CAM III - Rheumatology pt.2

Raynaud's phenomenon color changes

Pallor - vasoconstriction

Cyanosis - deoxygenation

Erythema - reperfusion

Gold standard finding in primary Raynaud's disease

Normal nailfold capillaroscopy findings

Secondary Raynaud's phenomenon triggers

SLE, carpal tunnel, scleroderma

Smoking

Injury

BB, ADHD meds, chemo

SLE symptoms

Fatigue, malar rash, arthritis, alopecia, weight changes

Complications of severe SLE

Nephritis -> proteinuria

Pleurisy

Pericarditis

Brain inflammation (rare)

SLE labs

High titer ANA

Elevated ESR/CRP

CBC, CMP, UA, elevated creatinine

Urinalysis findings in SLE

RBC/WBC casts -> active nephritis

Proteinuria

*renal bx if heavy proteinuria + hematuria

SLE tx for flares

Prednisone PO (acute flares)

Solumedrol IV for severe flares

ASA

Long term SLE tx

Hydroxychloroquine 5mg/kg/day

HCQ + MTX -> moderate disease

HCQ + MTX + immunosuppressant (mycophenalate, cyclophosphamide, rituximab) -> severe

Maintenance treatment for SLE

If chronic steroids -> < 7.5mg/d + calcium, Vit d, biphosphonate

Long term anticoagulant needed w antiphospholipid Ab or h/o thrombosis

Presence of antihistone antibodies

Drug induced lupus

*no anti-dsDNA antibodies

MCC of drug induced lupus

hydralazine, procainamide, isoniazid

Drug induced lupus treatment

Steroids

*hydroxychloroquine if persistent

Persistent scaly plaques on scalp, face, and ears -> scarring, atrophy, dyspigmentation, permanent hair loss

Discoid lupus

How do you diagnose discoid lupus

Biopsy

Discoid lupus treatment

Topical/intralesional corticosteroids

Sun protection

50 y/o woman is complaining of reduced tear and salivary production, bilateral parotid swelling, vaginal dryness, joint pain, and dry skin. She says she can no longer wear contacts d/t dry eyes. What do you suspect

Sjogren's syndrome

Labs for Sjögren's syndrome

SS-A (anti-Ro)

SS-B (anti-La)

ANA+ w speckled pattern

RF+

CBC -> neutropenia and anemia

Sjögren's syndrome diagnosis

Lip or salivary gland biopsy gold standard (rarely done)

MC clinical dx + presence of AB

Sjögren's syndrome treatment

Artificial saliva

Eye drops (cyclosporine + lifitegrast)

Cholinergic agents (pilocarpine, cevelimine)

PO steroids for acute flares

What meds should you avoid in Sjögren's patient

Anticholinergic drugs (antihistamines)

Certain antidepressants

30 y/o w joint pain, fatigue, migraines/seizures, livedo reticularis, and recurrent miscarriages

Antiphospholipid syndrome

Complications of antiphospholipid syndrome

DVT/PE

Miscarriages

MI/CVA/TIA

Libman-Sacks endocarditis

Gangrene

Labs seen in antiphospholipid syndrome

Prolonged PTT

ESR/CRP not elevated

APS antibodies:

- lupus anticoagulant

- anticardiolipin antibody (IgG)

- anti-beta2 glycoprotein 1

How do you treat antiphospholipid syndrome

Warfarin for INR 2-3

LMWH + ASA 81 if pregnant

Antiphospholipid syndrome prophylaxis

Enoxaparin 40 mg qd

Hx of thrombosis -> full dose 1.5 mg/kg/day

How do you treat the livedo reticularis manifestation of APS

Keep legs warm

No smoking

Avoid stress

Tx underlying disease

30 y/o black woman presents with a pinched nose, pursed lips, lip retraction, immobile fancies, and cannot evert her eyelids. Pt has a hx of GERD, pulmonary HTN. What do you suspect

Limited scleroderma - limited to distal extremities and face, minimal internal organ involvement

Calcinosis, Raynaud's, esophageal dysfunction, sclerodactyly, telangectasias is associated with what?

Crest syndrome - subtype of limited scleroderma

Serious complication of limited scleroderma

Digital ischemia -> ulcerations or finger loss

Morphea

type of localized scleroderma; patchy area that becomes hardened and pigmented

Linear scleroderma

Strip of hardening skin down the leg

*can stunt bone growth of affected limb in kids

Labs seen in limited scleroderma

CBC -> anemia

CMP

CRP/ESR +/- elevation

ANA+ w speckled pattern, +anti-centromere AB, SCL-70 AB

These antibodies would make you highly suspicious of limited scleroderma?

