KAPNotes: Rheum

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54 terms

Define scleroderma:

Chronic multi-system dz characterized clinically by thickening of the skin dz and accumulation of connective tissue and involvement visceral organs and replace of smooth muscle

All patients with sclecroderma have

Raynaud's and skin thickening

The pathophysiology of Raynaud's?

diminished blood flow and vascular damage

GI features associate w scleroderma

esophageal dysmotility, achalasia, bacterial overgrowth, dilation of large intestines, hypomotility of small intestine, malabsorption

Pulmonary features assoc. w scleroderma

pulmonary fibrosis, cor pulmonale, restrictive lung dz

Leading cz of death in scleroderma

pulmonary fibrosis, cor pulmonale, restrictive lung dz

Renal features assoc w scleroderma

renal crisis w malignant HTN

Previously the m/c cz of death in scleroderma

ARF

Why is acute renal failure no longer the cz of death in scleroderma

can be tx'd w ACEI

Heart assoc. features assoc w scleroderma

fibrosis

Limited form of scleroderma

CREST syndrome

CREST syndrome is a dz w skin limited to?

distal extremities and face

What does CREST mean?

C-Calcinosis, R-Raynaud's, E-Esophageal dysmotility, S-Sclerodactyly, T-Telangiectasia

Ab assoc w scleroderma?

scl-70

New name for CREST syndrome?

limited scl or limited cutaneous systemic sclerosis

Calcinosis is a condition in which

calcium deposits occur in tissues (usually fingers) PIPs, knees, elbows

Most involved

knees and elbows

Limited scleroderma usually does not have dz

above the elbow or knee

compared to scleroderma limited dz progress vs diffuse

slowly

Raynaud's phenomenon

episodes of pallor or cyanosis in response to cold or emotional stimuli

Define: hyperemia

rebound blood flow

Primary Raynaud phenomenon is called?

Raynaud's dz

Underlying dz in Raynaud's is called

primary raynaud's

Raynaud's w/o underlying dz is referred to as

secondary raynaud phenomenon

How do you distinguish between primary and secondary Raynaud's phenomenon?

nailfold capillaryoscopy (place oil drop on fingernail base look under)

How do you treat Raynaud's phenomenon severe

Penicillinamine, CCBs, ACEIs

How do you tx HTN ?

HTN

Define: Sjogren

chronic autoimmune disorder charcterize by lymphocytic infiltration of exocrine glands w dry eyes and mouth

Is Sjogren a primary or secondary dz?

either.

Sjogren's may be secondary to?

RA, primary biliary cirrhosis, SLE

Progressive Sjogren's involves?

major organs and progresses into lymphoproliferative dz (malignant lymphoma)

Define keratoconjunctivitis?

dry eyes and mouth

Other sxs assoc w Sjogren's aside from sicca syndrome?

parotid enlargement, dental caries

Dx test for Sjogren's?

Schirmer's test (tear production), Bengal stain (corneal stains), ANA +, anti-la (SSB), anti-Ro (SSA)

Salivary gland bx will show?

lymphocytic infiltration

Tx for dz?

No definitive tx

Define: spondyloarthopathy

group of disorders that share clinical features w HLA-B27

List the the seronegative spondyloarthropathies

ankylosing spondylitis, reactive arthritis, psoriatic arthritis, enteropathic arthropathy

Define: acute spondylitis

disorder primarily affects axial skeleton and peripheral joints

Gender predominance assoc w

male greater than female (3-4 Xs)

Cz?

unknown

90% of pts have

HLA-B27

Ankylosing spondylitis presents w?

chronic lower back pain, young female, morning stiffness x 1 in, improved w exercise

Rarely

c-spine (late)

Common extraartiular dz w ankylosing spondylitis

anterior uveitis, tertiary heart block, aortic insufficiency progresses to CHF

Clinical exam of ankylosing spondylitis reveals?

limited spine mobility, obliterans of the lumbar lordosis, minimal trauma

XR in AS?

sacroilitis

What do spondyloarthropathies have in common?

seronegative, involve lower back, extracurricular, HLA-B27

Rarely involved?

c-spine

Earliest XR evidence of AS

sacroilitis

Late XR evidence of AS

fused sacroiliac

Tx for AS

NSAID, physiotherapy, exercise

Tx for Reactive arthritis

NSAIDS, physical therapy

A severe form of reactive arthritis

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