RHEUMATOLOGY MBBS

REVIEW
DR. K. MALONEY
 RHEUMATOLOGY REVIEW
• Rheumatoid arthritis

• Systemic Lupus Erythematosus

• Systemic Sclerosis

• Mixed Connective Tissue Disease

 RHEUMATOID ARTHRITIS – CLINICAL
MANIFESTATIONS
• Symmetrical polyarthritis
• Involves small, medium and large joints
• Prolonged morning stiffness (60 mins)
• MCPJ, PIPJ, wrists, MTP almost always affected
• Shoulders, elbows, hips, knees, ankles and cervical spine commonly involved
• DIP, lumbar spine are spared

• Atypical presentations:
• Oligoarthritis  symmetrical polyarthritis
• Monoarthritis (must rule out septic arthritis)
SPECTRUM OF PRESENTATION
 DIAGNOSIS

2010 ACR/EULAR Criteria A. Joint involvement C. Inflammatory markers

1 large joint – 0 Normal ESR/CRP -0
2-10 large joints- 1 Elevated ESR/CRP - 1
At least 1 joint of clinical 1-3 small joints- 2
synovitis 4-10 small joints- 3
>10 (at least 1 small joint) - 5 D. Duration
≤ 6 weeks - 0
Synovitis not better explained ≥ 6 weeks - 1
by any other disease B. Seropositivity
Negative - 0
Low - 1
≥ 6 criteria High - 1
 EVALUATION
• Laboratory studies: • Synovial fluid analysis
• ESR, CRP
• Elevated leucocyte with neutrophil
• CBC predominance
-Anemia of chronic disease
-Thrombocytosis
• Imaging studies
• LFT’s:Hypoalbuminemia
• Renal function • Plain radiographs
-Periarticular osteopenia
• Rheumatoid Factor (present in 70%, but present in -Marginal erosions (occur at joint margins)
other diseases : SLE, Sjogren's syndrome)
-Joint space narrowing
• Anti-CCP (present in 60%, 95% specificity)
• ANA (present in 40%)
 EXTRA-ARTICULAR MANIFESTATIONS &
COMPLICATIONS
• Felty syndrome (pancytopenia, • Cardiovascular disease
splenomegaly, leg ulcers) • Secondary to chronic inflammation
• Independent of traditional CV risk factors
• Rheumatoid cutaneous vasculitis • DMARDs ↓ CV risk (MTX, TNF α inbitors)

• Rheumatoid nodules

• Osteoporosis
• Scleritis
• Decreased mobility, glucocorticoid use,
chronic inflammation
• Interstitial lung disease • Bone mineral density alone does not fully
reflect fracture risk

• Secondary amyloidosis
 MEDICAL THERAPY
• DISEASE –MODIFYING ANTIRHEUMATIC
DRUGS (DMARDS)

• Reduce or prevent joint damage

• Initial choice of DMARD depends on

• Disease duration
• Disease activity
• Prognostic factors

• Subsequent therapy dependent on

patient response

• Nonbiologic DMARDs
• Methotrexate
• Leflunomide
• Hydroxychloroquine
• Sulfasalazine
• Minocycline
• Combination therapy
• Poor prognostic factors & moderate
/high disease activity
DMARDS
• Biologic DMARDs
• TNF α inhibitors – Etanercept,
adalimumab, infliximab
• TNF α inhibitor failure
• Blocks T cell costimulation -
abatacept
• JAK/STAT pathway inhibitor –
tofacitinib
• Anti- Interleukin6 - Tociluzimab
• Anti-CD20 – rituximab
 MONITORING DMARDS
• All except hydroxychloroquine require • Biologicals
blood monitoring  Screen prior to use for and annually
• Malignancy
• Hydroxychloroquine – ophthalmology • CHF (NYHA >class 2)
exam annually for maculopathy • Acute infection
• Tb
• Hepatitis B (C)
• Methotrexate
- hepatitis, pneumonitis
- CBC, Renal function, LFTs 4- 6 months
 SYMPTOMATIC & ADJUNCTIVE
THERAPY
• Steroids

