Rheumatic Disorders Part IV

Rheumatic Disorders Part IV:
Fibromyalgia, Spondyloarthropathies
(Polymyositis, Dermatomyositis), Bursitis,
Vasculitis, Polymyalgia rheumatica, Giant cell
arteritis , Mixed connective tissue disease,
Lyme disease

Maria Carmela L. Domocmat, RN, MSN
Instructor, School of Nursing
Northern Luzon Adventist College

Fibromyalgia (FM)
Idiopathic inflammatory myopathy
Bursitis
Vasculitis
Polymyalgia rheumatica
Giant cell arteritis
Lyme disease
Sarcoidosis


Fibromyalgia (FM)

is a disorder of chronic widespread pain
with associated fatigue, poor sleep, stiffness, cognitive difficulties,
multiple somatic symptoms, and, not infrequently, anxiety and/or
depression.


Fibromyalgia (FM)

Pain - radiates diffusely from the axial skeleton
over large areas of the body,
predominantly involving muscles and musculoskeletal
junctions,
but also in joints (arthralgia without actual synovitis)
described as exhausting, burning, miserable, or unbearable.
may also be multifocal and can wax and wane in a migratory
fashion.
Described as "pain all over."
However, multifocal pain or recurrent episodes of regional pain are
essentially equivalent to the classic "pain all over" description.


Algometer or dolorimeter
A useful device
for rough
quantitation of
pain sensitivity
is a pressure
algometer, or
dolorimeter.


Tender points in fibromyalgia.


Fibromyalgia (FM)

Fatigue and poor sleep
Most patients with fibromyalgia also meet the classification criteria
for chronic fatigue syndrome.


Fibromyalgia (FM)

Cognitive problems
(known as "fibrofog") - primary symptom of
fibromyalgia,
reflecting impairments in working, episodic, and
semantic memory that are roughly equivalent to 20
years of aging.
Cognitive symptoms associated with
fibromyalgia are exacerbated by pain, mood
and anxiety disorders, and poor sleep.


Other common symptoms
Weight fluctuations
Allergic symptoms (eg, nasal congestion) and
hypersensitivity to environmental stimuli (eg, odors, bright
lights, loud noises) and medications
Regional pains, including noncardiac chest pain,
dyspepsia, headache, abdominal cramping (irritable bowel
syndrome), temporomandibular pain, chronic pelvic pain,
and others (Patients with fibromyalgia may meet criteria for
3 or more central sensitivity syndromes.)


Other common symptoms
Syncope or dizziness
Shortness of breath
Urinary frequency and urgency (female urethral syndrome,
interstitial cystitis)


Causes
multifactorial.
Engel's biopsychosocial model of chronic illness
(ie, health status and outcomes in chronic illness
are influenced by the interaction of biologic,
psychologic, and sociologic factors) provides a
useful way to conceptualize fibromyalgia


Laboratory Studies
do not have characteristic or consistent abnormalities as
determined by laboratory test results.
Laboratory studies - important to help rule out diseases
with similar manifestations


Laboratory Studies
Thyroid-stimulating hormone:
hypothyroidism shares many clinical features with fibromyalgia,
especially diffuse muscle pain and fatigue.
Creatinine phosphokinase (CPK)
to exclude inflammatory myopathies
Erythrocyte sedimentation rate (ESR):
The normal ESR in patients with fibromyalgia contrasts with the
high ESR in elderly patients with polymyalgia rheumatica.
Obtaining an ESR can assist in identifying an underlying
inflammatory disorder or occult malignancy.


Laboratory Studies
Antinuclear antibodies (ANAs):
Many patients with SLE have comorbid fibromyalgia. A low-titer
ANA is common in the general population and may be of no clinical
significance if diagnostic features of SLE or related autoimmune
disorders are absent.
Rheumatoid factor:
Many patients with RA have comorbid fibromyalgia. However, a
positive result for rheumatoid factor does not support a diagnosis of
RA in the absence of objective evidence of characteristic joint
inflammation. A positive result for rheumatoid factor is
diagnostically nonspecific in other clinical settings.