Anti-centromere AB -> highly specific for CREST

Limited scleroderma management

Echo for pulm HTN

PFTs

CXR

GI consult

Keep warm- CCB (nifedipine, amlodipine), NTG, sildenafil

Omeprazole BID, dietary changes

What meds do you want to avoid using for limited scleroderma

Avoid steroids - high doses can exacerbate sx

Pt presents with Raynaud's, weight loss, fatigue, sclerodactyly, and polyarthralgia. Pt has skin changes extended from the trunk to her proximal extremities. What do you suspect

Systemic scleroderma

Which internal organs are MC involved in systemic scleroderma

Kidneys, lungs, GI tract, heart

Leading cause of mortality in systemic scleroderma

Pulmonary disease (pulmonary HTN, interstitial fibrosis)

Systemic scleroderma labs

CBC -> Anemia (MAHA)

CMP

UA: proteinuria if renal disease

CRP/ESR +/- elevation

ANA+ w speckled pattern

Small percent of anti-centromere AB

Anti-Scl-70 AB

Anti-RNA polymerase III AB

what is anti-RNA polymerase III AB associated with?

Diffuse skin disease and renal hypertensive crisis

Systemic scleroderma management

2d ECHO, PFTs, EKG, CXR, chest CT

Tx: manage organ involvement, ACEi

Mycophenolate mofetil -> pulm disease, cyclophosphamide if severe pulm fibrosis,

Bosentan -> pulm HTN

Systemic scleroderma prognosis

9 year survival average

Breast and lung cancer common

Need annual PFTs, ECHO, CXR

50 y/o woman complains of bilateral proximal muscle weakness. She cant raise her hands overhead and has difficulty standing from a chair. She c/o dysphagia, facial weakness, and a widespread rash. What do you suspect

Polymyositis

What is polymositis associated with

Occult malignancy: ovarian, lung, pancreatic, stomach, colorectal, non-Hodgkin lymphoma

Pt with polymyositis comes in with a malar rash, gottron's papules in nail beds, heliotrope patterned periorbital edema, erythema of face/neck/shoulders/upper chest (shawl sign)

Dermatomyositis

Lab found w polymyositis and dermatomyositis

Anti-jo 1+ (myositis)

Anti-MI2 (dermatomyositis)

Elevated CK and Aldolase

CRP/ESR normal

Need to biopsy

What will EMG show Polymyositis and dermatomyositis

spontaneous fibrillations

Gold standard to dx polymyositis and dermatomyositis

Muscle biopsy

Polymyositis/Dermatomyositis treatment

Initial -> corticosteroids

Limit sun exposure

MTX, rituximab, HCQ if unresponsive to steroids

Pt presents with tea-colored urine (myoglobinuria), tachycardia, N/V, abdominal pain. What lab findings do you expect to find?

Markedly elevated CK

What acid base disorder is seen w rhabdomyolysis

Metabolic acidosis

Rhabdomyolysis causes

Crush injuries

Drugs (statins, niacin, illicit drug use/alcohol)

Excessive exercise

All statins except pravastatin and rosuvastatin are metabolized by CYP-P450, which drugs increase risk of rhabdomyolysis when taken w statins

Protease inhibitors, erythromycin, itraconazole, Clindamycin, diltiazem, verapamil, niacin, fibric acids

Rhabdomyolysis treatment

isotonic IVF if CK > 5000

IVF rate is 1-2 L/hr

Goal urine 200-300 mL/hr

32 y/o Asian woman comes in with weak peripheral pulses, fever, fatigue, weight loss, arthritis. What do you suspect

Takayasu arteritis in systemic phase

Takayasu arteritis symptoms

High BP, bruits, ophthalmic artery involvement, pulmonary artery aneurysms

Lab findings in Takayasu Arteritis

ESR/CRP elevated

US

CT w mural thickening in involved arteries

MRA

Gold standard dx for Takayasu arteritis

Conventional angiogram -> segmental stenosis, occlusion, dilation, aneurysm

Takayasu arteritis treatment

Steroids

40 y/o male w active Hepatitis B presents with fever/sweats, weight loss, myalgias, HTN, livedo reticularis, ulcers, nodules, and mononeuritis multiplex

Polyarteritis Nodosa - medium vessels, lung sparing

Labs seen with polyarteritis nodosa

CBC: anemia + leukocytosis

CMP: monitor RFTs, LFTs

UA: proteinuria

ESR/CRP elevated

Hep B

Lack disease markers: ANCA negative, low titers of RF and ANA

Dx of PAN

Tissue biopsy -> edema and necrosis across all layers of arterial wall + lymphocytes, neutrophils, eosinophils

EMG: mononeuritis multiplex

Angiogram

Polyarteritis Nodosa tx

High dose prednisone QD

Pulse methylprednisolone IV QD x 3

PAN prognosis

5-year survival rate w meds