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Fall 2024 > Systemic Connective Tissue Diseases > Flashcards

Systemic Connective Tissue Diseases Flashcards

1
Q

What is sarcoidosis characterized by?

A

accumulation of granulomatous inflammation in involved tissues

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2
Q

What is most commonly involved in sarcoidosis?

A

The lungs; Bilateral hilar lymphadenopathy

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3
Q

What are the 3 most common presenting symptoms of sarcoidosis?

A
  1. Malaise
  2. Fever
  3. Dyspnea
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4
Q

What are some of the multisystem disorder components of sarcoidosis?

A

Lupus pernio
granulomatous uveitis
arthralgias/anorexia
Cardiomyopathy
*basically wherever the inflammation occurs/granulomas deposit

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5
Q

What diagnostics support sarcoidosis?

A

CBC - leukopenia
ESR - elevated
ACE levels - increased
Hypercalciuria
Imaging - bilateral hilar adenopathy
Diagnostic: Transbronchial biopsy via fiberoptic bronchoscopy

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6
Q

What should be done for patients with radiographic findings of sarcoidosis but no symptoms?

A

No treatment needed, but need to be monitored closely with eye exams, CXR, EKG and labs

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7
Q

How should moderate - severe sarcoidosis be treated?

A

Oral corticosteroids - long term therapy usually required over months to years

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8
Q

What labs will go down with clinical improvement of sarcoidosis?

A

Serum ACE levels

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9
Q

What is scleroderma?

A

Chronic systemic connective tissue disease with diffuse fibrosis of the skin and internal organs

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10
Q

What causes scleroderma?

A

Excessive collagen deposition

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11
Q

What is limited scleroderma

A

hardening of the skin is limited to the face and hands with minimal organ involvement

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12
Q

What is systemic scleroderma

A

significant organ involvement that typically has a poorer outcome

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13
Q

What is typically the first manifestation of both forms of scleroderma?

A

Raynaud phenomenon that usually starts years before other symptoms (white to red to blue)

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14
Q

What are the clinical manifestations of limited scleroderma?

A
  1. Thickening of the skin with loss of normal folds
  2. Digital ischemia leading to finger loss
  3. CREST syndrome
  4. Pulmonary HTN
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15
Q

What are the clinical manifestations of diffuse scleroderma

A
  1. Polyarthralgia, weight loss, and malaise are common early symptoms
  2. Skin is first organ affected
  3. Non-pitting edema and pruritus often come first
  4. Organs often infected are heart, lungs, kidneys, GI tract
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16
Q

What is CREST syndrome

A

Associated with limited scleroderma:
Calcinosis
Raynaud phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasias

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17
Q

What lab test findings support diagnosis of scleroderma?

A

Based on clinical findings
CBC may show anemia
Proteinuria if renal involvement
PFTs elevated if pulmonary involvement
***ANA is almost always positive
Anticentromere antibodies are highly specific for limited scleroderma

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18
Q

How is scleroderma treated?

A
  1. Mainly supportive
  2. DMTs
  3. Treatment is focused on involved organ systems
  4. Raynaud - CCB
  5. GERD - PPI and behavior modification
  6. HTN/renal crisis - ACE inhibitors
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19
Q

What is polymyalgia rheumatica?

A

Inflammatory disorder that causes muscle pain and stiffness

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20
Q

What is the onset of PMR?

A

Quick onset that is worse in the morning

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21
Q

What is the hallmark presentation of PMR?

A

Pain and stiffness in shoulders and hips lasting several weeks without other explanation

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22
Q

What other condition presents with PMR 1/3 of the time?

A

giant cell arteritis

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23
Q

What are the clinical manifestations of PMR?

A
  1. Pain in hips, shoulders, and lower back
  2. Trouble with combing hair, rising from chair, etc; due to pain and stiffness, not weakness
  3. Joint stiffness, esp after rest
  4. Systemic findings: Fever weight, loss fatigue
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24
Q

What are the diagnostic findings in PMR?

A
  1. CBC - may show anemia
  2. ESR and CRP usually markedly elevated
  3. RF, ANA, CCP ab, and CK all WNL which R/O RA
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25
Q

How are patients with PMR treated?

A
  1. Prednisone 12.5-25 mg daily
  2. Higher dose if above the neck symptoms
  3. Taper prednisone if patients respond well
  4. Oftentimes prednisone is needed for about a year
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26
Q

What is SLE?

A

Inflammatory autoimmune disorder characterized by autoantibodies to nuclear antigens

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27
Q

What are the clinical manifestations of SLE?

