Polymyositis, Dermatomyositis, Polymyalgia rheumatica, Giant (temporal) cell arteritis, Polyarteritis nodosa, Hypersensitivity vasculitis, Granulomatosis with Polyangiitis, Osteoporosis Flashcards
(25 cards)
Polymyositis: overview
- Immune-mediated disease leading to muscle inflammation.
- Due to CD8+ lymphocyte infiltration of the endomysium. This causes muscle cell destruction.
- Immune cells confuse normal muscle proteins with foreign antigens. - Molecular Mimicry. (A host protein is mimicking a foreign or tumor protein.)
- Inflammation in & around muscle cells.
- Repeated attacks over time.
- Can involve different muscle groups (proximal limbs, neck, pharynx, heart lungs, GI tract.
- Women 30-50 y.o.
- Can happens alongside RA, Sjogren’s syndrome, or scleroderma.
*
Polymyositis
Symptoms
- Progressive, symmetric bilateral weakness & atrophy**
- Affects large & proximal muscles (Shoulders, hips). Spares small distal muscle (hands)**
- Difficulty rising from chair, combing hair, lifting arms, carrying bags, stair climbing
- Tender muscles
- Decreased muscle strength.
- Severe cases = dysphagia and/or respiratory weakness.
Polymyositis
Diagnosis and tx
dx:
* muscle specific antibodies: Anti J0-1, Anti-signal recognition protein**
Best initial test: Elevated muscle enzymes (creatine kinase, aldolase)*
* Muscle Bx. Definitive diagnosis. Shows ENDOMYSIAL inflammation
* Electromyography
Treatment
* Corticosteroids - Suppress the immune response.
* Physical Therapy
* Steroid sparing agents - Azathioprine or Methotrexate.
Dermatomyositis: overview
- Immune-mediated disease leading to skin & muscle inflammation.
- Due to CD4+ lymphocyte infiltration of the perimysium (perivascular involvement). This causes muscle cell destruction.
- Immune cells confuse normal muscle & skin proteins with foreign antigens. - Molecular Mimicry. (A host protein is mimicking a foreign or tumor protein.)
- Inflammation & cellular destruction → Loss of blood vessels → Tissue ischemia in affected muscle & skin.
Dermatomyositis sx and skin manifestations
- Related to muscle (Similar to polymyositis).
- Progressive, symmetric bilateral weakness & atrophy***
- Affects large & proximal muscles (Shoulders, hips). Spares small distal muscle (hands)***
- Difficulty rising from chair, combing hair, lifting arms, carrying bags, stair climbing
- Tender muscles
- Decreased muscle strength
- Severe cases = dysphagia and/or respiratory weakness.
Skin sx:
* Heliotrope rash - edema & blue or purple discoloration of the upper eyelids**
* Gottron papules - raised & violaceous scaly patches. (e.g. dorsum of the PIP & MCP joints)
* Malar rash that involves the nasolabial folds.
* Rashes are photosensitive, itchy and may bleed.
Dermatomyositis: dx and tx
Diagnosis
* Combination of skin & muscle findings:
* Elevated muscle enzymes - best initial test (creatine kinase, aldolase)**
* Autoantibodies: Anti Jo-1, Anti-Mi-2.**
* Abnormal Electromyogram
* Muscle Biopsy - definitive diagnosis. Inflammation of the perimysium**
Treatment
* Suppress the immune response: Corticosteroids
* Antimalarial Meds: Hydroxychloroquine & Chloroquine - useful for skin lesions.
* Sun avoidance and protection.
Polymyalgia rheumatica
Poly = Many
Mya = Muscles
Algia = Pain
Rheumatica = Joints & Connective tissues
overview and sx
- Immune mediated disease
- Muscle is usually spared and the tissue around the joints, bursae, and tendons are inflamed (shoulder & hip girdle)
- Associated with Giant cell arteritis (alone or in part). Due to inflammatory cytokine travelling to the walls of the arteries in the temples.
- Exact cause unknown
- Associated with genetic (HLA-DR4) and environmental factors.
- Women >50 y.o
Symptoms:
* Pain & stiffness in the proximal joints. Decreased ROM
* May begin unilaterally and progress to bilateral.
* Morning predominance of pain and improves with physical activity.
* Constitutional symptoms - malaise, fatigue, anorexia, low-grade fever.
Polymyalgia rheumatica
dx and tx
Diagnosis
* Confirmed by increased inflammatory markers: ESR, CRP
* Normal muscle enzymes (creatine kinase, aldolase) -> Distinguishes PMR from polymyositis.
Treatment
* Suppress the immune response: Corticosteroids; Methotrexate if corticosteroids CI
* Healthy diet & exercise. (P.T.)
