Dr.

Khaled khalilia 1

Rheumatoid Arthritis SLE Scleroderma Dermatomyositis

Def: Def: Def: Def:

 Autoimmune disorder  Autoimmune Disorder
 Joints involvement  Inflammation diffusely through the
 systemic manifestations of chronic body (skin, brain, kidneys, joints).
inflammation.  production of Antibodies

Et: Et:
NO DIP
 HLA-DR4 /DR1 Involvement  HLA-B8/DR3
 Drugs: Phenytoin, Hydralazine, OCs

S+S: S+S:  4 out of 11  + SLE

 Bilateral + symmetrical Joint
 Morning Stiffness ( X≥ 30 min )
 Cervical joint  C1,C2  Subluxation
 Swelling
 Fatigue + Anorexia
 Nodule
 Boutonniere Deformity
 Swan Neck Deformity
 Hammer toe, Claw toe, Mallet toe
 Extra-articular:
o Skin  ulcer
o Ocular  Scleritis, episcleritis
o Hemato  Felty’s syndrome
o Renal  Amyloidosis
Clinical:
 Malar Rash  Butterfly
 Discoid Rash
 Photosensitivity
 Ulcer (Oral/Nasal)  painless
 Arthritis
 Serositis  pleuritic, pericarditis
 Neurological  Seizure, Psychosis
Laboratory:
 Renal disorder  Proteinuria, casts
 Hematological  Anemia, Leukopenia
 Immunological  Anti-dsDNA, Anti-sm
 ANA  Most sensitive (98%)

Dx: Dx:
 Rheumatoid factor (RF)  nonspecific  + ANA (Most sensitive (98%))
 anti-CCP   Anti-ds-DNA (Anti-double-stranded
o 80% sensitive
DNA) (specific 99%)
o 95% specific.
o Correlate directly with kidney
 ↑ESR, ↑ CRP
 Radiograph: Erosion, Osteopenia injury severity
 Anti-Sm (Specific 99%)
 Anti-SSA and anti-SSB
X ≥ 6 = Rheumatoid Arthritis
 Joint involvement (5 points)
 ESR or CRP (1 point)
 X > 6 weeks (1 point)
 RF or anti-CCP (1 point)

Tx: Tx:
 DMARDS  Slow the progression  NSAIDS
 Methotrexat (1st choice)  Hydroxychloroquinine (Anti malarial)
o Side effects:  Steroids (Acute lupus flare)
 Liver Toxicity
 Pulmonary toxicity
 BM-Suppression
 TNF-α-inhibitors: (TB-reactivation)
o Infliximab
o Adalinumab
o Etanercept
 Retuximab
 Hydroxychloroquine  pregnancy
o Retinal Toxicity
 Sulfazalazine
o G6PD-deficiency
o Bone marrow Toxicity
 NSAIDS  best initial  for pain
 Steroids  for pain

Khaled khalilia
 Dr. Khaled khalilia 2

Rheumatoid Arthritis SLE

Def: Def:
 Autoimmune disorder  Autoimmune Disoreder
 Joints involvement
 inflammation diffusely through the body (skin, brain, kidneys, joints).
 systemic manifestations of chronic inflammation.
 Characterized by production of Antibodies
 Chronic synovitis  pannus formation  damages all the structures
surrounding the joint (bone, ligaments, tendons, and cartilage).
 Coronary artery disease  most common cause of death Et:
Et:  HLA-B8/DR3
 Associated with: HLA-DR4  Drugs: Phenytoin, Hydralazine, OCs, Minocycline

