Rheumatology Lectures 1,2, 4

oligoarthritis vs polyarthritis

oligo 2-4

poly 5+

_________syndromes are accompanied by crepitus, swelling, effusion and articular heat

regional

__________ syndrome accompanies burning, numbness, electric shock and tingling

neurogenic

_____ syndrome means symptoms are felt at distance from their origin

referred

red flags of low back pain

inflammatory rhythm, localized/nocturnal pain, visceral symptoms, onset <30 or >50, pain in movement of all directions, history neoplasm, risk osteoporosis, neuro symptoms

osteoporosis risk factors

smoking, alcohol, sedentary, family history, history fx, late menarche

what kind of exam must you always perform if muscle syndrome

neuro exam

rule out _______ and _______ before considering rheuma disorders

malignancies

tb

environmental factors involved in the pathogenesis of RA

• infections (cmv, ebv, parvo)

• smoking, coffee

• high bmi

• mineral oils

• sex hormones

• pregnancy

elementary lesion of RA

synovitis

epidemio RA

women, 50y old

pattern along body of polyarthritis in RA

bilat, symmetrical

RA - joints to check

wrist, MCP II and III, PIPs

what joints are not involved in RA

DIPs

when to refer to rheumatologist if suspicion RA

3+ swollen joints

Gaenslen test/squeeze test

morning stiffness >30m

deformities of joints in RA

fusiform swelling, swan neck, boutonniere, ulnar deviation fingers, piano key ulnar head

RA foot joint - description

platfus in valgus

RA shoulder - effects

glenohumeral arthritis, retractile capsulitis → no abduction, erosion distal part of clavicle + rotator cuff tear

extra articular general manifestations RA

fever, fatigue, malaise

amyloidosis

rheumatoid nodules in RA - description

hard, mobile, painless

pulmonary extra-articular manifestations of RA

• pleural disease

• nodules

• sd caplan

• interstitial fibrosis

• bronchiolitis obliterans ± pneumonia

  • interstitial pneumonitis

renal manifestations RA

proteinuria, nephrotic sd, renal failure

interstitial or extramembranous GN

blood manifestations RA

anemia, felty sd, leukoneutropenia, thrombocytopenia

RF + ANA +

infections

felty sd

common in RA

splenomegaly, leukoneutropenia

ANA +

derma manifestations RA

livedo reticularis, purpura, ulcers, digital necrosis, leuocytoclastic vasculitis

lab tests to ask for in dg RA

• cbc, blood smear

• ESR, CRP

• liver test

• creat

  • urine analysis

• RF, anti CCP, ANA

Igs matching with RF, anti-CCP

RF - IgM

Anti-CCP - IgG

imaging to ask for in RA

thoracic, hand, wrist, foot, cervical spine

what can we see in cervical spine xray in RA

medular compression

what can we see on US in RA

erosions, teno/synovitis, tendinous tears

classification criteria fpr RA

ACR/EULAR (look at it)

prognostic factors RA

FR +, anti CCP +, ESR and CRP high

RA remission - NPJ, NSJ, CRP, ESR values

NPJ and NSJ /<1

CRP /< 1mg/dl

ESR /<1

management of symptoms RA

NSAIDs, SAIDs, cortico

rehab, reeducation, orthesis

treat2target for RA

1. classical DMARDs - methotrexate, sulfasalasine

2. bio DMARDs -

3. target DMARDs

infliximab, golimumab and adalimumab are what kinds of drugs

tnf inhibitors (t2t for RA)

what kind of drug is anakinra

antagonist il 1 receptor

what Ab is a good predictor for outcome of early RA

anti-CCP

what kind of drug is abatacept

inhibitor co-stimulation

what disease is Witebiski’s criteria for

SLE

onset SLE

15-40yo

general signs SLE

fever, asthenia, anorexia

what are the most common joints in SLE

small joints of hand, wrists, knees

________ joints have no erosion and mild synovial thickening

SLE

where are subluxations on SLE

hands

joint involvement in late SLE

hook-like erosions

complications of joints in SLE

septic arthritis

osteonecrosis

SLE myositis is secondary to

• joint inflammation

  • drugs (steroids, antimalarials)

characteristics of SLE myositis

muscle atrophy, mononuclear cell infiltrate

acute skin manifestations SLE

days to weeks - photosensitivity

pruritis, pain

non transient buttery fly

generalized erythema

bullous lupus erythematous (neck, axilla, inguinal)

subacute skin manifestations of SLE

in sun-exposed area, annular → polycylic, psoriasiform → papulosquamous

chronic SLE skin manifestations

erythematous papules or plaque w/ scaling → thick and adherent w/ hypopigmented central area

localized discoid if no systemic manifestations

generalized discoid if systemic manifestations

lupus profundus → panniculitis + skin lesion

non-specific skin manifestations of SLE

vasculitis, livedo reticularis, oral lesions, panniculitis (deep firm nodule), urticarial lesions (mucous membrane lesions), alopecia

