4- Other Rheumatoid Diseases Flashcards
- Name signs
- List 4 other dermatologic manifestations of diagnosis
- Heliotrope rash
- Shawl Sign
- Gottron’s papule
- Gottron’s sign
- Poikiloderma
- Raynaud phenomenon
- Mechanic’s hands: rough cracked skin @ tips + lateral aspects of fingers.
If u have dermatomyositis (DM) patient and started on steroids but weakness worsening What could be the cause?
Medication
- Steroid induced myopathy
- Dose of steroids not sufficient
Disease
- Wrong diagnosis
- 10% of PM non responsive to steroids
- Myositis worsening
Patient
- Disuse atrophy
List 6 Lab tests for dermatomyositis (DM).
- CK
- Aldolase.
- AST/ALT.
- LDH.
- ESR/CRP.
- CBC.
- urinalysis, stool specimens, CT C/A/P or other malignancy screen.
- Antibodies. a. anti-MI2 – specific for DM. b. ANA – 24-60% of pts with DM. c. anti-Jo1 – poor prognostic factor for interstitial lung disease.
Ref: Amato chapter 30. Current dx and tx of rheumatology textbook, pg 411.
List the five criteria for diagnosis of Polymyositis
- Symmetrical muscle weakness
- Elevated CPK/aldose
- Muscle biopsy with inflammation
- EMG With myopathic pattern
- Dermatologic features (dermatomyositis)
PMR Secrets 3rd Edition Chapter 68 Rheumatology pg592
Describe an appropriate exercise program for patients with polymyositis/SLE
STRETCHING
- To maintain ROM chronic and stable, active disease.
AEROBIC EXERCISE
- Low-level aerobic program on a cycle or in a pool
STRENGTH EXERICS
- Three-times-a-week or even daily isometric program consisting of 6-10 isometric contractions. each held for 6 sec, with a 20-sec recovery time between contractions.
- The main muscles to exercise are the deltoids, biceps, hip abductors, extensors, and quadriceps muscles
- Those patients who also have some distal weakness (20-40%) may wish to exercise wrist and hand muscles and ankle dorsiflexors/plantar flexors.
- A few studies support isotonic resistive exercise for myositis with low 1-2 lb weight two to three times a week.
PRECAUTIONS
- Increased muscle weakness and soreness
- Significant rises in CPK.
PMR Secrets 3rd Edition Chapter 68 Rheumatology pg593-594
How can you stabilize the knee in the presence of a very weak quadriceps mechanism?
- Patients with polymyositis often develop very weak quadriceps muscles and begin to fall when their strength is 3 out of 5 or below.
- Ground reaction force AFO
- Do not put a dorsiflexion assist on a brace when the quadriceps is weak. A flexion moment will be created at the knee and make it less stable.
PMR Secrets 3rd Edition Chapter 68 Rheumatology pg593
What is Mixed connective tissue disorders (MCTDs)?
💡 They are RF (+) and ANA (+)
Disorders with characteristics of several other diseases, in particular
- SLE
- Scleroderma
- Polymyositis
Cuccurollo 4th Edition Chapter 3 Rheumatology pg131
What does CREST stand for? What condition is it associated with?
ACR Criteria For Scleroderma
- Thickened skin proximal to MCP joints
Or ≥2 of
- Sclerodactyly.
- Digital pitting (ischemic loss of tissue, finger pads).
- Bi-basilar pulmonary fibrosis.
≥3/5 Features of CREST syndrome:
- Calcinosis
- Raynauds
- Esophageal dysfunction
- Sclerodactyly
- Telangectasias
Ref: Current diagnosis and treatment in rheumatology pg 369.
American College of Rheumatology (ACR) Criteria - Lab tests for SLE 🔑
Eleven Diagnostic Criteria for Systemic Lupus Erythematosus
4/11 or more of following criteria
SOAP
- Serositis - Pleuritis or pericarditis
- Oral ulcers - Oral or nasal mucocutaneous ulceration
- Arthritis - Nonerosive arthritis
- Photosensitivity
BRAIN
- Blood disorder - Cytopenia
- Renal disorder - Proteinuria, cellular casts or nephritis
- ANA Ab titer - Positive ANA titer
- Immunologic - Anti-DNA antibody, or anti-Sm
- Neurologic disorder - Seizure, psychosis or encephalopathy
MD
- Malar rash
- Discoid rash - Discoid lupus rash
LAB
- Ds-DNA: Specific for SLE
- Anti-Sm: Specific for SLE
- Depressed complement levels—C3 and C4
Cuccurollo 4th Edition Chapter 3 Rheumatology pg127
Cuccurollo 4th Edition Chapter 10 Peds 759 Table 10-14
Answer
Jaccoud’s Arthritis
- Nonerosive deforming arthritis
- Ulnar deviation of the fingers and subluxations
- Reversible early, but may become fixed
Cuccurollo 4th Edition Chapter 3 Rheumatology pg128
List 4 Criteria for primary Raynaud’s disease
- Symmetric intermittent raynaud’s phenomenon attacks.