Giant (temporal) cell arteritis: overview and sx
- Immune mediated inflammatory vasculitis of large & medium arteries.
- Temporal m.c. Main aortic, carotid, subclavian, brachial, axillary all can be affected.
- Most common systemic vasculitis in adults.
- Blindness is a significant complication if left untreated. Occurs in 20% of patients.
- Same clinical spectrum as PMR
- Female > 50 y.o.
- New onset of headache, usually unilateral & temporal region. Worsens over time.
- Scalp tenderness
- Constitutional symptoms (fever, malaise, weight loss)
- Severe cases:
- Ischemic changes; Ophthalmologic Emergency
- Diplopia
- Eye pain
- Vision loss
- Jaw Claudication - pain with chewing
Clinical presentation in GCA: PE, fundoscopy, labs
Physical exam
* If suspect PMR evaluate for GCA
* Abnormal temporal artery exam
* Tenderness to palpation
* Ropiness
* Nodularity
* Decreased pulse
Fundoscopic exam
* Optic disc pallor
* Edema
* Optic disc atrophy (Advanced)
Labs
* Increased inflammatory markers (↑ESR ↑CRP)
* CBC (thrombocytosis, anemia)
Giant (temporal) cell arteritis
Diagnosis/Treatment; what to do with symptomatic relapse
- Do not delay treatment!!
- Temporal biopsy -standard. Do not wait for scheduling or results. Also try to obtain as quick as possible to prevent false-negative results.
- Begin IV glucocorticoids. Oral may be used if no vision changes.**
- Low-dose Aspirin
- Ophthalmology consult***
- Temporal artery biopsy on affected side → Positive =Transmural inflammation, multinucleated cells, and mononuclear infiltrate
- +/- Steroid sparing therapy
- Interleukin-6 inhibitor - Tocilizumab
- Disease Modifying Rheumatic Drugs - Methotrexate, Abatacept
Giant (temporal) cell arteritis
Symptomatic Relapse e.g. visual loss is common while tapering steroids.
* If Biopsy is negative may obtain ultrasound or mri.
* No curative treatment.
Polyarteritis nodosa: overview and sx
- Necrotizing inflammation of muscular arterioles and medium sized arteries.
- A systemic vasculitis confined to arteries only. Affects the vessels of the skin, peripheral nerves, GI tract, kidneys and cardiac. Spares the lungs.
- Men 40-60 y.o.
- Association with chronic Hepatitis B. 30% of patients positive for Hep B surface antigen.
Symptoms:
* Constitutional symptoms (fever, arthralgia, arthritis, myalgies, malaise, weight loss).
* Kidneys - renin-mediated HTN due to renal ischemia
* Skin -nodules, papules, ulcers
* GI - mesenteric ischemia, postprandial abdominal pain, GI bleeding.
* Neurologic - Multiple peripheral neuropathies. Peroneal nerve most common.
*
think this when pt presents with HTN, peripheral neuropathy (peroneal nerve - foot drop), and postprandial ab pain; could have nodules on the skin
HBsAg: Positive
ANCA: Negative
Polyarteritis nodosa
Diagnosis and tx and complications
dx:
* Requires either a tissue biopsy (necrotizing medium-vessel vasculitis & no granulomas or an angiogram demonstrating microaneurysms.
* Transmural inflammation, but segmental.
* Not associated with any of the autoantibodies found in other immune mediated conditions.
* Increase ESR, anemia, thrombocytosis.
* Proteinuria
* Nerve conduction studies - EMG
Treatment:
* Suppress the immune response
* Corticosteroids
* Methotrexate if corticosteroids contraindicated
* ACEI for HTN
* Antivirals for HBV
Complications:
* Advanced mononeuritis multiplex - severely disabling.
* Months to years of recuperation if at all.
* Mesenteric vasculitis can cause bowel ischemia wit bleeding & perforation.
Hypersensitivity Vasculitis: overview and sx
- Small vessel vasculitis of the skin.
- Does not involve organs,
- Drug or accompanying infection often present.
- 40% of cases no cause identified.
- AKA leukocytoclastic vasculitis, cutaneous leukocytoclastic angiitis, cutaneous small-vessel vasculitis.
- Immune complex-mediated pathophysiology
Signs & Symptoms:
* Skin Purpura (palpable or nonpalpable), papules, urticaria/angioedema, erythema multiforme, vesicles, pustules, ulcers and necrosis.
* Arthralgias, arthritis of large joint joints.
Hypersensitivity Vasculitis: dx and tx
American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. need 3 out of 5:
* Age at disease onset > 16 years
* Medication at disease onset
* Palpable purpura
* Maculopapular rash
* Biopsy including arteriole & venule, showing granulocytes in a perivascular or extravascular location
tx:
* Empiric
* Leg elevation
* NSAIDs
* Antihistamines
* Persistent cases; colchicine, hydroxychloroquine or dapsone
Granulomatosis with Polyangiitis: overview
- Pathologic hallmarks; granulomatous inflammation, vasculitis, and necrosis
- Granulomas - collection of immune cells that cluster when they can’t kill pathogens.
