Rheum E2 -Systemic Sclerosis, Morphea, Raynaud's, Sjogren's

What is Systemic sclerosis (Scleroderma)?

chronic autoimmune proliferation of collagen with small vessel vasculopathy and fibrosis that primarily targets the skin, GI, lungs, and kidneys

Who does Systemic sclerosis predominantly affect?

women of childbearing age

Diffuse or Limited cutaneous scleroderma:

Thick skin distal AND proximal to elbows and knees, with or without the face

Diffuse cutaneous scleroderma

Diffuse or Limited cutaneous scleroderma:

Thick skin distal, but NOT proximal to elbows and knees, with or without the face

Limited cutaneous scleroderma

What cutaneous changes are seen in diffuse cutaneous Systemic Sclerosis?

early -diffuse swelling & stiffness of fingers, rapid progression, involves skin above & below wrist/forearm

"C" in CREST (lcSS)

Calcinosis- calcium deposits in the skin

"R" in CREST (lcSS)

Raynaud's phenomenon- spasm of blood vessels in response to cold or stress

"E" in CREST (lcSS)

Esophageal dysfunction- acid reflux and decrease in motility of the esophagus

"S" in CREST (lcSS)

Sclerodactyly- thickening and tightening of the skin on the fingers & hands (distal)

"T" in CREST (lcSS)

Telangiectasias- dilation of capillaries causing red marks on the skin

Which antibody is often present in CREST (limited cutaneous scleroderma)?

Anti-centromere antibody

What is ALWAYS present in CREST (limited cutaneous scleroderma)?

Raynaud's

What antibody is seen in Progressive systemic sclerosis (PSS) or dcSS?

Anti-SCL 70 antibody

What develops within the first 4 years of Progressive systemic sclerosis (PSS)/dcSS?

Interstitial lung disease — especially with the presence of anti SCL-70 antibody

Diffuse or Limited cutaneous scleroderma:

Telangiectasis & Calcinosis more common

Limited cutaneous scleroderma

What are features of both lcSS & dcSS?

sclerodactyly, ulcers, tight oral orifice

What can you give to relieve MSK sx of dcSS?

(polyarthralgia, friction rub, contractures, myopathy)

NSAIDs, Plaquenil, steroids

What is a major cause of death in Diffuse cutaneous scleroderma if not treated early?

Renal crisis

What can you give to prevent/treat malignant arterial HTN in dcSS?

ACE-I

Is renal disease common in lcSS?

No- rare

What cardiac manifestations can dcSS cause?

CHF & arrythmias -get ECHO annually

Diffuse or Limited cutaneous scleroderma:

Pulmonary HTN leading to cor pulmonale

Limited cutaneous scleroderma

What is the 1st line tx for ILD caused by dcSS?

MMF (cellcept)

can add Nintedinib (TKI) also

What is the 1st line tx for PAH caused by lcSS?

BOTH: CCBs & Bosentan (tracleer)

What testing needs to be done in pts w/ dcSS to check for ILD?

PFTs & high-resolution CT of the lungs

Diffuse or Limited cutaneous scleroderma:

Watermelon stomach (GAVE)

Diffuse cutaneous scleroderma

What is GAVE?

Gastric antral vascular ectasia (watermelon stomach)

What antibodies are associated w/ greater risk for GAVE (watermelon stomach)?

Anti-RNA III polymerase antibodies

What lab do 90-95% of SS pts have?

+ANA

Diffuse or Limited cutaneous scleroderma:

Anticentromere antibody

Limited cutaneous scleroderma

Diffuse or Limited cutaneous scleroderma:

Anti SCL-70 antibody

Diffuse cutaneous scleroderma (lung dx)

Diffuse or Limited cutaneous scleroderma:

Anti-RNA polymerase III antibody

Diffuse cutaneous scleroderma (worse skin disease, GAVE, renal crisis)

Who is Anti-U3-ribonucleoprotenin antibody (fibrillarin, AFA) seen in?

more in AA, younger age, males, associated w/ myositis

What tends to worsen SS, and often precipitates HTN or renal crisis?

steroids

If you have to use steroids for SS, what is important to do?

low doses of Prednisone for as short of a duration as possible

What is Morphea?

Localized scleroderma

What demographic is most affected by Morphea?

whites, F>>M, peds, adolescents, & YA

What parts of the body does morphea affect?

Trunk & lower extremities

What is the most common pattern of morphea?

linear

What is En coup de Sabre (scar from sword)? (occasionally seen in Morphea)

thickened skin extending from the hairline onto the forehead

What is Parry Romberg syndrome?

Progressive facial hemiatrophy that looks like linear scleroderma en coup de sabre but involves the brain

What is the 1st line tx for localized/linear Morphea?

Phototherapy

What is the 1st line tx for deep/generalized Morphea?

IV steroids + MTX, then Cellcept

What is primary Raynaud's phenomenon?

idiopathic — present beyond 4 yrs w/o a secondary disease

What is the mneumonic for secondary Raynaud's?

BAD CT

"BAD" in BAD CT

Blood disorder (Polycythemia)

Arterial (blockage)

Drugs (BBs)

"CT" in BAD CT

Connective tissue disorders (SLE, DM, PSS)

Traumatic (vibration injury)

What is the cycle of color changes in Raynaud’s?

white, blue, red, normal

What diagnostic test is used for differentiating primary vs. secondary Raynaud's phenomenon?

Nailfold capillaroscopy

What is the 1st line tx for Raynaud's phenomenon?

CCBs

What are other 1st line tx for Raynaud's phenomenon if CCBs are contraindicated?

PDE4 inhibitors or topical nitroglycerin ointment

What is Sjogren's syndrome?

Autoimmune lacrimal & salivary gland dysfunction

What is the classic triad of Sjogren's syndrome?

Xerostomia, Keratoconjunctivitis sicca, Arthritis

What is another sx of Sjogren’s in addition to the classic triad?

Bilateral parotitis

What other AI condition is Sjogren’s MC seen with?

RA

What does histopathology reveal in lacrimal & salivary glands of Sjogren’s?

Lymphocytic cells

What causes Sjogren’s?

inc CD helper T cells, B cell autoantibodies, +SSA (Ro) & SSB (La)

What other testing can help dx Sjogren’s?

(+) Schirmer’s & (+) Lissamine green or Rose bengal staining

What is the most definitive diagnostic study for Sjogren's syndrome?

Abnormal salivary gland or lip biopsy showing lymphocytic infiltration +ANA: SSA (Ro) and/or SSB (LA)

Which antibodies are more pathogenic for Sjogren's syndrome?

Anti-SSA (Ro)

What is considered a + Schirmer's test?

< 5 mm tear production in 5 mins (Sjogren's)

What is the preferred eye staining test for Sjogren's?

Lissamine green staining (less pain)

What is the most severe extra-glandular symptom of Sjogren's syndrome?

Renal tubular acidosis

What is the 1st line tx for Sjogren's?

Supportive (hydration), Sialogogues (pilocarpine, cevimeline, salagen), Tear duct ablation/plugs

What can be used to treat extra-glandular sx of Sjogren’s?
*in order

steroids (as bridging), then Plaquenil, then MTX

True or False: Biologics can be used for Sjogren's syndrome

False

What is the greatest complication of Sjogren's syndrome?

Non-Hodgkin's lymphoma

*check lymph nodes every 6-12 mos