Bastion 1905
But before we get there, what are antiphospholipid antibodies?
Group of antibodies with affinity for phospholipids
Presence of aPL
Thrombosis or recurrent fetal loss or thrombocytopenia
With no other underlying disorder as the cause of the vascular thrombosis
(ex SLE with LA)
> 1 thrombotic epidsode AND
confirmed by imaging AND absence of vessel wall inflammation
> 1 unexplained fetal death beyond 10 wks GA OR
> 1 premature fetal birth before 34th wk due toecclampsia or placental insufficiency OR
> 3 unexplained consecutive SAB’s before 10 wkGA
ACL Ab IgG/M isoptype present on 2 occasions 6 wks apart OR
Lupus anticoagulant present on 2 occasions 6 weeks apart
Definite APAS if at least 1 clinical and 1 lab criteria met
heparin coumadin goal INR 2.5-3
high dose prednisone AND
heparin coumadin PLUS
plasmapheresis or IVIG
What your mind does not know, your eyes will not see.
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