- Affects nose, lungs and kidney.
- Involves small - medium blood vessels of both the arterial and venous circulations.
- Unknown cause, but suggests a response to an inhaled antigen.
Granulomatosis with Polyangiitis: sx
- Signs & Symptoms: Triad of Upper resp tract, Lower resp tract, Glomerulonephritis
- Prodrome of nasal stuffiness and decreased hearing. Approx 90% ***
- Lower resp tract; cough, dyspnea, wheezing. (usually does not improve with abx)
- Glomerulonephritis; may progress rapidly- RBC casts, hematuria, increasing serum creatinine
- May also involve skin - purpura of lower extremities.
- Neurologic - multiple mononeuropathy, cranial nerve abnormalities
- Prodrome of nasal stuffiness and decreased hearing -> Sinusitis + lung nodules + hematuria + c-ANCA
ang (deep nose breathing) + ANCA (appa)
Granulomatosis with Polyangiitis
Diagnosis
and tx
dx:
* Chest x-ray - nonspecific e.g. nodule, cavitary lung nodules*
* Biopsy definitive - lung & kidney preferred. Shows large necrotizing granulomas
* Labs- Cytoplasmic anti-nuclear cytoplasmic antibody positivity is hallmark. Negative does not exclude disease.*** ANCA
Treatment
* Severe or life-threatening: Glucocorticoids plus Rituximab
* Non life-threatening: Glucocorticoids with methotrexate
Osteoporosis: primary vs secondary
Primary
* Common in postmenopausal women
* Decreased levels of sex hormones ( ↓Estrogen → ↑Osteoclastic activity → ↑Bone resorption → ↓Bone Density)
Secondary
* Due to chronic disease e.g. RA or medications e.g. glucocorticoids
* Kidney disorders
* Malnutrition or malabsorption
* Endocrine disorders
* Vitamin D deficiency
*
Osteoporosis
def and sx and rf
Def:
* Metabolic bone disease
* Decreased bone density
* Decreased bone mass
* Risk of fracture
* Bone resorption > formation of new bone
Signs & Symptoms:
* Usually asymptomatic
* Pathologic fractures -VERTEBRAE MC, hip, distal radius.
* Spine compression - Loss of vertebral height, kyphosis, back pain
Risk factors:
* Postmenopausal
* Fracture w/o major trauma
* Low BMI <20
* Osteopenia
* Family Hx of osteoporosis or parental hip fx.
* Smoking or alcohol use
* RA
* Long-term glucocorticoid use
Osteoporosis: screening and PE
Screening
Biological females >60-65
Biological males >70
5 yrs post menopause
PE:
Kyphosis
Loss of height
osteoporosis: Diagnosis - which scans?
Dual-Energy X-Ray Absorptiometry (DEXA):
* T-Score compares bone density with the bone density of a young female.
* Normal = -1.0 or >
* Osteopenia = -1.0 - -2.5
* Osteoporosis = T score -2.5 or less
Fracture Risk Assessment Test (FRAX Score):
* Estimates 10 yr risk of fx. Based on:
* Age, sex, weight, height.
* Hx. or personal or parental fx.
* Smoking & alcohol use
* Glucocorticoid use
* Hx. of RA
* Bone mineral density
Osteoporosis
Treatment: primary
Primary:
* Lifestyle modifications
* Bisphosphonates - 1st Line. Inhibit osteoclast-mediated bone resorption & turnover.
* Cautions: Cause or worsen esophageal disorders - Do not lie down for at least 30 min after taking.
* Caution with chronic kidney disease
* Rare- osteonecrosis of jaw and atypical stress fractures
* Stop treatment after 3-5 years.
* Denosumab - RANKL Inhibitor- reduces bone resorption
* Teriparatide - parathyroid related analogs. Reserved for men or postmenopausal women with severe osteoporosis (T-score -3.5 & nbo fx. Or -2.5 or less with fragility fracture).
* Estrogen related therapy - Raloxifene (increase risk of DVT)
* Repeat DEXA @ 1 year
* Stable or Improving & no new factures - Continue current treatment.
* Worsening or new fracture - Assess for secondary cause
* Consider alternate medication
Osteopenia def and tx
- Osteopenia -1.0 - -2.5
- Lifestyle Modifications
- Vitamin D (e.g Ergocalciferol 800mg/IU) + Calcium 1500 mg/IU)
- Smoking & alcohol cessation
- Weight Bearing exercises
- Physical therapy