Treatment
validation of the patient’s illness
empathetic listening and acknowledgment that the
patient is indeed experiencing pain
first crucial element in the treatment of pain, fatigue,
and other diverse symptomatology in patients with
fibromyalgia (FM) I


Treatment
Accurately assess possible causal or perpetuating
factors,
including attention to psychologic and sociocultural
factors
and identification of specific regional sources of ongoing
nociceptive pain (eg, degenerative spondylosis,
bursitis).


Comments such as "it’s all in your mind" or "I
cannot find anything wrong with you" only add to
the patient's frustration.


Psychologic and behavioral
approaches
Depression must be treated aggressively.
Depression, anxiety, stress, sleep disturbance, pain
beliefs and coping strategies, and self-efficacy all are
central to the pain experience in many patients and
frequently determine the outcome of chronic pain.
Unless psychosocial and behavioral variables are
recognized and approached, strictly
pharmacologic interventions are of limited benefit.


Psychologic and behavioral
approaches
Cognitive-behavioral therapy (CBT) and operant-
behavioral therapy (OBT)
both effect clinically meaningful improvements in pain
intensity and physical impairment in approximately one
third to on half of patients with fibromyalgia.


Patient Education
Education is an essential element in therapy for
fibromyalgia.
It begins with an empathetic manner on the part of
the nurse/physician, who must affirm the patient's
pain, explore social and behavioral variables (both
in childhood and current) that influence this
illness, and explain to the patient how stress and
distress can amplify pain and fatigue.


Medication
Anxiolytics/hypnotics
Antidepressants
Tricyclics antidepressants
Selective Serotonin-reuptake Inhibitors (SSRIs)
Serotonin Norepinephrine Reuptake Inhibitors (SNRIs)
Central Nervous System Depressants
Opioids
Anticonvulsants
Analgesics
Nonsteroidal anti-inflammatory drugs (NSAIDs)


often used in combination with antidepressants
and anticonvulsant drugs (both of which also have
efficacy for anxiety and insomnia)
Benzodiazepines
alprazolam [Xanax]
temazepam [Restoril
clonazepam [Klonopin]
buspirone [BuSpar]
trazodone [Desyrel])


Tricyclic antidepressant
Amitriptyline (Elavil)
Desipramine (Norpramin)
Doxepin (Sinequan)
Imipramine (Tofranil)
Trazodone (Desyrel)
Nortriptyline (Pamelor)


Selective Serotonin-reuptake
Inhibitors (SSRIs)
Fluoxetine (Prozac)
Sertraline (Zoloft)
Paroxetine (Paxil)
Fluvoxamine (Luvox)
Citalopram (Celexa)


Serotonin Norepinephrine
Reuptake Inhibitors (SNRIs)
Milnacipran (Savella)
Duloxetine (Cymbalta)


Central Nervous System
Depressants
Zolpidem (Ambien)
Zaleplon (Sonata)
Sodium oxybate (Xyrem)


Opioids
Morphine
Oxycodone (OxyContin)
Hydrocodone (Vicodin, Percocet)
Hydromorphone (Dilaudid)
Meperidine (Demerol)


Anticonvulsants
Gabapentin (Neurontin)
Pregabalin (Lyrica)


Pain Relievers
Acetaminophen (Tylenol)


Nonsteroidal anti-inflammatory
drugs (NSAIDs)
Aspirin
Ibuprofen (Advil)
Naproxen (Aleve)


Sodium oxybate (Xyrem)
a sedative hypnotic, prolongs stage III/IV
restorative sleep, which is essential to awaken
rested and refreshed.


Anticonvulsants
Pregabalin (Lyrica)
Gabapentin (Neurontin)
Clonidine (Catapres)


Polymyositis
a persistent inflammatory muscle disease that
causes weakness of the skeletal muscles, which
control movement.
Medically, polymyositis is classified as a chronic
inflammatory myopathy — one of only three such
diseases.