- PVD: no evidence of peripheral vascular disease.
- Gangrene: no evidence of tissue gangrene or digital pitting.
- No abnormal nailfold capillary microscopy (normal capillaroscopy).
- Negative ANA; normal ESR.
Ref: Current dx and treatment in rheumatology textbook pg 358. Memory aid: criteria are a bunch of ‘negatives’.
List 4 Causes and treatment for Raynaud’s Phenomenon 🔑
💡 Present in 90% of patients with scleroderma
CAUSES
- Skin: SLE, RA, Scleroderma, Dermatomyositis/ polymyositis
- Vessels: Vasculitis
- Blood flow: Drugs - beta-blockers, Polycythemia, TOS, Hypothyroidism
- Neurologic: SCI, CVA
- Obstruction: Trauma
- Oxygenation: Pulmonary hypertension
TREATMENT
- Avoid triggers—cold, smoking
- Keep extremity warm
- Calcium channel blockers—nifedipine
- EMG and biofeedback—self-regulation
Cuccurollo 4th Edition Chapter 3 Rheumatology pg128
Temporal Arteritis: Presentation - Association - Treatment 🔑
Temporal Arteritis
- Giant cell arteritis (GCA) → Involves large arteries
Presentation
- Tenderness of the scalp and in the muscle of mastication
- Headaches
- Abrupt visual loss in 15% of patients
- Associated with polymyalgia rheumatica
Diagnosis
- Elevated ESR
- Temporal artery biopsy
Treatment
- High-dose steroids ASAP imperative to preventing permanent vision loss
- ASA (325 mg daily—improves prognosis)
Cuccurollo 4th Edition Chapter 3 Rheumatology pg132
Explain Polymyalgia Rheumatica, Presentation, DDx, Diagnosis & Treatment
Polymyalgia Rheumatica
- Systemic inflammatory syndrome (Synovitis of the hips and shoulders)
- Associated with frequent constitutional symptoms
Theories
- Synovitis of the hips and shoulders
- Tenosynovitis (biceps)
- Bursitis (subdeltoid, subacromial, trochanteric, and interspinous muscles)
Presentation
- Patient age ≥50 years
- Systemic:
- Fever, weight loss, malaise
- MSK
- Morning stiffness for >45 minutes (neck, shoulders, pelvic girdle)
- Abrupt myalgias/arthralgia
- Hallmark—difficulty abducting shoulders above 90°
- Bilateral aching involving the shoulder girdle for ≥2 weeks
- Capsular contracture of the shoulder (limiting passive motion) and muscle atrophy
- Bilateral hip pain or a limited range of motion
- Pain at night is common and may wake the patient
- Normal strength.
Lab
- ESR >50
- Normal CK.
- Negative RF and ANA.
Differentials
- Shoulder OA, rotator cuff, frozen shoulder: Physical examination, x-rays, normal ESR
- Fibromyalgia syndrome: Tender points, normal ESR
- Rheumatoid arthritis: RF (+), small joint involvement, MTPs
- Myopathy: Polymyositis (↑ Weakness, elevated creatine kinase, abnormal EMG)
- Hypothyroidism: Elevated TSH, normal ESR
Treatment
- Prednisone at a dose of 15 to 20 mg/day (few days to be maximally effective)
Cuccurollo 4th Edition Chapter 3 Rheumatology pg123-133
Explain Polymyalgia Rheumatica, Presentation, DDx, Diagnosis & Treatment
Polymyalgia Rheumatica
- Systemic inflammatory syndrome (Synovitis of the hips and shoulders)
- Associated with frequent constitutional symptoms
Theories
- Synovitis of the hips and shoulders
- Tenosynovitis (biceps)
- Bursitis (subdeltoid, subacromial, trochanteric, and interspinous muscles)
Presentation
- Patient age ≥50 years
- Systemic:
- Fever, weight loss, malaise
- MSK
- Morning stiffness for >45 minutes (neck, shoulders, pelvic girdle)
- Abrupt myalgias/arthralgia
- Hallmark—difficulty abducting shoulders above 90°
- Bilateral aching involving the shoulder girdle for ≥2 weeks
- Capsular contracture of the shoulder (limiting passive motion) and muscle atrophy
- Bilateral hip pain or a limited range of motion
- Pain at night is common and may wake the patient
- Normal strength.