S+S:
S+S:
 Bilateral, symmetrical joint involvement: PIP joints of the fingers, MCP
4 out of 11  + SLE
joints of the hands, and involvement of the wrists, knees, and ankles.
Clinical:
 Morning Stiffness ( X≥ 30 min ):
1. Malar Rash  Butterfly
o Improve with use NO DIP 2. Discoid Rash
o Increase with Rest Involvemen
3. Photosensitivity
 Cervical joint  C1,C2  Subluxation t
4. Ulcer (Oral/Nasal)  painless
 Swelling 5. Arthritis  pain without deformation or erosion x-ray is normal.
 Fatigue + Anorexia Wrist  most common site 6. Serositis  pleuritic, pericarditis
 Nodule 7. Neurological  Seizure, Psychosis
 Boutonniere Deformity Laboratory:
 Swan Neck Deformity 8. Renal disorder  Proteinuria, casts (Membranous Glomerulonephritis)
 Hammer toe, Claw toe, Mallet toe 9. Hematological  Hemolytic Anemia, Leukopenia, Thrombocytopenia
 Carpal tunnel syndrome 10. Immunological  Anti-dsDNA, Anti-sm
 Extra-articular: (↑↑ RF  + Extra-articular manifestation) 11. ANA  Most sensitive (98%)
o Skin  ulcer
o OcularScleritis, episcleritis  Most common pathology  Musculoskeletal (Arthralgia, Myalgia)
o Hemato  Felty’s syndrome  Renal Disorder  deposition of circulating immune complexes
o Renal  Amyloidosis
o Lung  nodules  Pleural effusion, interstitial lung disease Dx:
o Ulnar deviation (digits)  + ANA (Most sensitive (98%))  initial test
o Radial deviation (Wrist) Felty Syndrome  Anti-ds-DNA (Anti-double-stranded DNA) (specific 99%)
DX:  RA o Correlate directly with kidney injury severity
 Rheumatoid factor (RF)  nonspecific
 Splenomegaly  Anti-Sm (Specific 99%)  only 30% of patients have a positive test
 anti-CCP  (80% sensitive, 95% specific.
 Neutropenia  Anti-SSA and anti-SSB
 ↑ESR, ↑ CRP
 Radiograph: Erosion, Osteopenia  Renal Biopsy  severity of lupus nephritis
Tx:  Antihistone  most sensitive test for drug-induced lupus
Caplan Syndrome
 RA
 DMARDS  Slow the progression Tx:
 Lung nodules
 Methotrexat (1st choice)  NSAIDS
 Pneumoconiosis
o Side effects:  Hydroxychloroquinine (Anti malarial)
 Liver Toxicity  Steroids (Acute lupus flare)
 Pulmonary toxicity  Lupus nephritis:
 BM-Suppression o steroids alone
 TNF-α-inhibitors: side effects  (TB-reactivation) o Steroids + cyclophosphamide or mycophenolate.
o Infliximab 
o Adalinumab
o Etanercept
 Retuximab
 Hydroxychloroquine good in pregnancy, Retinal Toxicity
o Retinal Toxicity
 Sulfazalazine:
o G6PD-deficiency
o Bone marrow Toxicity
 NSAIDS  best initial  for pain
 Steroids  for pain

Khaled khalilia
 Dr. Khaled khalilia 3

Scleroderma Sjögren's syndrome

Def: Def:
 Non-inflammatory Autoimmune disorder  Autoimmune disorder Lymphocytic infiltration of Lacrimal Gland and
 Small vessel vasculopathy and fibrosis. Salivary Gland:
 Scleroderma is diffuse in 20% of cases and limited in 80%. Limited  Progressive disease affect  lungs, kidney, GIT
 known as CREST syndrome:  Associated with: SLE, RA, Polymyositis, Hashimoto thyroiditis
o Calcinosis  Ca deposition in tissue S+S:
o Raynaud’s syndrome  Dry eye (Xerophthalmia)  keratoconjunctivitis sicca
o Esophageal dysmotility  Dry mouth (Xerostomia) 
o Sclerodactyly  dental caries
o Telangiectasia  Arthritis  most common Extra glandular site
Et:
 most dangerous complication  Lymphoma
 Associated with:
Dx:
 HLA-DR1  Schrimer Test  best initial
 Silica  Biopsy (Gland)  Lymphocyte infiltrate
S+S:
 Anti Ro, Anti La
 Skin  thickening, tightness
Tx:
 Raynaud’s syndrome: white  Blue  Red (↑ by cold )
 Water the mouth, artificial tears, fluoride
 Gastrointestinal  GERD, dysphagia
 Pilocarpine  + Parasymapthic  + Saliva
 Lung  fibrosis, Pulmonary Hypertension
 Renal  hypertensive Crisis
 Cardiac  pericarditis, AV-Block
Dx:
 ANA
 Anti-Scl-70 (Antitopoisomerase 1)  most specific (only in 30%)
 Anticentromere Antibodies  specific for CREST syndrome
 Eosinophilia
Tx:
 NO CURE
 Methotrexate
 GERD  PPI
Behçet Syndrome
 Raynaud’s syndrome  Ca-Blocker
 Renal Crisis  ACE-I
Def:
 Systemic vasculitis which can involve any organ
 Associated with: HLA B51
S+S:
 Mouth ulcer
 Genital ulcer
 Uveitis
 erythema nodosum-like lesions of the skin
 Arthritis
 Stomach/bowel inflammation
 Pathergy  sterile skin pustules from minor trauma to the skin
Tx:
 Steroids  first line
 Azathioprine
 Cyclophosphamide
 Colchicine

