Rheumatology Zebras

Question 1

Treatment of IgG4-RD dx

Answer 1

immunosuppression (azathioprine, mycophenolate mofetil, rituximab) for refractory dx

simple cases with steroids

Question 2

Diagnosis of IgG4 dx

Answer 2

tissue infiltration with mainly IgG4 positive plasma cells and small lymphocytes
elevated serum IgG4 levels
peripheral eosinophilia

Question 3

Clinical features of IgG4 RD dx

Answer 3

bilateral salivary and or lacrimal gland involvement without significant symptoms of dry mouth and eyes
May see Thyroid dx with Ridel’s thyroiditis, fibrous or Hashimotos.
Retroperitoneal fibrosis
tubulointestitial nephritis
sclerosing cholangitis
Type 1 autoimmune pancreatitis
aysmptomatic generalized LAD

Question 4

Who gets IgG4 dx?

Answer 4

middle aged men

Question 5

What is IgG4 related dx

Answer 5

fibroinflammatory dx causing fibrosis and tumor like swelling of affected organs and unclear etiology

Seen with idiopathic pancreatitis, sclerosing cholangitis and bilateral salivary or lacrimal gland enlargement

Question 6

What abnormality is seen on blood work for IgG4 dx

Answer 6

eosinophilia (40%)

IgG4 positive plasma cells and lymphocytes on tissue biopsy

Question 7

What should be considered on differential for IgG4 dx?

Answer 7

Lymphoma (will have B symptoms though)
Alcoholism ( can have parotid swelling but pancreatitis will increased MCV and elevated LFTs)

Sjogren’s- with dry eyes and parotid enlargement but no IgG4 on biopsy or pancreatitis.

Question 8

hypertrophic osteoarthropathy

Answer 8

digital clubbing with severe pain due to periostitis and seen with intrathoracic malignancies (lung cancer) or infectious disease like pulmonary infection.

Radiographs of long bones show periostitis (onion skinning) instead of destructive changes.

Question 9

Behcet’s dx diagnosis

Answer 9

clinical diagnosis

recurrent painful oral ulcers (>3 times a year AND 2 of following: recurrent gential ulcers often with scarring, ocular manifestations of uvietis or retinal vasculitis, or skin manifestations (erythema nodosum, superficial thrombophlebitis, papulopustular lesions, acneiform nodules)
positive pathergy test

Question 10

manifestations of Behcet’s dx

Answer 10

superficial and deep vein thrombosis
GI ulceration
pulmonary artery aneurysm
assymetric and non deforming arthritis

pathergy - see papule >2mm develops 24 to 48 hours after insertion of a needle in the skin.

Question 11

Treatment of Behcet’s dx

Answer 11

colchicine, steroids and immunosuppressives

Question 12

Familial mediterrean fever presentation

Answer 12

paroxsymal episodes of fever, concomitant serositis with peritonitis, pleuritis, and percaridits

Question 13

Herpes simplex infection

Answer 13

see oral ulcers, keratitis, sometimes uveitis

Question 14

Adult onset Still’s disease

Answer 14

presents with arthritis and rash
rash is evanescent (disappears) and only appears with fever
rash is salmon colored
pts have high fever and leukocytosis

Question 15

what is seen on labs with acute onset still’s dx

Answer 15

high ferritin.

Question 16

history of smoking, clubbing in fingers, and symptoms of periosteal inflammation

Answer 16

hypertrophic osteoarthropathy or HOA

Question 17

if someone has hypertrophic osteoarthropathy or inflammation of the periosteal structures and see proliferation of skin and osseus tissue.
What do you order nexst?

Answer 17

CXR or lung imaging

look for non small lung cancer.

Question 18

behcet’s dx is a

Answer 18

vasculitis of small (most common), medium or large vessels that involves multiple organs

Question 19

diagnosis of behcet’s dx

Answer 19

recurrent oral aphthous ulcers >3 times/yr + two of the following: eye lesions, recurrent genital ulcers, skin lesions, positive pathergy (>2 papules forming 24-48 hrs after inserting a 20-25 gauge needle 5mm into skin. Tx involves steroids directed therapy towards the involved organ system.