Lab
- ESR >50
- Normal CK.
- Negative RF and ANA.
Differentials
- Shoulder OA, rotator cuff, frozen shoulder: Physical examination, x-rays, normal ESR
- Fibromyalgia syndrome: Tender points, normal ESR
- Rheumatoid arthritis: RF (+), small joint involvement, MTPs
- Myopathy: Polymyositis (↑ Weakness, elevated creatine kinase, abnormal EMG)
- Hypothyroidism: Elevated TSH, normal ESR
Treatment
- Prednisone at a dose of 15 to 20 mg/day (few days to be maximally effective)
Cuccurollo 4th Edition Chapter 3 Rheumatology pg123-133
RA vs PMR, List 4 differences. 🔑🔑
💡 Clinical - Investigations - Radiology
-
Presentation
- Lack of involvement of small joints (metacarpophalangeal and proximal interphalangeal joints, MTPs) of the hands and feet
-
Serology
- Absence of RF and anti-CCP antibodies
-
Radiology
- Lack of development of joint damage
- Absence of erosive disease during follow-up
What are 3 conditions associated with polyarteritis nodosa?
List 4 features of polyarteritis nodosa
List 4 neurologic complications of PAN (polyarteritis nodosa).
💡 Polyarteritis nodosa is a rare multi-system disorder characterized by widespread inflammation, weakening, and damage to small and medium-sized arteries
Associated with
- Rheumatoid arthritis.
- SLE
- Sjögren’s syndrome
- Hepatitis B virus
- Hepatitis C virus.
- HIV infection.
Cuccurollo 4th Edition Chapter 3 Rheumatology pg133
Ref: emedicine - http://emedicine.medscape.com/article/330717-overview#aw2aab6b2b3
Features
- Glomerulonephritis—#1 cause of death
- Constitutional symptoms (fevers, weight loss, malaise).
- Intestinal angina (postprandial pain).
- Lower limb nodules/ulcerations.
- Mononeuritis multiplex
- Arthritis
Cuccurollo 4th Edition Chapter 3 Rheumatology pg133
Ref: Current dx and tx rheumatology pg 512.
Neuro (Brain - Spinal Cord - Periphral Nerve)
- Cerebral arteritis.
- Transient ischemic attacks
- Myelopathy
- Peripheral polyneuropathy
- Mononeuritis multiplex.
Ref: Emedicine - http://emedicine.medscape.com/article/330717-clinical
Fibromyalgia 🔑🔑 Dr. Dia’a & Dr. Aziz
Definition - Diagnosis - Clinical Presentation - Triggers Investigations
List 2 Important Deferential Diagnosis - Treatment
FIBROMYALGIA
- Chronic disorder with widespread pain and tenderness
- Females—20 to 60 years old
- May be associated with irritable bowel syndrome, RA.
2010 ACR Criteria
- A widespread pain index (WPI) score
- A symptom severity score (SSS) (which includes fatigue, as well as cognitive and somatic symptoms)
- Have symptoms present consistently for ≥3 months
- No other medical disorder to explain the pain
- Tender points (no longer part of criteria, but helpful)
Diagnosis
- Widespread Pain Index (WPI) & Symptom severity (SS) of 12
- WPI ≥ 7 + SS scale ≥ 5
- WPI 3-6 + SS scale ≥ 9
- Must rule out other disorders that could cause the pain symptoms
- Have symptoms present consistently for ≥3 months
Triggers
- Physical activity
- Physical inactivity
- Sleep disturbance
- Emotional stress
Presentation (MSK & non-MSK)
- Neck and upper trapezius discomfort
- Morning stiffness
- UE paresthesias
- Fatigue
- Headaches
- Lack of sleep
- Cognitive difficulties
Investigation
- Anemia → Complete blood count & Iron studies
- Hypothyroidism → Thyroid function
- Rheumatoid Arthritis → RA, ESR
- Polymgyalgia Rheumatica
- Systemic lupus erythematosus
Deferential Diagnosis for Fibromyalgia
- Myofascial Pain Syndrome
- Local pain and tender points that resolve with local treatment, but may recur
- Fatigue, morning stiffness uncommon
- Chronic Fatigue Syndrome
- Disabling fatigue for at least 6 months
- Often preceded by a viral syndrome
1. Risk Factor & Education
- Stress management
- Sleep therapy (eg, education/instruction on sleep hygiene)
- Psychologic/behavioral therapy (eg, cognitive-behavioral, operant-behavioral)
2. Protection
- Energy saving technique
3. Optimal Loading
- Low-impact aerobics: walking, water aerobics, stationary bicycle
4. Modalities
- Biofeedback, tender point injections
- Acupuncture
5. Medications
💡 FDA approved: Pregabalin (Lyrica), duloxetine (Cymbalta)
- Tricyclic antidepressants (amitriptyline, duloxetine)
- Anticonvulsants (pregabalin, gabapentin)
- Muscle relaxants (tizanidine)
Cuccurollo 4th Edition Chapter 3 Rheumatology pg141-142
Sjogren’s syndrome: Define & List 4 clinical features associated.
