Rheumatological emergency
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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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Rheumatological emergency

RHEUMATOLOGICAL Emergency

Dr.Majeed Shekhany

7th Jan 2014


Case 1

CASE 1


History

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.

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.


Rheumatological emergency

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


Physical

Physical

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


What are the 2 most reasonable choices in the differential diagnosis

a. Trauma

b. Crystal induced arthritis

c. Fungal septic arthritis

d. Bacterial septic arthritis

e. Rheumatoid arthritis

f. Osteoarthritis

g. Reiter’s syndrome

h. Prodrome of hepatitis B

What are the 2 most reasonable choices in the differential diagnosis?


What would be your next step

a. CBC, SMA 12, UA

b. Arthroscopy

c. ANA, RF

d. ESR/CRP

e. Blood cultures

f. X-ray the feet

g. X-ray the hands

h. Aspirate the MTP joint

What would be your next step?


Labs tests

Labs & Tests

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


Rheumatological emergency

Under polarizing microscopy see:

Intra- and extracellular needle shaped crystals

Yellow when aligned parallel to the slow axis of rotation


What is your diagnosis doctor

What is your diagnosis Doctor?

More specifically, what does your patient have in common with Michelangelo?

Raphael’s fresco at the Vatican “School of Athens” 1509


Rheumatological emergency

Gout

First described 5th century BC by Hippocrates

Uric acid overproduction OR underexcretionurate 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


Gout treatment

Gout Treatment

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


Case 2

CASE 2


History1

History

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.


Rheumatological emergency

PMH/PSH: juvenile onset DM

MEDS/ALL: nkda, on insulin

SH: neg x 3, is sexually active, works as a lawyer

FH: nc


Physical1

Physical

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


Stat labs

Stat Labs

Wbc 20, 000 with 70% PMN, 20% bands

Hb 15

PT normal

Glucose 350

Creatinine 1.2

UA- glucose 3+


The most reasonable diagnosis are

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

The most reasonable diagnosis are:


Which diagnostic tests would you order

a. MRI of the knee

b. Bone scan

c. Arthrocentesis

d. Arthroscopy

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

Which diagnostic tests would you order?


Diagnostics

Diagnostics

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


Classes of synovial fluid

Classes of Synovial Fluid


What is your diagnosis doctor1

What is your diagnosis Doctor?

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)


Septic arthritis

Septic Arthritis

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


Conditions that predispose a patient to joint infections

Conditions That Predispose a Patient to Joint Infections

Immune suppression

cancer, DM, liver disease, SLE, sickle cell disease, complement deficiency

Joint destruction

RA, gout, pseudogout, OA, hemarthrosis

Regular introduction of organism

IVDA, pregnancy, menses, etc


Treatment of septic arthritis

Treatment of Septic Arthritis

Choose 3 courses of action before the synovial fluid culture and sensitivity test results are available


Rheumatological emergency

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


Case 3

CASE 3


History2

History

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.


Rheumatological emergency

PMH/PSH: htn

MEDS/ALL: nkda; on hctz, asa

SH: smokes a pipe, occasional etoh use

FH: nc


What is in our differential diagnosis

a. Hypertensive encephalopathy

b. Subdural hematoma

c. Brain tumor

d. CVA

e. Giant cell arteritis

g. Migraine

h. Rheumatoid arthritis

i. Cerebral artery aneurysm

j. SLE

What is in our differential diagnosis?


Further history elicited

Further History Elicited

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.


Physical2

Physical

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.


Which of the following tests would be abnormal

a. ESR/CRP

b. CBC with diff

c. UA

d. Serum creatinine

e. CPK

f. ANA

h. Rheumatoid factor

i. Spinal fluid analysis

j. Chest x ray

k. Brain MRI

l. Cerebral angiogram

Which of the following tests would be abnormal?


What is your diagnosis doctor2

What is your diagnosis Doctor?

What does your patient have in common with this Bishop?

Virgin with Canon Van der Paele by Jan Van Eyck 1436


Giant cell arteritis

Giant Cell Arteritis

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


Gca clinical findings

SYSTEMIC FINDINGS:

PMR

Fever

Anorexia

Malaise

Weight loss

Elevated ESR

Abnormal lfts

LOCAL MANIFESTATIONS:

Temporal headache

Blindness

Scalp necrosis

Jaw claudication

Cranial and peripheral neuropathies

Rare organ involvement

GCA Clinical Findings


Diagnosis of gca

Diagnosis of GCA

Diagnose with 3/5 of the following:

Age > 50 years

New headache

Temporal artery abnormality

ESR > 50mm/hr

Abnormal artery biopsy with mononuclear cell infiltrate, granulomatous inflammation, usually with multinucleated giant cells


Now what is your next step

a. Anticoagulation

b. Oral analgesics

c. IV steroids

d. Temporal artery biopsy now

e. Temporal artery biopsy at later date

f. Aspirin

h. Referral to neurology

i. Oral steroids

Now what is your next step?


