Rheumatic Disorders Part IV

Rheumatic Disorders Part IV:

Maria Carmela L. Domocmat, RN, MSN

Instructor, School of Nursing

Northern Luzon Adventist College

Fibromyalgia, Spondyloarthropathies

(Polymyositis, Dermatomyositis), Bursitis,

Vasculitis, Polymyalgia rheumatica, Giant cell

arteritis , Mixed connective tissue disease,

Lyme disease

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


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

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

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/


http://www.nlm.nih.gov/medlineplus/ency/article/000839.htm


http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001842/

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001842/

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 Maria Carmela L. Domocmat, RN, MSN



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 the need for a transplant


Health & Medicine

Rheumatic Disorders Part IV