Khaled khalilia
 Dr. Khaled khalilia 4

Osteoarthritis Septic Arthritis

Def:
 degenerative joint disease (DJD)
 chronic, slowly progressive, erosive damage to joint surfaces
 The loss of articular cartilage causes increasing pain with minimal or
absent inflammation.
 Osteophytes can cause pain by stretching periosteal nerve endings
Et:
 ↑Age ↑Pain with motion
 Trauma
↓Pain with Rest
 Obesity
 Post-infection
 Endocrine: (Acromegaly, Hyperparathyroidism)
 Metabolic: (Gout, Pseudogout, Hemochromatosis, Wilson disease)
 Avascular necrosis
S+S:
 Localized  affected joint  (knee, hip, ankle)  Asymmetric
 Pain  insidious, progressive +/- Flares, Remission
 Joint tenderness, enlargement, limited motion, crepitus
 ↑Pain with motion
 ↓Pain with Rest
Dx:
 X-rays  most accurate test (x-ray severity doesn’t correlate with
symptomatology).
o Joint space narrowing
o Osteophytes
o Dense subchondral bone
o Bone cysts
Tx:
 Weight loss
 Physiotherapy
 Medicaments:
o Acetaminophen
o NSAIDS
 Surgery: Joint replacement

Khaled khalilia
 Dr. Khaled khalilia 5

Seronegative Spondyloarthropathies  NSAIDs

Ankylosing spondylitis NSAIDs Psoriatic arthritis NSAIDs

Def: Def:
 Chronic inflammatory arthritis involving the Sacroiliac joints and  immune-mediated arthritis affecting up to 30% of patients with
vertebrae. psoriasis
 ↑Men, Young S+S:
 psoriasis
Et:  papulosquamous rash
 Tendon, Ligament inflammation  Osteopenia  erosion   Nail pitting
ossification  Syndysmophytes  digits Enthesopathy  sausage digits ( dactylitis)
 HLA-B27  Opthalmo  Anterior uveitis
Worsened by Rest
S+S:  Asymmetric arthritis
Relieved by activity
 low backache (↓by activity, ↑by Rest) Dx:
 stiffness  X-ray  best initial:
 pain (radiates to the buttocks) o pencil in a cup deformity
 flattening of the normal lumbar curvature o Bony erosions
 decreased chest expansion o irregular bone destruction
 Kephosis  Uric acid ↑
 Enthesopathy  Achilles tendon.
 IBD Tx:
 Av-Block  NSAIDs  best initial therapy
 Aortic insufficiency  Methotrexate  severe disease or no response to NSAIDs.
 Anterior Uveitis ( most common extra-articular manifestation)  Anti-TNF  when methotrexate does not control disease.
 Bamboo spine  Fusion of vertebrae

Dx:
 X-Ray  best initial (Fusion, syndysmphytes, sclerosing)
 MRI  most accurate
 ↑ ESR
 + Faber Test
 Shoeber test