• NSAIDS

• Omega 3

• Calcium

• Vitamin D
 NON-PHARMACOLOGICAL
MANAGEMENT
• Patient education and counselling
• About the disease (relapsing/remitting; deformity and disability)
• Smoking cessation
• Modifying risk factors for osteoporosis and atherosclerosis

• Physical therapy

• Occupational therapy

• Nutritional support to target ideal body weight

• Administration of vaccines (influenza, pneumoccal)

 SYSTEMIC LUPUS ERYTHEMATOSUS
• Chronic systemic inflammatory disease characterized by production of ANA

• Multiple autoantibodies

• Broad spectrum of disease severity

• Intermittent exacerbations and remissions

 MUCOCUTANEOUS
• Photosensitivity

• Common rashes:
• Malar rash
• Discoid lupus
• Lupus profundus
• Subacute cutaneous lupus

• Alopecia

• Mucosal ulcerations
MUCOCUTANEOUS
 MUSCULOSKELETAL
• Arthralgia
• Arthritis

• Tendon inflammation/rupture: Jacouds

Arthropathy

• Inflammatory myositis
• Myalgia

• Complications of disease/therapy:
• Osteonecrosis (avascular necrosis)
• osteoporosis

• Affects 50 -75%
• Nonwhite patients more affected
• Proteinuria, hematuria, cellular casts
• Uncontrolled: ↓kidney function, hypertension,
edema, kidney failure
• Biopsy important for diagnosis, guide therapy
and prognosis
KIDNEY
• DO NOT WAIT FOR BIOPSY READING TO
COMMENCE THERAPY
• Start corticosteroids immediately if
suspicion high
• Once biopsy establishes disease add
induction agent (CYC/MMF)
• Anti-ds DNA and ↓ complement with renal
activity
• Poor prognosis:
• Older age, black race
• Elevated creatinine, low hematocrit
• Class IV, cellular crescents, interstitial fibrosis
 NEUROPSYCHIATRIC
• 15-95% • Wide range of presentations
• Aseptic meningitis
• Stroke
• Important contributor to morbidity
• Seizures
and mortality
• Transverse myelitis

• Must differentiate from

• Stroke
• Medication effects
• Active lupus inflammation
• Metabolic abnormalities
• Antiphospholipid antibodies
• CNS infection

2012 SLICC Criteria
≥ 4 criteria (1 clinical, 1
laboratory)
OR
Biopsy proven lupus
nephritis with +ANA/anti-ds
DNA
DIAGNOSIS
CLINICAL CRITERIA LABORATORY CRITERIA
Acute cutaneous lupus ANA
Chronic cutaneous lupus
Oral/nasal ulcers Anti-ds DNA
Nonscarring alopecia
Arthritis
Anti-Smith
Serositis
Renal
Anti-phospholipid
Neurologic antibodies/ fasle + VDRL
Hemolytic anemia
Leucopenia Low complement
thrombocytopenia

DCT
 DRUG INDUCED LUPUS
• Clinical presentation, history of • Typical antibodies
medication use, symptom resolution ≤ 6 • ANA
weeks of drug discontinuation • Anti-single stranded DNA
• Anti-Histone
• Typical manifestations
• Rash • Other findings
• Arthritis • Absent Anti-double-stranded DNA
• Pleuropericarditis • Absent anti-Smith
• Cytopenias • Complement levels normal
• Fever

• Procainamide, hydralazine,
methyldopa, TNF α inhibitors

NSAIDs & Steroids
• NSAIDS - For arthralgia
• Glucocorticoids
 Topical, systemic, intra-
articular
 Low dose for rash, arthritis
 High dose - severe
manifestations
Antimalarials
(hydroxychloroquine)
• Rash, arthritis
• Other benefits
 Prevents lupus flares
 May reduce organ damage
 Reduces thrombosis risk
 Reduces bone loss
 Increases long term survival
TREATMENT
Immunosuppressives/ Steroid
sparing
• Necessary when sustained
moderate/high dose steroids
required
• Azathioprine, methotrexate,
mycophenolate moefetil,
cyclophosphamide
• Biological
1. Belimumab (B cell activating
factor)
2. Rituximab (anti- CD20)
 SYSTEMIC SCLEROSIS-
PATHOPHYSIOLOGY & EPIDEMIOLOGY
• Unknown cause

