Musco - Connective Tissue - Idiopathic Flashcards

1
Q

Fibromyalgia

A

A syndrome of widespread diffuse chronic pain of idiopathic etiology

AKA - Fibromyositis, Fibrositis, Idiopathic myalgia, Interstitial myofibrositis, Muscular rheumatism, musculorheumatism, myofibrositis, myogelosis, myositism, nodular rheumatism,nonarticular rheumatism

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2
Q

Fibromyalgia - Epidemiology

A
  • Chronic pain and fatigue are extremely prevalent in the general population, especially among women and persons of lower socioeconomic status
  • Female to male ratio 9:1
  • Prevalence in 1990 was 3.4% of women, and 0.5% of men
  • Most prevalent in women 50 years of age or older.
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3
Q

Fibromyalgia - Pathogenesis

A
  • Possible sleep disturbance
  • Possible disturbance of serotonin metabolism
  • Possible disturbance in growth hormone production
  • Possible disturbance in hypothalamic-pituitary-adrenal axis
  • Possible dysregulation in the autonomic nervous system
  • Possible over production of Substance P
  • Possible dysregulation in the central pain modulation system
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4
Q

Fibromyalgia - Presentation

A
  • Generalized muscle pain
  • Increased pain on exertion
  • Pain described as burning or gnawing or as a sore, stiffness that is aching
  • Perception of swollen joint, not present on physical examination
  • Fatigue and exhaustion and complain of waking up tired
  • Can be accomplished with cognitive impairment, difficulty thinking, loss of short-term memory
  • Anxiety and depression
  • Stress aggravates symptoms
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5
Q

Fibromyalgia - Diagnostic Testing

A
  • Widespread musculoskeletal pain of at least three months duration
  • Significant pain or tenderness in 11 out of 18 tender points
  • Normal neurologic exam
  • No laboratory abnormalities suggestive of other rheumatologic diseases
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6
Q

Fibromyalgia - Treatment

A
  • Improve quality of sleep
  • Exercise is critical; only consistently beneficial treatment to date
  • Treat depression and anxiety if present
  • Treat musculoskeletal issues involving somatic dysfunction (OMT)
  • Maintain adequate circulation
    *Acupuncture
  • Pharmacologic approach can include:
  • low dose tricyclic antidepressants (amitriptyline)
  • cyclobenzaprine (Flexeril)
  • SSRIs
  • Gabapentin
  • Pregabalin (lyrica)
    NSAIDs
  • Zolpidem (ambien)
  • dextromethorphan
  • capsaicin
  • Guifenesin (no evidence of efficacy)
  • melatonin
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7
Q

Fibromyalgia - Management with complications

A
  • Institute a program of low impact daily exercise when appropriate
  • Stretching exercises
  • Avoid all glucocorticoids and opiates based treatments
  • Other therapies that can be helpful include:
  • biofeedback
  • Behavioral modification
  • hypnotherapy
  • stress management
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8
Q

Fibromyalgia - Prevention and Health promotion

A
  • Identification and alteration of stressful conditions in life
  • Diet
  • decrease caffeine intake
  • increase tryptophan containing foods (turkey, bananas, skim milk, peanut butter)
  • vitamin supplement, esp B6
  • Group or individual therapy can be helpful
  • Patient education and reassurance
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9
Q

Polymyalgia Rheumatic

A
  • Polymyalgia rheumatica is of idiopathi origin, possible related to an infection, and can feature pain and stiffness in the neck, shoulders, and pelvic region. PM is closely related to giant cell arteritis and could be a manifestation of the later disease.
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10
Q

Polymyalgia Rheumatic - History

A
  • One study revealed a prevalence of 1 in 200 people aged 50 years or older
  • Can occur in isolation, but is comorbid in 40-50% of patients with giant cell arteritis
  • PMR is not a life threatening disease, but it does require treatment for 2-4 years
  • Mean age of onset is 70 years, range 50-90
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11
Q

Polymyalgia Rheumatic - Presentation

A
  • Onset can be abrupt or insidious
  • Patient typically in good health before developing PM
  • Low grade fever and weight loss present in over half the patients
  • Arthralgias and myalgias
  • Morning stiffness and getting up after inactivity are common complaints
  • Malaise, fatigue, and depression
  • Typically the shoulder girdle is the first area involved but hips or neck can also present initially
  • Late stages can feature muscle atrophy
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12
Q

Polymyalgia Rheumatic - Diagnostic Testing

A
  • History and physical exam
  • ESR greater than 50 mm/h
  • Normochromic normocytic anemia in 50% of cases
  • Normal creatinine kinase level
  • Rheumatoid factor negative
  • Negative muscle biopsy
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13
Q

Sarcoidosis

A
  • Chronic, multisystem granulomatous disorder of unclear etiology characterized pathologically by the accumulation of immune cells and the formation of noncaseating granulomas in effected tissue.