Tx:
 NSAIDs  best initial treatment
 exercise Reactive arthritis (Reiter syndrome) NSAIDs
 anti-TNF  etanercept, adalimumab, or infliximab.
Def:
 Sterile arthritis following an infection
 Reiter syndrome is characterized as a triad of:
o Oligoarticular Arthritis
o Conjunctivitis
o urethritis
 ↑ young men
 associated with HLA-B27.
Et:
 Secondary to:
o Inflammatory bowel disease (IBD)
o Sexually transmitted infection (↑men)
o Gastrointestinal infection (Yersinia, Salmonella,
Campylobacter)
S+S:
 Can’t see, can’t pee, can’t climb a tree”
 Urethritis/ Cervicitis / balanitis
 Anterior uveitis, Conjunctivitis
 Arthritis
 Keratoderma blennorhagicum
Dx:
 There is no specific test for reactive arthritis.
 Clinical
Tx:
 NSAIDs
 correct the underlying cause
 Sulfasalazine ( when NSAIDs do not control it).

Khaled khalilia
 Dr. Khaled khalilia 6

Vessel Vasculitis
Temporal arteritis (Giant cell, Horton) Polyarteritis nodosa

Def: Def:
 Giant cell arteritis, Horton disease  a disease of small- and medium-sized arteries leading to a diffuse
 Large vessel Vasculitis vasculitis that inexplicably spares the lungs.
 X > 50 years  associated with Chronic hepatitis B and C
S+S:
 Headache (Temporal) S+S:
 No Lung involvement
Scalp tenderness  Myalgia
 Sudden loss of vision (Painless), or Diplopia  Renal: glomerulonephritis
 Tongue + Jaw claudication  (pain in jaw when chewing)  Neurological: Any large peripheral nerve can be involved  foot drop
 Aortic arch syndrome  ↓arm pulse, aortic aneurysm  most common Look for a stroke in a young person.
 Gastrointestinal:
Dx: o Abdominal pain is worsened by eating from vasculitis of
 ↑↑ESR / C-reactive protein the mesenteric vessels.
 biopsy  temporal artery  most accurate test o Bleeding
Tx: o Nausea and vomiting
 prednisone  Starting high-dose  Skin: ulcers (Lower extremity)  livedo reticularis, purpura, nodules

 The renal lesions are ischemic secondary to necrosis of the vessels.

Dx:
 Biopsy
Tx:
 Steroids + Cyclophosphamide

Wegner granulomatosis (Granulomatosis with Polyangitis) Cryoglobulinemia

Def: Def:
 Granulomatous inflammation of vessels that may affect the upper  associated with chronic hepatitis C , endocarditis, connective tissue
airways (Rhinitis, Sinusitis), lungs (pulmonary nodules) and kidneys disorders such as Sjögren syndrome
(Glomerulonephritis, kidney failure).  IgM antibodies.
 Associated with C-ANCA  Don’t confuse cryoglobulins with cold agglutinins. Both are IgM
S+S:  antibodies.
 combination of upper and lower respiratory tract findings in Dx:
association with renal insufficiency.  positive rheumatoid factor
 Sinusitis  cold precipitable immune complexes.
 Otitis media Tx:
 Mastoiditis  Treat the underlying cause, especially hepatitis C  interferon and
 Oral and gingival involvement ribavirin.
 skin, joint, and eye lesions.

Dx:
 c-ANCA  best initial
 Biopsy  most accurate (Lung > kidney > Sinus)
Tx:
 Steroids (Prednisone) + cyclophosphamide.