• Characterized by
• Fibrosis of dermal, visceral organs and blood vessels (digital and pulmonary)
• vasculopathy (raynauds, pulmonary hypertension)

• Women most commonly affected

• Peak incidence 3rd – 4th decade of life

• Severity greatest in black females

 CLASSIFICATION
• Limited cutaneous systemic sclerosis (lcSSc) • Diffuse cutaneous systemic sclerosis(dcSSc)
• Skin involvement restricted to face and • Skin involvement proximal to distal forearms
distal extremities and knees

• Does not progress proximal to distal • Greater risk of

forearms or knees 1. Interstitial lung disease
2. Renal (scleroderma renal crisis)
• More likely to develop pulmonary
hypertension
• Systemic sclerosis sine scleroderma
• Visceral disease in the absence of skin
• CREST involvement
• calcinosis, raynauds phenomenon,
esophageal dysmotility, sclerodactyly an • Internal organ manifestations similar to
telangiectasia dcSSc
• Subset of lcSSc
 DIAGNOSIS
• Antinuclear antibodies present in up to
95%

• Anti-centromere – lcSSc, PAH

• Anti-Scl-70 – dcSSc, ILD

• Anti-U3-RNP- dcSSc, PAH, myositis

• Anti-PM-Scl – myositis
 CUTANEOUS MANIFESTATIONS
• Initial manifestation • Antihistamines
• Swelling and puffiness of hand digits
• Emollients
• Pruritus
• Avoid glucocorticoids!
• Dermal induration

• Immunosuppressives for
• Tellangiectasias moderate/severe dermal
edema/swelling
• calcinosis

• Vasospasm  raynauds
• Intimal proliferation
• Terminal arterioles in microvasculature
• luminal obliteration
• Terminal digital pits
• Digital ulcerations
• Digital gangrene
• Affects digits, lungs, myocardium, GI
tract, kidneys
VASCULAR
• Avoidance of cold exposure
• Calcium channel blockers
• Antiplatelet agents
• Topical nitrates
• Phosphodiesterase-5- inhibitor: Sildenafil
• Endothelin receptor antagonists
• Prostacyclin analogues
• Regional sympathetic blockade / digital
sympathectomy

• Sceroderma renal crisis
• dcSSc
• Intimal proliferation, luminar
thrombosis in afferent renal arterioles
• High renin levels
• Glomerular ischemia
• Acute hypertension, azotemia,
micoangiopathic hemolytic anemia
• Corticosteroid Rx is the greatest risk
factor
KIDNEY
• Ace inhibitor
• Prompt and aggressive titration
• Low threshold for commencing
• Dleays in Rx  renal failure
• Maintain Rx even if renal failure
present bc renal improvement may
improve months later
• ARBs
• alternative if ACEI not tolerated
• But not as effective
 PULMONARY
• ILD/PAH
• primary cause of morbidity and mortality

• Pleuritis

• Recurrent aspiration

• Organizing pneumonia

• Hemorrhage from endobronchial telangiectasias

• Higher risk of lung cancer

 MIXED CONNECTIVE TISSUE
DISEASE
• Overlapping clinical features of SLE, Polymyositis and Systemic sclerosis

• High titers of anti-U1-ribonucleoprotein and Ant1-RNP antibodies

• 10: 1 female predominance

• Age of onset: 2nd – 3rd decade

 CLINICAL MANIFESTATIONS
• Raynauds

• Swollen hands

• Arthritis
• Usually nonerosive
• RA like erosions may occur along with deformities

• Fatigue
 TREATMENT & PROGNOSIS
• Tailored to individual symptoms/manifestations

• NSAIDs
• Antimalarials
• Corticosteroids
• Immunosuppressive medications

• PAH is the main cause of death

END OF REVIEW