Question 20

pulmonary hypertrophic osteoarthropathy (PHO)

Answer 20

uncommon disorder characterized by abnormal proliferation of skin and osseous structures in distal extremities

Primary or idiopathic form with a genetic basis that presents as childhood

secondary form- presents as adult with underlying etiology of LUNG cancer, adenocarcinoma. Can also be caused by pulmonary infections, CF, intracardiac shunt or lymphoma.

Question 21

work up for pulmonary hypertrophic osteoarthropathy

Answer 21

need a CXR at minimum and consider lung cancer

Question 22

presentation and symptoms of pulmonary hypertrophic osteoarthropathy (PHO)

Answer 22

see clubbing - proliferation and not related to hypoxia

painful arthropathy can be seen in in wrists, hands, ankles and feet and is usually preceded by clubbing.

Question 23

what can cause secondary PHO or pulmonary hypertrophic osteoarthropathy

Answer 23

secondary form- presents as adult with underlying etiology of LUNG cancer, adenocarcinoma. Can also be caused by pulmonary infections, CF, intracardiac shunt or lymphoma.

Question 24

what is seen on XR imaging of extremities in pulmonary hypertrophic osteoarthropathy

Answer 24

periostosis on imaging

see clugging
arthropathy
skin proliferation of distal extremities

seen with lung cancer

Question 25

treatment of Behcet's dx of acute oral and genital ulcers

Answer 25

topical glucocorticoids

Question 26

Behcet's ulcers resolve in

Answer 26

1-3 weeks spontaneously resolve.

Question 27

what helps to prevent recurrent mucocutaneous lesions of Behcet's ulcers?

Answer 27

colchicine only consider azathioprine, interferon alfa and TNF alpha inhibitors should be considered in recurretn and chronic arthritis.

Question 28

what are the main causes of morbidity and mortality in Behcet's pts?

Answer 28

they have vascular involvement - see hemorrhage, aneurysm, and clotting acute MI can happen as a result of coronary artery vasculitis renal involvement can see proteinuria or hematuria GI can lead to abdominal pain, diarrhea, or bleeding. can have scarring and bilateral panuveitis and can rapidly progress to blindness.

Question 29

how to treat Behcet's disease?

Answer 29

steroids to suppress activity and | longer term. TNF inhibitors depending on severity of disease

Question 30

familial mediterranean fever is

Answer 30

autosomal recessive with the MEFV gene see recurrent fever that lasts for 12-72 hrs and see inflammation of the peritonium that mimics surgical abdomen presents at age 10-20 yrs old

Question 31

Treatment of familial mediterranean fever is

Answer 31

colchicine or IL-1 inhibitors if refractory. this is done to prevent secondary amyloidosis and small bowel obstruction and infertility. loosely associated with HSP, Behcet's dx, polyarteritis nodosa and ankylosing spondylitis.

Question 32

DISH or diffuse idiopathic skeletal hyperostosis is

Answer 32

non inflammatory condition involving the ossification of spinal ligaments and enthesis. DISH can be asymptomatic or present in various ways including pain and stiffness in the spine, with thoracic spine being the most involved.

Question 33

DISH demographics

Answer 33

not seen in young men (<40 yrs) --> think ankylosing spondylitis (also must have sacroiliac joint involvement). DISH is old man's dx.

Question 34

What seen on XR with DISH?

Answer 34

see confluent ossification of at least four continguous vertebral levels usually on the right side of the spine. left side sparing is likely related to mechanical pressure of aorta and it's pulsations as a barrier toward boney hyperostosis on the left side.

Question 35

DISH syndesmophytes are

Answer 35

ligamentamenous ossification, see "bridging ossification on the right side" of vertebral bodies