Polymyositis
can occur at any age,
adults -30s, 40s or 50s.
Blacks
Women


Polymyositis
signs and symptoms usually develop gradually,
over weeks or months.
Remissions - rare

Remissions: periods during which symptoms spontaneously
disappear

Signs and symptoms
appear gradually,
Progressive muscle weakness
Difficulty swallowing (dysphagia)
Difficulty speaking
Mild joint or muscle tenderness
Fatigue
Shortness of breath


Signs and symptoms
affects the muscles closest to the trunk,
particularly hips, thighs, shoulders, upper arms
and neck.
weakness is symmetrical
worsens over time.
As muscle weakness progresses,
difficult to climb stairs, rise from a seated position, lift
objects or reach overhead.


Complications
Dysphagia
Which in turn may cause weight loss and malnutrition.
Aspiration pneumonia
Shortness of breath or respiratory failure.
Calcinosis
Calcium deposits in muscles, skin and connective
tissues


Associated conditions
polymyositis is often associated with other
conditions that may cause further complications
of their own, or in combination with polymyositis
symptoms. Associated conditions include:
Raynaud's phenomenon.
Other connective tissue diseases.
Cardiovascular disease.
Lung disease.


Treatment
corticosteroid
When muscle strength improves, usually in 4 to 6
weeks, the medication is slowly tapered off.
Maintenance therapy with prednisone may be
continued indefinitely.
DMARDs - If unresponsive to corticosteroids
methotrexate and azathioprine,


Treatment
Intravenous gamma globulin
IVIG
is a purified blood product that contains healthy
antibodies from thousands of blood donors.
The healthy antibodies in IVIG can block the
damaging antibodies that attack muscle in
polymyositis.
Repeat infusions q 6-8 weeks


Other immunosuppressive
medicine
Tacrolimus (Prograf)
is a transplant-rejection drug that may work to inhibit
the immune system.
Immunosuppressants,
cyclophosphamide (Cytoxan) and cyclosporine
(Gengraf, Neoral, Sandimmune),


Biological therapies
Rituximab (Rituxan)
Tumor necrosis factor (TNF) inhibitors
etanercept (Enbrel) and infliximab (Remicade),


Other treatment approaches
Physical therapy
Dietetic assessment
Speech therapy


Nursing management
Coping and support
Educate about the illness
Balance Rest and exercise


Sources
http://www.mayoclinic.com/health/polymyositis/DS00334/METHOD=
print&DSECTION=all
http://www.mayoclinic.com/health/polymyositis/DS00334


Dermatomyositis
a muscle disease characterized by inflammation
and a skin rash. It is a type of inflammatory
myopathy.
5 - 15 and adults age 40 - 60.
Women
Polymyositis is a similar condition, but the
symptoms occur without a skin rash.


Symptoms
Dysphagia
Muscle weakness, stiffness, or soreness
Purple or violet colored upper eyelids
Purple-red skin (violaceous) rash
SOB


Symptoms
The muscle weakness may appear suddenly or develop
slowly over weeks or months. may have difficulty raising
arms over head, rising from a sitting position, and
climbing stairs.
The rash may appear over the face, knuckles, neck,
shoulders, upper chest, and back.


reddish-purple (violaceous) rash
reddish-purple
(violaceous) rash.
The rash is named after
the tendency of plants to
grow toward the sun
(heliotropic) and is
characteristic of
dermatomyositis.


purple (violaceous) plaques
The appearance of purple
(violaceous) plaques on
the knees may be
associated with
dermatomyositis.


Gottron's sign
Red, thickened, scaly skin over the knuckles


Heliotrope eyelids
eyelids develop a brown
(violaceous - rather than
red) color.
Heliotrope eyelids and
Gottron's papules on the
knuckles are
characteristic findings in
dermatomyositis.


violet-colored inflammation (erythema) over the
knuckles


periungual erythema
Candida paronychia produced periungual
erythema, edema and nail fold maceration.


Dx Exams
CPK & aldolase
ECG
Electromyography
Magnetic resonance imaging (MRI)
Muscle biopsy


Treatment
Corticosteroids
Immunosuppressants
When muscle strength gets better – taper off
corticos
However, most people take prednisone
indefinitely.
If the condition is associated with a tumor, the
muscle weakness and rash may improve when
the tumor is removed.


