Rheumatology Zebras Flashcards
Treatment of IgG4-RD dx
immunosuppression (azathioprine, mycophenolate mofetil, rituximab) for refractory dx
simple cases with steroids
Diagnosis of IgG4 dx
tissue infiltration with mainly IgG4 positive plasma cells and small lymphocytes
elevated serum IgG4 levels
peripheral eosinophilia
Clinical features of IgG4 RD dx
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
Who gets IgG4 dx?
middle aged men
What is IgG4 related dx
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
What abnormality is seen on blood work for IgG4 dx
eosinophilia (40%)
IgG4 positive plasma cells and lymphocytes on tissue biopsy
What should be considered on differential for IgG4 dx?
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.
hypertrophic osteoarthropathy
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.
Behcet’s dx diagnosis
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
manifestations of Behcet’s dx
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.
Treatment of Behcet’s dx
colchicine, steroids and immunosuppressives
Familial mediterrean fever presentation
paroxsymal episodes of fever, concomitant serositis with peritonitis, pleuritis, and percaridits
Herpes simplex infection
see oral ulcers, keratitis, sometimes uveitis
Adult onset Still’s disease
presents with arthritis and rash
rash is evanescent (disappears) and only appears with fever
rash is salmon colored
pts have high fever and leukocytosis
what is seen on labs with acute onset still’s dx
high ferritin.
history of smoking, clubbing in fingers, and symptoms of periosteal inflammation
hypertrophic osteoarthropathy or HOA
if someone has hypertrophic osteoarthropathy or inflammation of the periosteal structures and see proliferation of skin and osseus tissue.
What do you order nexst?
CXR or lung imaging
look for non small lung cancer.
behcet’s dx is a
vasculitis of small (most common), medium or large vessels that involves multiple organs
diagnosis of behcet’s dx
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.
pulmonary hypertrophic osteoarthropathy (PHO)
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.
work up for pulmonary hypertrophic osteoarthropathy
need a CXR at minimum and consider lung cancer
presentation and symptoms of pulmonary hypertrophic osteoarthropathy (PHO)
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.
what can cause secondary PHO or pulmonary hypertrophic osteoarthropathy
secondary form- presents as adult with underlying etiology of LUNG cancer, adenocarcinoma. Can also be caused by pulmonary infections, CF, intracardiac shunt or lymphoma.
what is seen on XR imaging of extremities in pulmonary hypertrophic osteoarthropathy
periostosis on imaging
see clugging
arthropathy
skin proliferation of distal extremities
seen with lung cancer
treatment of Behcet’s dx of acute oral and genital ulcers
topical glucocorticoids
Behcet’s ulcers resolve in
1-3 weeks spontaneously resolve.
what helps to prevent recurrent mucocutaneous lesions of Behcet’s ulcers?
colchicine
only consider azathioprine, interferon alfa and TNF alpha inhibitors should be considered in recurretn and chronic arthritis.
what are the main causes of morbidity and mortality in Behcet’s pts?
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.
how to treat Behcet’s disease?
steroids to suppress activity and
longer term. TNF inhibitors depending on severity of disease
familial mediterranean fever is
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
Treatment of familial mediterranean fever is
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.
DISH or diffuse idiopathic skeletal hyperostosis is
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.
DISH demographics
not seen in young men (<40 yrs) –> think ankylosing spondylitis (also must have sacroiliac joint involvement).
DISH is old man’s dx.
What seen on XR with DISH?
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.
DISH syndesmophytes are
ligamentamenous ossification, see “bridging ossification on the right side” of vertebral bodies