AKA - Lofgrens syndromeis an acute subgroup of sarcoidosis.

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14
Q

Sarcoidosis - Epidemiology

A
  • Incidence rnages from 5-40 per 100,000
  • Incidence for whites is 11 per 100,000
  • Incidence for african americans is 34 cases per 100,000
  • Prevalence is 10 x greater for african americans than for whites
  • More frequent in women than in men
  • Most often presents between ages 20 to 40 but ranges from children to the elderly
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15
Q

Sarcoidosis - Etiology

A
  • Disordered immune regulation in the setting of a genetic background and an environmental trigger
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16
Q

Sarcoidosis - Pathogenesis

A
  • Most common organ involvement is lung, eye and skin lesions are next in frequency
  • Accumulation of T-cells and mononuclear phagocytes, noncaseating epithelioid granulomas and derangement of normal tissue architecture
  • TH1 cell process
17
Q

Sarcoidosis - Presentation

A
  • Most common presentation involves insidious onset of shortnessof breath, cough, chest pain, and hemoptysis (40-70% of cases)
  • The same presentation can occur in the setting of constitutional signs as well
  • Subacute presentations also occur involving several weeks duration
  • Bilateral hilar lymphadenopathy or lung involvement is present on chest rediographs in 90% of cases
  • Incidental finding on a chest film is a common presentation (10-20% of all cases)
  • Other presentations involve peripheral lymphadenopathy with cutaneous lesions, eye involvement, splenomegaly, and hepatomegaly
  • Arthralgias and arthritis present in 25-50% of cases
  • Other symptoms can be very protean due to the wide range of systems involved in the disease
  • Temporal profile can be unrelentingly progressive or fluctuant
  • Overall, 65-70% of individuals recover with minimal residual
18
Q

Sarcoidosis - Diagnostic Testing

A
  • History and physical exam
  • Routine blood
  • Chest x-ray
  • Biopsy
19
Q

Sarcoidosis - Treatment

A
  • Glucocorticoids first line

* Methotrexate second line

20
Q

Amyloidosis

A
  • Rare, multisystem chronic disease.
  • It features the deposition of amyloid - a proteinaceous substance - between cells in various tissues of the body.
  • The disease can be systemic in nature, or it can target individual organs. It can also be classified as primary or secondary

AKA - Generalized amyloidosis

21
Q

Amyloidosis - History

A
  • There are approx. 2450 new cases in the US annually, and prevalence is 0.89/100,000
  • Amyloid is a proteinaceous substance of pathological origin that is deposited in the tissues of the body.
  • Amyloidosis results from the abnormal folding of a protein, which are then deposited as fibrils in the extracellular tissue, and can disrupt function
22
Q

Amyloidosis - Three types

A
  • AL or light chain amyloid is derived from plasma cells and contains immunoglobulin light chains
  • AA or amyloid associated protein secreted by the liver
  • Ab or beta amyloid found in the brain
  • Amyloidosis is a group of diseases all having in common the deposition of amyloid in tissue
23
Q

Amyloidosis - Classification

A
  • Systemic amyloidosis
  • primary amyloidosis (AL amyloidosis)
  • —Immunocyte dyscrasis
  • secondary amyloidosis (AA amyloidosis)
  • —Reactive amyloidosis due to chronic diseases
  • Localized amyloidosis
  • senile cerebral amyloidosis (Alzheimers disease and other senile diseases)
  • endocrine amyloidosis
  • atrial amyloidosis
  • Prion Disease
24
Q

Amyloidosis - Presentation

A
  • Can be asymptomatic insidious onset of non-speficic symptoms such as weakness, light headedness, synocope, or loss of weight
  • Later stage symptoms depend on the organ system effected
25
Q

Amyloidosis - DIagnostic testing

A
  • Biopsy and congo red staining