Outlook (Prognosis)
Some recover and have symptoms completely
disappear - especially in children.
In adults, death may result from severe and
prolonged muscle weakness,
malnutrition, pneumonia, or lung failure. The
major causes of death are cancer (malignancy)
and lung disease.


Possible Complications
Acute renal failure
Cancer (malignancy)
Inflammation of the heart
Joint pain
Lung disease


http://www.nlm.nih.gov/medlineplus/ency/article/
000839.htm
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH
0001842/


Bursitis
A painful inflammation of the bursae


Bursitis
bursae
closed, minimally fluid-filled sacs that are
lined with a synovium similar to the lining of
joint spaces
function: to reduce friction between adjacent
tissues (tendon and bones or tendon and
ligaments) by lubricating these enclosed
structures with synovial fluid from bursal sac


Bursitis
Bursae
there are 150 bursae in human body
cover bony prominences (e.g., olecranon, trochanter,
and patella)
or provide protection between the skin and other
structures (e.g., calcaneal bursa)
usually thin, but with repeated stress – can become
thickened and fluid-filled secondary to inflammation


Bursitis
peaks – 40 to 50 yrs
affected areas – shoulder joints (most
common), elbow, knee, hip; dominant arm


Etiology and risk factors
acute or chronic trauma (mechanical, highly
repetitive activities)
arthritic conditions (e.g., RA), gout, tumors,
degenerative changes
occupational or avocational activities (e.g., wood
carver – acute subacromial bursitis;
businesswoman walking long distance on high
heels – retrocalcaneal bursitis)


Clinical manifestations
exquisite localized pain in target area
point tenderness (can specifically point the spot
of greatest discomfort)
diffuse soreness radiating to the tendons at the
site
interrupted sleep (e.g., with subacromial bursitis,
calcaneal bursitis)


difficulty walking (e.g., trochanteric bursitis,
calcaneal bursitis)
difficulty performing ADL (e.g., with subacromial
or olecranon bursitis)


Dx
diagnosis is based on PE and history
radiographs – usually normal in acute bursitis;
calcium deposits in chronic
lab tests and synovial fluid analysis – normal
unless bursa become infected


Management
Goals
Rests and immobilization of affected joint
Non-opoiod analgesics
ROM exercises
NSAIDs


Nursing Management
Client education
Focus on causes and prevention of additional attacks
by avoiding activities that cause constraint friction or
pressure
correct application of moist heat
medication
exercise instruction
intra-articular injections of cortisone


Nursing Diagnoses
Acute or Chronic Pain
Impaired Physical Immobility
Temporary Self-Care Deficits


Interventions
Goal: pain reduction (without pain reduction –
joint mobility is impaired thru guarding,
protective measures)
Teach purpose, dose and side effects of anti-
inflammatory meds
Resting or immobilizing joint or elevating or
compressing involved area to control edema
Teach about correct application of ice ad heat


Teach postinjection flare of intra-articular
cortisone
Self-care: oversized garment, especially those
with long sleeves or wide pant legs
Minimize shoulder or elbow pain – by putting
clothing on affected arm first and by taking it off
the affected arm last.


Vasculitis
a group of disorders leading to inflammation and
necrosis of blood vessel walls
includes:
polyarteritis nodosa
systemic necrotizing vasculitis
allergic granulomatous angitis


Vasculitis
Pathophysiology
soluble immune complexes are deposited in blood
vessel walls in areas where capillaries have
increased permeability
after deposition, the immune system is activated and
the complex is destroyed along with the blood vessel
wall
inflammation and damage to large and small vessels
result in end-stage organ damage


vary
depending on organs affected


Management
Steroids


Polymyalgia rheumatica
a clinical syndrome
more common women
disease of aging, rarely occur before age 60
years


pain and stiffness in neck, shoulder, back, and
pelvic girdle esp in the morning
headaches or painful areas on head
low grade fever
temporal arteritis