renal involvement SLE

renal failure, NS

what paraclinicals to check renal involvement in SLE

urine analysis, serum creat, renal biopsy

6 classes of SLE

1. normal

2. mesangial nephritis

3. focal proliferative GN

4. diffuse proliferative GN

5. membraneous nephropathy

   1. advanced sclerosing GN

neuro manifestations SLE

• stroke sd

• seizures

• headaches

• transverse myelitis

• cranial or peripheral neuropathy

• movement disorder

psychiatric manifestations

psychosis, organic brain sd, psychoneurosis, neurocognitive dysfunction

characteristics of pleuritis in SLE

small, bilateral, fluid is exudate w/ normal glucose conc

increased WBC (neutrophils if acute, Ly if chronic)

what kind of patient with SLE gets pleuritis

old or drug induced lupus

clinic of pericarditis in SLE

no pericardial rub/chest pain

fluid is high leukocytes, complement, ANA, + LE cells

when do we have big pericardial effusions in SLE pericarditis

if associated with uremia

types of pulmonary involvement in SLE

pleuritis, pneumonitis, pul hemorrhage/embolism/HTA, shrinking lung sd

cardiac involvement SLE

myo/endocarditis

coronary heart disease

manifestations of GI involvement inSLE

pseudo-obstruction - peritonitis, bowel vasculitis, pancreatitis, IBD

chronic ascites

___________ in SLE are soft, hard and variable in size

lymphadenopathy nodes

spleen involvement in SLE

“onion skin lesions”

periarterial fibrosis

splenic atrophy or lymphoma

vision problems in SLE

cataract, vitreous hemorrhage, vasculitis retina, choroid and neuro issues

Evans sd

in SLE

hemolytic anemia + thrombocytoepenia

_______ present with proximal and symmetrical m weakness

myositis

_______ is a group of autoimmune rare and heterogenous m disorders

idiopathic inflammatory myopathies (IIM)

markers IIMs

HLA DRB1 DQA1

B27 (protective factor)

environmental factors to IIM

infections, exposure UV and vit D, drugs

clinical features dermato/polymyositis

symmetric and proximal m weakness, impaired m endurance, m fatigue

aspiration pneumonia

cutaneous manifestations dermato/polymyositis precedes

m involvement

amyopathic dermatopolymyositis - cutaneous manifestation

gottron’s papule

hypomyopathic dermatopolymyositis - cutaneous manifestation

heliotrope rash

post myopathic dermatopolymyositis - cutaneous manifestation

shawl/V sign

dermatopolymyositis sine dermatitis - cutaneous manifestation

holster sign

dermato/polymyositis - visible signs

mechanic’s hand, periungual nailfold telangiectasis, cuticular hemorrhage, panniculitis, livedo reticularis, alopecia, vesicobullous lesions, cutaneous calcinosis

lung involvement of dermatopolymyositis

organizing pneumonia, usual interstitial pneumonia, diffuse alveolar damage

restrictive pattern, pul HTA

heart involvement dermatopolymyositis

conduction problems, arrhythmias, HR variability

GI problems in dermato/polymyositis

swallowing problems, esophageal dysfxn, GERD, GIT symptoms

cancers common in dermato/polymyositis

lung, ovary, breast, colon, NH lymphoma

causes of elevated m enzymes

genetic (m dystrophies, beckers), congenital myopathies, neuropathies, metabolic myopathies, acromegaly, cushing sd, infections, toxins

Dg tests for dermato/polymyosutus

m enzymes, biopsy, EMG (2w after biopsy), mri

myosutis specific antibidies

anti JO1 (good prognosis)

SRP (bad)

Mi2 ab

Anti La

Anti Ro52

Anti RNP

ttt IIM

steroids, immunosuppressives (Mtx, aza, Cyc)

Anti tnf

__________ is a connective tissue disease characterized by fibrosis of skin and organs, alterations in microvasc and immunity abnormalities

systemic sclerosis

localized scleroderma

morphea, linear scleroderma, en coup de sabre

systemic sclerosis - types

limited cutaneous

diffuse cutaneous

sine scleroderma

crest sd

calcinosis, raynaud, esophageal dismotility, sclerodactyly, teleangiectasia

IcSSC vs dcSSc

limited cutaneous systemic sclerosis (distal to knees and elbows)

diffuse cutaneous systemic sclerosis (proximal to knees and elbows)

paraclinicals if red flags of early systemic sclerosis

1. capillaroscopy, serology

   1. esophageal manometry, chest HRCT, pul fxn test

mechanisms of systemic sclerosis

vasc damage, immune activation/inflammation, fibrosis

causative viruses of systemic sclerosis

herpes, retroviruses, cmv

vascular manifestations systemic sclerosis

raynauds, vasospastic attacks, pallor, cyanosis, rubor

what do you see in nailfold video-capillaroscopy of  a patient with raynauds from systemic sclerosis - early stage

giant capillaries, capillary hemorrhages, capillary distribution disorganization and absent new capilaries in active stage

if late stage - irregular enlargement capillaries, severe loss capillaries + avascular areas, disorganized capillary array, ramified capillaries

what sd is possible if we have fingertip ulcers in systemic sclerosis

raynaud

gi involvement systemic sclerosis

esophagus reflux, dysphagia (dysmotility, uclers)

bloating, N-V, stomach bleeding (gastroparesis, telangiectasia)

diarrhea/constipation, weight loss (dysmotility)

fecal incontinence