Treatment of gca

Treatment of GCA

Corticosteroids

Oral prednisone 1mg/kg/day

IV steroids if visual loss, etc

Obtain temporal artery biopsy within 7-14 days

Follow symptoms and ESR


Treatment of gca cont d

Treatment of GCA (cont’d)

Cytotoxic agents if steroid resistant

Methotrexate

Azathioprine

Cyclophosphamide

Monitor for late thoracic aortic aneurysms

Relapses in 60%


Case 5

Case 5


History3

History

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.


Rheumatological emergency

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


Physical3

Physical

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

No synovitis


List the most reasonable diagnoses

a. Giant cell arteritis

b. Rheumatoid arthritis

c. Polymyositis

d. Dermatomyositis

e. Sjögren’s syndrome

f. Scleroderma

g. Hepatitis b prodrome

h. Ankylosing spondylitis

i. Polyarteritis nodosa

j. Systemic lupus erythematosus

List the most reasonable diagnoses:


What tests would you choose next

a. CBC

b. BMP

c. Liver enzymes

d. UA

e. ESR/CRP

f. RF

g. ANA

h. Anti-dsDNA

i. Anti-SCL-70

j. Anti-centromere Ab

k. TSH

l. Hand films

m. Nailfold capillary microscopy

What tests would you choose next?


Rheumatological emergency

Hgb/Hct 13.1/40.2

WBC 7,800

Plt 265,000

Creatinine 0.9

UA wnl

ANA 1:2560 nucleolar

Anti dsDNA neg

Anti centromere neg

Anti SCL 70 positive

Labs


What is your diagnosis now doctor

What is your diagnosis now Doctor?

What does your patient have in common with the artist of this painting?

Embrace 1939 by Paul Klee (1879-1940) Pioneer of modern art


Scleroderma

Scleroderma

Endothelial disruption and fibroblast proliferation with unknown etiology

Localized - cutaneous only

Morphea

Linear

Systemic - internal organ involvement

Limited/CREST  pulmonary htn, GI issues

Diffuse  ILD, cv disease, renal disease,joint deformities, myopathy


Scleroderma diagnosis and treatment

Scleroderma Diagnosis and Treatment

Diagnosis = clinical sclerosis +/- organ involvement + auto antibodies

(older method outdated)

Treatment really symptomatic

Calcium channel blockers

Proton pump inhibitors

Ace inhibitors

Manage pulmonary, CV issues


Then 3 months later

Then 3 months later…

Patient’s skin thickening has progressed to mid upper arm

BP 140/90

Hct 30%, Plt 85,000

UA 1+ protein, 5-10 RBCs

Creatinine 1.3


Then 2 weeks later

Then 2 weeks later…

Patient now comes back with sob and malaise

Admitted to hospital

BP 140/90

Hct 20%

Plt 40,000

Creatinine 4.1

Microangiopathic changes noted on peripheral smear

Intubated for congestive heart failure


What is your diagnosis at this time

What is your diagnosis at this time?


Scleroderma renal crisis

Scleroderma Renal Crisis

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


What treatment is indicated at this time

What treatment is indicated at this time?

a. High dose steroids

b. Cyclophosphamide

c. Ace inhibitor

d. Plasmapheresis


Treatment course

Treatment & Course

Ace inhibitor was started

BP remained around 130/80

Renal function deteriorated further

WHAT WOULD YOU DO NEXT?


Further treatment

Further Treatment

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?


Rheumatological emergency

ACE inhibitor was restarted

BP stabilized

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


Features of scleroderma renal crisis

Features of Scleroderma Renal Crisis

Accelerated arterial htn

 rapidly progressive renal failure

 increased plasma renin

 MAHA, thrombocytopenia

 CHF, asymptomatic pericardial effusions

 headache, fundoscopic changes, seizures

 abnormal UA


Factors predicting incipient src

Factors Predicting Incipient SRC

Early disease

Diffuse scleroderma

Rapid progression of skin thickening

New anemia

New cardiac events (CHF/pericardial effusions)

Possibly high dose steroids


Management of src

Management of SRC

Early diagnosis and treatment

ACE Inhibitors

Normalize BP

If renal dysfunction progresses

Continue ACE Inhibitors

Dialysis prn

Steroids/plasmapheresis NOT indicated


Case 6

Case 6


History4

History

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.