Dx
Elevated ESR
mild anemia
elevated Ig


Management
steroids


AKA temporal or cranial arteritis
disease of aging


a clinical syndrome
more common women
disease of aging, rarely occur before age 60
years


polymyalgia rheumatic for months, then
suddenly experiences severe headaches assoc
with temporal arteritis
sudden onset with severe pain often appearing
in temporal area (can also be in occipital area,
face, or side of neck


hyperesthesia (unusual or pathological
sensitivity of the skin or of a particular sense of
stimulation) – makes any touch exquisitely
painful
visual changes – blindness in one or both eyes


Management
Corticosteroids


Mixed connective tissue disease
a combination of several connective tissue
diseases
frequent combinations are SLE and SSc and RA


Mixed connective tissue disease
clinical manifestations
have manifestations that are not typical of any one
disorder

management
according to manifestations


Complex multisystem disease
One of form of rheumatic joint disease with a
known cause
Included as a connective tissue disorder bcoz
the skin, joint, nervous system, and heart are
involved


Etiology and risk factors
cause: spirochete Borrelia burgdorferi

Male tick Female tick


Risk factors
Doing activities that increase tick exposure (for
example, gardening, hunting, or hiking)
Having a pet that may carry ticks home
Walking in high grasses

Tick imbedded in the skin
This is a close-up photograph of a
tick embedded in the skin. Ticks are
important because they can carry
diseases such as Rocky Mountain
spotted fever, tularemia, Colorado
tick fever, Lyme disease, and others.
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001690/bin/2060.jpg


Large ―bull’s –eye‖ circular rash; red flat rash
that clears in the center
severe headache
severe malaise
stiff neck
fever chills
myalgias
joint pain
fatigue

3 stages of Lyme disease
Stage 1 : primary Lyme disease.
Stage 2 : secondary Lyme disease and early
disseminated Lyme disease.
Stage 3 : tertiary Lyme disease and chronic
persistent Lyme disease


Stage 1 :
primary Lyme disease.
Occurs 3 to 32 days after the bite
Flu-like symptoms , bull’s eye rash, pain and stiffness
in muscles and joints
For some – arthritis is the first and only sign of the
disease


Stage 2 :
secondary Lyme disease and early disseminated
Lyme disease.
Occurs 2-12 wks after bite
Carditis with dysrhtmia, dyspnea, dizziness,
palpitations
CNS disorders – meningitis, facial paralysis,
peripheral neuritis


Stage 3
tertiary Lyme disease and chronic persistent Lyme
disease
develop months or years after first develop Lyme
disease infection
Occurs when disease is not diagnosed and treated in
earlier stages
s/s - arthralgias, fatigue, memory/thinking problems


Management
Stage 1
Antibiotic therapy – PO, for 10-21 days
Doxycycline
Amoxicillin
Cefuroxime
Stage 2
IV Antibiotic therapy
ceftriaxone, cefotaxime


Management
Intra-articular steroids and NSAIDs
To reduce inflammation and pain


Client Education guide
Avoid heavily wooded areas or areas with thick
underbrush
Walk in the center of the trail
Avoid dark clothing. Lighter-colored clothing
makes spotting ticks easier
Use an insect repellent on your skin and clothes
when in an area where ticks are likely to be
found
Wear long-sleeved tops and long pants


Wear closed shoes and a hat or cap
Bathe immediately after being in an infested
area, and inspect your body for ticks (abt the
size of a pinhead); pay special attention to arms,
legs and hairline
Gently remove with tweezers, or finger any tick
that you find. Dispose of the tick by flushing it
down the toilet (burning could spread infection)


Wait 4-6 weeks after being bitten by a tick b4
being tested for Lyme disease (testing b4 this
time is not reliable)
Report symptoms, such as rash or influenza-like
illness, to the physician
Obtain a vaccine to prevent disease if you live
in a high-risk area


Complications
long-term joint inflammation (Lyme arthritis)
Arrhythmia
Brain and nervous system (neurological)
problems


Sarcoidosis is a disease in which swelling
(inflammation) occurs in the lymph nodes, lungs,
liver, eyes, skin, or other tissues.