Churg-Strauss Syndrome

Takayasu arteritis
Def:
 Granulomatous inflammation of the aorta and its main branches
 young women.
S+S:
 local vascular occlusion and may produce arm or leg
claudication
 arthralgia, fatigue, malaise, anorexia, and weight loss
 High pressure in the legs and low pressure in the arms
Dx: Anemia, ↑ESR, ↑CRP, Contrast angiography

Tx: Steroids  Prednisone

Khaled khalilia
 Dr. Khaled khalilia 7

Crystal Induced Arthropathies

Gout NSAIDs Pseudogout NSAIDs

Def: Def:
 Recurrent attacks of acute Monoarticular arthritis resulting from intraarticular  acute inflammatory arthritis due to phagocytosis of IgG-coated CPPD crystals by
deposition of monosodium urate crystals due to disorder of uric acid metabolism. neutrophils and subsequent release of inflammatory mediators within joint
 defect in urate metabolism with 90% of cases in men. space.
 can be from overproduction or underexcretion.  Calcium pyrophosphate deposition disease (CPPD) is from calcium-containing
 Can occur with normal serum uric acid salts depositing in the articular cartilage.
Et:  Self limited disease
 source of uric acid  diet + Endogenous Et:
 Hpoxanthin  xanthin  uric acid (Xanthin oxidase)  risk factors:
o hemochromatosis
 Associated conditions: HTN, DM, Obesity, alcohol o hyperparathyroidism.
 Overproduction: o associated with diabetes, hypothyroidism, and Wilson disease
o Increased turnover of cells: o advanced age and preexisting joint disease
 Cancer S+S:
 Hemolysis  affects knees, wrists, MCPs, hips, shoulders, elbows, ankles, big toe
 Psoriasis  DIP and PIP are not affected.
 chemotherapy  asymptomatic crystal deposition (seen on radiograph only)
o Enzyme deficiency
 Lesch-Nyhan syndrome Dx:
 glycogen storage disease
 Arthrocenthesis  joint aspuiration  most accurate test:
 Underexcretion: o rule out septic arthritis, gout
o Renal insufficiency o positively birefringent rhomboid-shaped crystals.
o Ketoacidosis or lactic acidosis o ↑WBC between 2000 and 50,000/ µl
o Thiazides and aspirin
 x-rays :
S+S:
o calcification of the cartilaginous structures of the joint
 Arthritis o chondrocalcinosis
 Pain  sudden, severe, redness, swelling of joints
 Limited joint mobility Tx:
 Most common  first MTP (podagra), knee, ankle, wrist.  NSAIDs  best initial
 intraarticular steroids  triamcinolone (if severe , not responsive to
 Chronic: NSAIDs)
 Colchicine  helps prevent subsequent attacks as prophylaxis
o Tophi (urate deposits on cartilage tendons,soft tissue)
between attacks.
o Uric acid kidney stones
o Ma be asymptomatic
Dx:
 aspiration  (most accurate test):
o needle-shaped crystals with negative birefringence
o WBC on joint fluid is elevated between 2000 and 50,000/µl
o predominantly neutrophils.
 ↑Uric acid levels
 ↑ ESR
 ↑WBC
 X-rays: normal in early disease. Erosions of cortical bone happen later.
Tx:

Acute attack:
1. NSAIDS  best initial
2. Corticosteroides:
 No response to NSAIDs
 Contraindication to NSAIDs such as renal insufficiency
3. Colchicine
Chronic Attacks:
1. Life style change:
 Diet, ↓ consumption of alcohol, beer, meat, seafood
 Weight loss
2. Colchicine  prevent second attack
3. Allopurinol  decreases production of uric acid (don’t use in acute)
4. Probenecid  ↑uric acid excretion

Allopurinol  safe in renal failure

Colchicine  suppress white cell production

Khaled khalilia
 Dr. Khaled khalilia 8

Non Articular Rheumatism

Fibromyalgia Polymyalgia Rheumatica (PMR)

Def: Def:
 chronic (>3 mo), widespread (axial, left- and right-sided, upper and  pain and stiffness of the proximal extrimities (gridle area)
lower segment), non- articular pain with characteristic tender points  X > 50 years old
 associated with irritable bowel , bladder syndrome, migraines, tension S+S:
headaches, restless legs syndrome, obesity, depression, and anxiety  morning stiffness
S+S:  pain and stiffness of the symmetrical proximal muscles (Neck,
 chronic musculoskeletal pain and tenderness with trigger points. shoulder,Hip)
 widespread aching, stiffness  Tender muscles  but no WEAKNESS
 easy fatiguability
 sleep disturbance: non-restorative sleep, difficulty falling asleep, and Dx:
frequent wakening.  ↑ ESR
 symptoms aggravated by physical activity, poor sleep, emotional stress  Anemia (Normochromic, normocytic)