Rheumatological emergency

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

FH: nc


Physical4

Physical

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


What is in your differential diagnosis

What is in your differential diagnosis?

a. Bacterial endocarditis

b. Cerebral hemorrhage

c. Primary CNS vasculitis

d. SLE

e. Antiphosphlipid antibody syndrome

f. Subdural hematoma

g. CNS infection


Which tests would be helpful in this case

Which tests would be helpful in this case?

a. CBC with diff

b. ESR/CRP

c. CXR and EKG

d. Echo

e. MRI brain

f. Blood cultures

g. CMP

h. PT/PTT


Diagnostic tests

Wbc 12,500, normal diff

Hb 13.5

Platelets 280k

CMP wnl

PT/INR wnl

PTT 45 (H)

Blood cx neg

CXR wnl

EKG wnl

Echo no vegetations, mass, pericardial effusion

MRI shows acute cva on L

Diagnostic Tests


Which additional tests would you order

Which additional tests would you order?

a. CSF analysis

b. ANA

c. Tests for coagulation abnormalities

d. C3

e. MR angiogram

f. Anti-cardiolipin antibody

g. Lupus anticoagulant

h. Beta 2 glycoprotein I


Additional tests

Additional Tests

ANA negative

C3 wnl

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 is your diagnosis now doctor1

What is your diagnosis now Doctor?

What does your patient’s disease have in common with Budapest, Hungary?

Fisherman’s Bastion 1905


Antiphospholipid antibody syndrome

Antiphospholipid Antibody Syndrome

But before we get there, what are antiphospholipid antibodies?

Group of antibodies with affinity for phospholipids

Includes

Anticardiolipin antibodies

Lupus anticoagulant

Beta2-glycoprotein I

Anti-prothrombin antibodies


Rheumatological emergency

APAS

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)


Clinical criteria for apas research

Vascular thrombosis

> 1 thrombotic epidsode AND

confirmed by imaging AND absence of vessel wall inflammation

Pregnancy morbidity

> 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

Laboratory criteria

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

Clinical Criteria for APAS (research)


How would you treat this patient

How would you treat this patient?

a. Aspirin

b. Anticoagulation

c. Steroids

d. Cytoxan

e. Plaquenil


Treatment guidelines

Treatment Guidelines

Thrombosis

 heparin  coumadin goal INR 2.5-3

Catastrophic APAS

 high dose prednisone AND

 heparin  coumadin PLUS

 plasmapheresis or IVIG


What your mind does not know your eyes will not see

What your mind does not know, your eyes will not see.


References

REFERENCES

Calvo-Romero, JM. Giant Cell Arteritis, A Review. Postgraduat Medicla Jnl. 2003; 79:511-515.

Espinal, Carlos Hugo. Michelangelo’s gout ina fresco by Raphael. The Lancet. Dec 1999; 354(9196): 2149-2151.

Harris: Kelley’s Textbook of Rheumatology, 6th edition and 7th edition. CR 2000 & 2005.

Hoffman, Gary S, et. All. A multicenter, randomized, double-blind, placebo-controlled trial of methotrexate treatment ofr GCA. Arthritis & Rheumatism. May 200; 46 (5): 1309-1318.

Keystone, EC. The utility of TNF blockade in orphan disease. Annals of the Rheumatic Disease. Nov 2004; 63 (supp II); ii79-ii83.


Rheumatological emergency

Klinkhoff, Alice. Rheumatology: diagnosis and management of inflammatory polyarthritis. Canadian Medical Assoc Jnl. Jun 2000; 162(13).

Nesher, Gideon, et al. Low dose aspirin and prevention of cranial ischemic complications in GCA. Arthritis & Rheumatism. April 2004; 50 (4): 1332-1337.

Nesher, Gideon, et al. Risk factors for cranial ischemic complications in GCA. Lippincott Medicine. Mar 2004; 83(2): 114-122.

Nuenninghoff, Dirk M, et al. Mortality of large artery complication in patients with GCA: a popluation based study over 50 years Arthritis & Rheumatism. Dec 2003; 48 (12): 3532-3537.

Proven, Anne, et al. Glucocorticoid therapy in GCA: duration and adverse outcomes. Arthritis & Rheumatism. Oct 2003; 49 (5): 703-708.


Rheumatological emergency

Tekeltaub. Gout: clnical practice. NEJM. Oct 2003; 349(17) 1647-1655.

Tekeltaub et al. Gout: on the brink of novel therapeutic options for an ancient disease. Arthritis & Rheumatism. 2004; vol 50: 2400-2414.

Uptodate.com

Wilson, et al. Preliminary Classification Criteria for APAS. Arthritis & Rheumatism. 1999; 42(7):1309-1311.


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