Conditions that can cause Sjogren’s (associated conditions or secondary?).
Sjögren’s syndrome is an autoimmune-mediated disorder of the exocrine glands
- Dry eyes, corneal ulcers, conjunctivitis
- Dry mouth, dental caries
- Dry nose & throat, chronic cough
- Dry skin
- Dry vagina
Causes
- Primary → ANA (+), RF (+)
- Secondary to rheumatological disorder
- SLE → Face
- Rheumatoid arthritis → Hand
- Scleroderma → Hand
- Polymyositis/dermatomyositis → Muscles
- Autoimmune thyroiditis (hashimoto thyroiditis) → Gland
Extraarticular
- Arthralgias
- Raynaud’s phenomenon
- Vasculitis
- Myelopathy
- Periphral neuropathy
- Mononeuritis multiplex
- CN involvement (5, 7, 8)
Cuccurollo 4th Edition Chapter 3 Rheumatology pg134
Ref: Current diagnosis and treatment in rheumatology pg 384; neuromuscular website http://neuromuscular.wustl.edu/antibody/sneuron.html#sjogren.
Diagnostic Criteria in Kawasaki Disease 🔑🔑 MOCK
FACE
- Conjunctival injection
- Strawberry tongue
- Bright red, chapped lips
- Pharyngeal erythema
- Cervical lymphadenopathy
BACK
- Truncal rash
EXTREMITIES
- Edema of the hands or feet
- Erythema of the palms or soles with desquamation in later stages
SYSTEM
- High grade fevers lasting >5 days
Cuccorollo 4th Edition Chaper 10 Peds pg762
American College of Rheumatology (ACR) Diagnostic Criteria for JIA
💡 Juvenile (16yo) Idiopathic (No other causes) Arthritis (More than 1 Joint)
- Onset <16 years of age
- Persistent arthritis in ≥1 joint for at least 6 weeks
- Exclusion of other types of childhood arthritis: rheumatic fever, infection, SLE, vasculitis, etc
- Type of onset of disease during the first 6 months classified as polyarthritis, oligoarthritis, or systemic arthritis with intermittent fever
Cuccurollo 4th Edition Chapter 3 Rheumatology pg117
7 y/o child with inflammation in 2 joint, has +ve ANA (a) Diagnosis (b) Most important extra-articular manifestation.
Diagnosis
- Oligoarticular JIA.
Most Important
- Anterior uveitis (iridocyclitis).
- Chronic iridocyclitis—leads to cataracts, glaucoma, or blindness (often asymptomatic)
- Ophthalmology referral is mandatory.
- Slit lamp exam is required four times per year, for 4–5 years.
Key Points
- ANA (+), RF (–)
- HLA-B27 (+)
- No erosions
Cuccurollo 4th Edition Chapter 3 Rheumatology pg117-118
Picture hand + xray of child with polyarthritis. (a) Diagnosis (b) Extra-articular manifestations.
Polyarticular JIA
- Either RF +ve or RF -ve
- 5 or more joints involved.
Extra-Articular
- Uveitis (rare).
- Growth disturbance
- Delayed puberty
- Leg length discrepancy
- Osteoporosis / osteopenia
- Fever
- Anemia
- Nutritional impairment
- Micrognathia
- Bony overgrowth
Ref: Australian Family Physician Vol. 39, no. 9, september 2010
Specific Joints of Involvement in JIA
- Cervical spine: Subluxation of the atlantoaxial joint due to erosion of the transverse ligament
- Temporomandibular joint (TMJ): Micrognathia
- Wrist: Early loss of extension with progression of flexion contracture (Remember Splint)
- Hand: Swan-neck deformity, Boutonnire deformity
- Ankle/Foot: flat foot gait due to metatarsophalangeal joint pain
Cuccurullo 4th Edition Chapter 10 Peds pg757
Myofascial Pain Syndrome 🔑🔑 Dr. Dia’a & Dr. Aziz
Definition
How much pressure is needed to stimulate the pain?