A

Systemic:
1. Fever
2. Anorexia/weight loss
3. Fatigue**/Malaise
Skin lesions:
1. Malar
2. Discoid lupus
3. Periungal erythema
4. Splinter hemorrhages
5. Raynaud
6. More

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28
Q

MSK findings of SLE

A

Joint pain that can occur with or without synovitis and is commonly the earliest presenting complaint; Avascular necrosis

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29
Q

Eye findings of SLE

A

Conjunctivitis
Photophobia
Transient or permanent blindness/blurring

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30
Q

Lung findings of SLE

A

Pleurisy
Pleural effusions
Bronchopneumonia
Pneumonitis

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31
Q

Cardio findings of SLE

A

Pericarditis
Arrythmias
HTN and myocarditis may lead to heart failure

32
Q

Neuro findings of SLE

A

Psychosis
Seizures
CVA
Severe depression made worse with steroids

33
Q

Renal findings of SLE

A

Acute or chronic renal failure
Glomerulonephritis
very common*

34
Q

GI findings of SLE

A

Mesenteric vasculitis

35
Q

Diagnostic process for SLE

A
  1. ANA positive, then,
  2. Anti-dsDNA and anti-Smith antibodies
    **Anti-Smith is most specific test for SLE, while ANA is most sensitive
  3. CBC may show anemia
36
Q

How is SLE treated?

A
  1. Target individual symptoms
    - Topical steroids for skin lesions
    - NSAIDs for joint pain
    - Avoid sun and use sunscreen - vit D supplements
    - Hydroxychloroquine helps prevent flares
    - yearly eye exams
  2. Oral steroids for more severe manifestations
  3. Belimumab
37
Q

What should always be considered with diagnosis of SLE?

A

Could be drug-induced; check med list

38
Q

What differentiated drug-induced SLE from SLE

A
  1. fewer clinical manifestations
  2. M=F
  3. Anti-dsDNA antibodies are absent
  4. Symptoms return to normal once drug is discontinued (may take up to 6 months)
39
Q

What is Sjogren’s syndrome

A

A systemic inflammatory autoimmune disorder which results in immune-mediated dysfunction of the lacrimal and salivary glands

40
Q

What are the common features of Sjogren’s syndrome?

A

Dry eyes and mouth

41
Q

What does Sjogren’s syndrome increase the risk of?

A

Lymphoma

42
Q

What is keratoconjunctivitis sicca

A

Symptoms of Sjogren’s syndrome; lymphocyte and plasma cell infiltration of the lacrimal glands resulting in decreased tear production

43
Q

What is Xerostomia

A

Symptoms of Sjogren’s syndrome:
1. Dry “cotton mouth”
2. Dental carries
3. Inability to taste or smell
4. Parotid enlargement

44
Q

What are the systemic manifestations of Sjogren’s syndrome?

A
  1. Dysphagia
  2. Pleuritis/interstitial lung disease
  3. Small vessel vasculitis
  4. Peripheral neuropathies
45
Q

What are the diagnostic lab findings in Sjogren’s syndrome?

A
  1. Often RF +
  2. ANA +
  3. CBC has mild anemia, leukopenia, Eosinophilia
  4. ESR usually elevated
    * no test is 100%
46
Q

What non-lab tests can be done to support diagnosis of Sjogren’s syndrome?

A
  1. Sialography - radiopaque material injected into salivary glands
  2. Salivary scintigraphy
  3. Minor salivary gland biopsy*** best definitive test - focal aggregates of at least 50 lymphocytes
47
Q

What is the treatment for Sjogren’s syndrome

A
  1. Supportive - artificial tears, keep mouth lubricated
  2. Medications - cyclosporine ophthalmic drop, pilocarpine and cevimeline (xerostomia)
    *Avoid atropinic drugs and decongestants
  3. Dental care
48
Q

Sjogren’s syndrome prognosis?

A

Great, no decrease in life expectancy

49
Q

What are polymyositis and dermatomyositis characterized by?

A
  1. bilateral proximal muscle weakness;
  2. idiopathic immune mediated inflammatory myopathy
50
Q

What do patient with dermatomyositis have a greatly increased risk of?

A

developing occult malignancies

51
Q

What are the clinical manifestations of polymyositis

A
  1. Gradual and progressive muscle weakness, typically proximal and starting in the legs
  2. Dysphagia in 1/3 of patients
  3. Muscle atrophy/contractures
  4. Respiratory weakness may lead to need for mechanical ventilation
  5. No dermatological involvement
52
Q

What is the difference between polymyositis and dermatomyositis?

A

Dermatomyositis = polymyositis + skin involvement

53
Q

What does the rash associated with dermatomyositis look like?

A

Dusky red, may be malar or beyond, Shawl sign, Heliotrope rash, Gottron sign

54
Q

What is amyotrophic dermatomyositis?

