Rheumatology- Systemic Disease and Vasculitides

Question 1

List the diagnostic criteria for Systemic Lupus Erythematous

Answer 1

MD SOAP BRAIN (4/11)

• Malar rash
• Discoid rash
• Serositis (pleuritis or pericarditis)
• Oral ulcers
• ANA
• Photosensitivity
• Blood (hemolytic anemia, leukopenia, thrombocytopnenia)
• Renal (proteinuria, cellular casts)
• Arthritis (>=2 small joints or large peripheral, non-erosive)
• Immune (anti-dsDNA or antiphosphilipid Ab)
• Neurologic (headache, seizure, psychosis)

Question 2

Discuss the treatment of SLE

Answer 2

Goals of Therapy
- Immunosuppresive and corticosteroid
Dermatologic
- Sunscreen
- topical steroids
- Hydroxychloroquine
Musculoskeletal
- NSAIDs
- Hydroxychloroquine
Organ Threatening
- High dose prednisone
- Azathioprine, methotrexate

Question 3

Discuss scleroderma pathophysiology, criteria and CREST

Answer 3

Pathophysiology
- non-inflammatory autoimmune disorder characterized by widespread small vessel vasculopathy, production of antibodies and fibroblast dysfunction causing fibrosis
Criteria
- Skin thickening of fingers of both hands extending proximal to MCP (puffy fingers, sclerodactyly)
- Finger tip lesions (digital tip ulcers, fingertip pitting scars)
- Telangiectasia
- Abnormal nailfold capillaries
- Pulmonary arterial hypertension
- Raynauds phenomenom
- Scleroderma related antibodies (anti-centromere, anti-topoisomerase I, anti-RNA polymerase III)
CREST
- Calcionosis
- Raynauds
- Esophageal dysmotility
- Sclerodactyly
- Telangiactesia

Question 4

Discuss the treatment for scleroderma

Answer 4

Dermatologic
- Low-dose prednisone
Vascular
- Vasodilator (CCB, PDE5)
GI
- PPI for GERD
- small bowel bacterial overgrowth (antibiotics)
Renal 
- ACEi

Question 5

Discuss the pathophysiology, presentation and management of idiopathic inflammatory myopathy

Answer 5

Pathophysiology
- autoimmune proximal muscle weakness due to non-suppurative lymphocytic inflammatory process
- associated with malignancy
Presentation
- symmetrical proximal muscle weakness
- Gottron’s papules: pink-violaceous flat-topped papules overlying dorsal surface of IP joints
- Gottron’s sign: erythematous, smooth scaly pathc over dorsal IP, MCP, elbows
- heliotrope rash: violaceous rash over eyelid with edema
- mechanics hands
Investigation
- CK, ANA
- anti-Jo-I in dermatomyositis
- muscle biopsy with segmental fiber necrosis, perivascular invasion with dermatomyositis and endomysial inflammation in polymyositis
Treatment
- high dose corticosteroid
- immunosuppresive
- malignancy screen (breast, pelvic, rectal)

Question 6

Differentiate dermatomyositis and polymyositis

Answer 6

Dermatomyositis
- B-cell and CD4 immune complex mediated and cause peri-fascicular vascular abnormalities
- have Gottren's rash
Polymyositis
- CD8 mediated muscle necrosis

Question 7

Discuss the pathophysiology, presentation and management of Sjogren’s syndrome

Answer 7

Pathophysiology
- autoimmune condition due to lymphocytic infiltration of salivary and lacrimal glands
- increased risk of non-hodgkin lymphoma
Presentation (Triad)
- dry eyes
- dry mouth leading to dysphagia
- Arthritis (small joint)
Investigation
- positive anti-SSA/Ro and/or anti-SSB/La
Management
- Artificial tear
- good dental hygiene
- systemic get hydroxychloroquine

Question 8

Discuss the pathophysiology, presentation and management of small-ANCA associated vasculitis

Answer 8

Pathophysiology
- drug exposure (allopurinol, gold, penicillin, sulfonamides)
- viral or bacterial infection
- idiopathic
Presentation
- palpable purpura with possible vesicles and ulceration
Investigation
- skin biopsy establish vascular involvement
Management
- NSAID

Question 9

Discuss the pathophysiology, presentation and management of small vessel ANCA-associated vasculitides

Answer 9

Pathophysiology
- Granulomatous inflammation of vessels of upper airways, lungs, and kidneys
- necrotizing granulomatous vasculitis of lower and upper respiratory tract
- focal segmental glomerulonephritis
Presentation
- Malaise, fever, weakness
- sinusitis
- proptosis
- hematuria
Investigation
- ANCA positive, elevated Cr, ESR, platelet, WBC
- RBC casts
CXR: pneumonitis, lung nodules, cavitary lesions
Management
- pulse methyprednisolone x3days followed by prednisone 1mg/kg and cyclophosphamide for 36mon

Question 10

Discuss the pathophysiology, presentation and management of giant cell arteritis

Answer 10

Presentation
- new onset temporal headache with scalp tenderness over temporal artery
- sudden, painless loss of vision due to narrowing of ophthalmic or posterior ciliary artery
- tongue and jaw claudication
- aortic arch syndrome
Investigation
- increased ESR/CRP and temporal artery biopsy
Management
- immediately start prednison 1mg/kg for 4week
- if vision loss due methyprednisolone 1000mgIV for 3days
- yearly CXR and abdominal screening

Question 11

Discuss the pathophysiology, presentation and management of polymyalgia rheumatica

Answer 11

Pathophysiology
- pain and stiffness of the proximal extremities
- no muscle weakness
PResentation
- constitutional signs
- pain and stiffness of symmetrical proximal muscle (neck, shoulder, hip girdles, thighs)
- gel phenomenom (stiffness after prolonged inactivity)
Investigations
- elevated ESR/CRP/CK
Management
- prednisone 15-20mg tapering slowly over 1 yr