List 4 Precipitating Factors
Acute & Chronic Management
MYOFASCIAL PAIN SYNDROME
- Regional pain disorder, characterized by hypersensitive areas of taut muscle bands called myofascial trigger points (TrPs) most commonly located in the upper traps, rhomboids, and paraspinals.
- Trigger point is distinguished from a tender point by a circumscribed area of tenderness with a palpable, tense band of muscle fibers that causes concordant pain in a referred pain pattern with an associated local twitch response upon palpation. Also it can exhibit sensory, motor, and autonomic symptoms.
- Palpation or needling of a trigger point may elicit an involuntary focal muscle contraction called the twitch response.
- Chronic pain can be related to central sensitization
DIAGNOSIS
- Palpation with 2-4 kg/cm2 of pressure for 10 to 20 seconds over the suspected trigger point to allow the referred pain pattern to develop
PRESENTATION
- Trigger points (hypersensitive areas) that are localized with referred pain and twitch response
- Muscle tenderness, spasm
- Decreased ROM and weakness due to the pain
- Nonmuscular symptoms including paresthesias, poor sleep patterns, and fatigue
- Normal neurologic exam
- Normal radiology
ETIOLOGY
- Postural mechanics
- Overuse injuries
- Deconditioning
- Trauma
- Stress
PERCIPITATING FACTORS
- Central pain generator: Fibromyalgia (widespread pain problem, not a regional condition caused by specific TrPs.)
- Psychology: Depression, Anxiety, PTSD
- Sleep Disorder
- Mechanical
- Poor biomechanics
- Poor posture leading to repetitive microtrauma
- Decreased range of motion (ROM) of the muscle tissue.
- Regional pain and stiffness (arthritis, radicular pain)
- Muscle overload or repetitive activities.
- Vitamin deficiencies: C & B
- Hypothyroid
ER - POLICE - MIS
💡 “Massage, stretching, and activity are most effective.” Braddom
- EDUCATION & RISK FACTORS
- Correct underlying causes
- Psychological counseling: CBT
- LOADING:
- Flexibility & postural alignment
- Stretching trigger points
- Massage trigger points
- Aerobic exercises
- Strengthening & Spine stabilization
- MODALITIES
- Heat/ice, ultrasound
- Transcutaneous electrical nerve stimulation (TENS
- Cooling analgesic spray
- MEDICATION
- Muscle relaxant medications
- NSAIDs
- Amitriptyline → improve sleep cycle.
- Transdermal lidocaine patches
- INJECTION
- Trigger point injection with or without injectants (dry needling)
- Botulinum toxin has been used in significant, refractory cases
Cuccurullo 4th Edition Chapter 10 Pain Syndromes p829
Cuccurullo 4th Edition Chapter 4 Soft Tissue Disorder of the Spine pg311
Braddom 5th Edition Chapter 15 Therapeutic Exercise pg313
https://emedicine.medscape.com/article/313007-overview
What are the 5 major and 3 minor diagnostic criteria for myofascial pain syndrome? 🔑🔑
Major Criteria
- A patient’s regional pain complaint;
- Identification of a palpable taut band;
- Palpation of a trigger point elicits a stereotypic zone of referred pain specific to that muscle;
- As well as a palpable, and exquisitely tender spot along the length of that taut band;
- Some degree of a restricted range of motion of the involved muscle
Minor Criteria
- Reproduction of spontaneously perceived pain and altered sensations by pressure on a trigger point.
- Elicitation of a local twitch response of muscular fibers by transverse “snapping” palpation or by needle insertion into the trigger point.
- Pain relief is obtained by muscle stretching or injection of the trigger point.
Ref: http://www.pain-education.com/managing-muscle-pain-in-medical-practice.html
List 4 Contraindications to trigger point injections. 🔑🔑
DRUG - NEEDLE - LAYERS
- Anticoagulation or bleeding disorders.
- Aspirin ingestion within 3 days of injection.
- Local/systemic infection.
- Allergy to anesthetic agents.
- Acute muscle trauma.
- Extreme fear of needles.
Ref: 2002 – AAFP – trigger point injections, diagnosis and management.
List 4 Complications of trigger point injections. 🔑🔑
- Vasovagal syncope.
- Skin infection.
- Pneumothorax.
- Needle breakage.
- Hematoma.
Ref: 2002 – AAFP – trigger point injections, diagnosis and management.