Dx: Tx:
 there is no specific test  Steroids
 clinical picture
 identification of 11
specific trigger points
Tx:
 physical therapy
 tricyclic antidepressant Carpal tunnel syndrome
 amitriptyline
Def:
 Peripheral neuropathy from the compression of the median nerve as it
passes under the flexor retinaculum.
 Pressure on the nerve interferes with both sensory and motor function
of the nerve.
Et:
Polymyositis / Dermatomyositis  unclear etiology, but it is associated with
o overuse of the hand and wrist
Def: o Pregnancy
 Autoimmune disease characterized by proximal muscle weakness +/- o Diabetes
pain. o Rheumatoid arthritis
o Acromegaly
 Associated with malignancy (Lymphoma, ovary, Lung)
o Amyloidosis
 CD-8 cell mediated muscle necrosis
o Hypothyroidism
S+S:
S+S:
 progressive symmetrical proximal muscle weakness (shoulder and hip)
 pain :
developing over weeks to months.
 difficulty lifting head off pillow, arising from chair, climbing stairs o palm, thumb, index finger, and the radial half of the ring finger
 Dysphagia o worse at night
 Dermatomyositis presents with: o involves prolonged use of the hands such as typing.
o Malar involvement  muscle atrophy of the thenar eminence
o Shawl sign: erythema of the face, neck, shoulders, upper  Tinel sign: reproduction of the pain and tingling with tapping or
chest, and back percussion of the median nerve
o Heliotrope rash: edema and purplish discoloration of the
 Phalen sign: reproduction of symptoms with flexion of the wrists to 90
eyelids
o Gottron papules: scaly patches over the back of the hands, degrees
particularly the PIP and MCP joints  Sensory symptoms happen before motor symptoms.
Dx: Dx:
 ↑CK and aldolase  best initial test  Clinical Symptoms
 muscle biopsy  most accurate test  Tinel sign and Phalen signs
 Anti-Jo antibodies  simple compression of the nerve by squeezing it helps confirm the
Tx: diagnosis.
 Steroids
 electromyography and nerve conduction testing  most accurate Test
 unresponsive to steroids, use:
o Methotrexate  Do not do wrist MRI !!!!  
o Azathioprine Tx:
o Intravenous  wrist splints  immobilize the Hand  best initial Therapy
immunoglobulin  avoid manual activity
o Mycophenolate  Steroid injection  if splints and NSAIDs do not control symptoms
 Hydroxychloroquine  skin  Surgery  mechanically decompressing
lesions. (DMM)

Khaled khalilia
 Dr. Khaled khalilia 9

Adult onset still’s disease

Def:
 systemic inflammatory condition (ANA and RF negative) with fevers
and characteristic rash, numerous systemic symptoms, and may have
severe arthritis.
S+S:
 classic triad of symptoms
1. high-spiking fevers
2. characteristic “salmon rash  proximal limbs + trunk
3. arthralgia/arthritis  symmetric, large joints, wrists, knees
ankles
 sore throat, myalgias and serositis
 hepatomegaly
 splenomegaly

Dx:
 ANA and RF negative
 ↑ESR, CRP, ferritin
 anemia, thrombocytosis, leukocytosis
 response to NSAIDs (see Lange q.3 Rheumatology)

Tx:
 glucocorticoids ± MTX

Marfan syndrome

Def:
 e
 autosomal dominant

S+S:
 Dislocation of the lens
 chest deformities
 long limbs
 aortic dilatation

Khaled khalilia
 Dr. Khaled khalilia 10

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 11

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 12

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 13

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 14

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 15

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 16

Arterial Blood Gas (ABG)

Khaled khalilia
 Dr. Khaled khalilia 17

Arterial Blood Gas (ABG)

Khaled khalilia