A

A form of dermatomyositis with no musculoskeletal symptoms

55
Q

How are PMS and DMS diagnosed?

A
  1. **CK and aldolase are elevated
  2. ANA +
  3. Muscle biopsy is diagnostic test**
56
Q

What additional testing should be done for patients with PMS and DMS?

A

CT or MRI to rule out occult malignancy:
1. Ovarian***
2. Lung
3. Pancreatic
4. Stomach
5. Colorectal
6. Non-Hodgkin Lymphoma

57
Q

How are DMS and PMS treated?

A
  1. Corticosteroids are mainstay (40-60mg)
  2. Methotrexate or azathioprine if CS CI
  3. Avoid sun exposure
  4. Poor prognosis if associated neoplasm
58
Q

What is polyarteritis nodosa?

A

A systemic vasculitis in which medium-sized and small muscular arteries are affected by necrotizing inflammatory lesions; all 3 layers are effected; microaneurysms form and may lead to hemorrhage, ulcerations, ischemia and thrombosis

59
Q

What infection is strongly related to polyarteritis nodosa?

A

Hep B

60
Q

What are the clinical manifestations of polyarteritis nodosa

A
  1. insidious onset of fever, malaise, weight loss
  2. Mononeuritis multiplex - infarct or ischemia of named nerves
  3. Pain in extremities
  4. HTN and GI involvement
  5. Lungs are rarely involved
61
Q

What are common skin findings of PN?

A
  1. Livedo reticularis
  2. Subcut nodules - often 1st sign**
  3. often skin ulcers
  4. Digital ischemia
62
Q

PN diagnosis?

A
  1. CBC - anemia, leukocytosis, thrombocytosis
  2. CRP and ESR elevated
  3. Rule out Hep B infection
  4. Elevated Cr and proteinuria
  5. Confirm dx by tissue biopsy or angiogram
63
Q

PN treatment?

A
  1. High dose corticosteroids
  2. Antivirals and plasmapheresis if Hep B +
  3. Cyclophosphamide if severe
  4. Poor prognosis
64
Q

What is Marfan syndrome

A

A systemic connective tissue disorder caused by a mutation in the fibrillin gene

65
Q

MSK sx of Marfan?

A
  1. Tall, long arms, arachnodactyly
  2. Scoliosis
  3. Chest deformity
  4. Joint hypermobility
66
Q

Eye sx of Marfan?

A
  1. Ectopia Lentis
  2. Severe myopia
  3. Glaucoma/cataracts
  4. Retinal detachment
  5. Enophthalmos
67
Q

Cardiac sx of Marfan?

A
  1. Mitral valve prolapse
  2. Dilation of aortic root
    **Leading cause of death is aortic dissection
68
Q

What are some other common findings in Marfan?

A
  1. Spontaneous pneumothorax
  2. Striae atrophicae
  3. Highly arched palate and dental crowding
  4. Dural ectasia
69
Q

How is Marfan syndrome duagnosed?

A

Ghent criteria; gene mutation not specific (FBN1)

70
Q

How is Marfan syndrome treated?

A
  1. Moderate physical activity restriction
  2. Beta Blockers to protect aorta
  3. Mitral valve replacement
  4. Annual echo of aorta
  5. Annual eye exams
  6. Surgically correct pectus excavatum/scoliosis
  7. Genetic counseling
71
Q

What is fibromyalgia?

A

Widespread musculoskeletal pain accompanied by sleep, memory, and mood issues; pain at known tender points

72
Q

What are the symptoms of fibromyalgia

A
  1. Aching pain and stiffness
  2. Fatigue
  3. Sleep disorders
  4. Chronic headaches
  5. IBS
  6. Depression/anxiety
  7. Even minor exertion worsens pain and increases fatigue
73
Q

What are the fibromyalgia PE findings?

A

WNL except for “tender points”

74
Q

How is fibromyalgia diagnosed?

A

Diagnosis of exclusion: many labs indicated to rule out other diseases
- If patient has had fever, weight loss, or other objective finding it is not fibromyalgia

75
Q

Treatment of fibromyalgia?

A
  1. Education
    -diagnosis is real even though no confirmatory tests
    -support groups
  2. Lifestyle changes
    -Avoid MSG and Aspartame
    -No tobacco
    -Good sleep habits
  3. Rehab/therapy
    -Limit activities
    -heat, massage, low-impact aerobics
    -behavioral therapy
  4. Medication
    -Lyrica, Cymbalta, Savella
    -AVOID: opioids, CS, NSAIDs
  5. Exercise is beneficial

Fall 2024 (34 decks)

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