Causes
The cause of the disease is unknown. In
sarcoidosis, clumps of abnormal tissue
(granulomas) form in certain organs of the body.
Granulomas are clusters of immune cells.
The disease can affect almost any organ of the
body, but it most commonly affects the lungs.


Possible causes of sarcoidosis include:
Excess sensitivity to environmental factors
Genetics
Extreme immune response to infection


The condition is more common in African
Americans than Caucasians.
Females are usually affected more often than
males.
typically begins between the ages of 20 and 40.
very rare in young children.


Symptoms
There may be no symptoms. When symptoms
occur, they can involve almost any part or organ
system in your body.
Almost all patients have lung or chest
symptoms:
Dry cough
Shortness of breath
Discomfort behind your breast bone
Abnormal breath sounds (such as rales)


Symptoms of general discomfort or uneasiness
often occur:
malaise
Fatigue (one of the most common symptoms in
children)
Fever
Weight loss (one of the most common symptoms
in children)
Joint achiness or pain (arthralgia)


Skin symptoms:
Skin rashes
Old scars become more raised
Raised, red, firm skin sores (erythema nodosum,
almost always on the front part of the lower legs
Skin lesions
Hair loss


Nervous system (neurological) and vision
changes:
Headache
Seizures
Weakness or paralysis (palsy) on one side of the
face
Eye burning, itching, and discharge
Symptoms of uveitis
Decreased tearing


Other symptoms of this disease:
Enlarged lymph glands - armpit lump
Enlarged liver
Enlarged spleen
Dry mouth
Nosebleed

http://www.nlm.nih.gov/medlineplus/ency/ar
ticle/000076.htm

Exams and Tests
Often the disease is found in patients with no
symptoms who have an abnormal chest x-ray.
Different imaging tests
Chest x-ray to see if the lungs are involved or lymph
nodes are enlarged
CT scan
Lung gallium (Ga.) scan


Biopsies of different tissues may be done:
Lymph node biopsy
Skin lesion biopsy
Bronchoscopy to perform a biopsy
Open lung biopsy
Mediastinoscopy with biopsy
Liver biopsy
Kidney biopsy
Nerve biopsy
Heart biopsy


lab tests
CBC
Chem-7 or chem-20
Quantitative immunoglobulins (nephelometry
PTH
Serum phosphorus
Immunoelectrophoresis - serum
Calcium - urine
Calcium - ionized
Calcium - serum
Liver function tests


Treatment
Sarcoidosis symptoms often get better on their
own gradually without treatment.
Severely affected patients
corticosteroids (prednisone or methylprednisolone).
This includes people who have involvement of the eyes, heart,
nervous system, and some with lung involvement.
Therapy may continue for 1 or 2 years.
Some of the most severely affected patients may require life-long
therapy.


Treatment
Drugs that suppress the immune system
(immunosuppressive medicines),
methotrexate, azathioprine, and cyclophosphamide
are sometimes used in addition to corticosteroids.
Rarely, some people with irreversible organ failure require an organ
transplant.
Although these treatments may temporarily improve the
symptoms of the disease, long-term treatment has not
been proven to prevent sarcoidosis from slowly getting
worse.


Outlook (Prognosis)
Many people are not seriously ill, and the
disease may get better without treatment.
About 30 - 50% of cases get better without
treatment in 3 years.
About 20% of those whose lungs are involved
will develop lung damage.
The overall death rate from sarcoidosis is less
than 5%.


Outlook (Prognosis)
Causes of death include:
Scarring of lung tissue (pulmonary fibrosis)
Bleeding from the lung tissue
Involvement of the heart (rarely)


Possible Complications
Osteoporosis and other complications of taking
corticosteroids for longer periods of time.
Diffuse interstitial pulmonary fibrosis
Pulmonary hypertension
Fungal lung infections (aspergilloma
Anterior uveitis
Glaucoma and blindness (rare)
Cardiac arrhythmias
Cranial or peripheral nerve palsies
High calcium levels (hypercalcemia
Kidney stones
gan failure, leading to Carmela L. Domocmat, RN, MSN
Maria the need for a transplant

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