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PAMS: Orthopaedics and Rheumatology > deck_diffuse connective tissue disorders > Flashcards

deck_diffuse connective tissue disorders Flashcards

1
Q

What is SLE ?

A

It is an immune complex mediated Type 03 HSR manifests as autoimmune multisystem inflammatory disease with prominent clinical presentation of rashes and arthralgia as well as serious renal and cerebrovascular complications.

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2
Q

What is the epidemiology of SLE

A

It is nine times more common in woman than in men with a peak age of onset between 20 and 40. It is more prevalent in African/ Caribbean woman (1:250) followed by hispanics and asians.

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3
Q

what are the most common causes of death in Lupus ?

A

Renal disease (7.9x gen population) Infection (5x gen population)* Haematologic malignancies and lung CA (2.1x gen pop)* Cardiovascular disease (1.7x gen pop)

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4
Q

What is the etiology of SLE ?

A

The exact cause is unknown and genetic factors are being considered.

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5
Q

What is the pathophysiology of SLE ?

A

The UVA induced damage causes skin cell apoptosis. The intracellular remnants of the dead cells appear on the cell surface as small blebs of self antigens consist of DNA and histone. The genetic defects in cellular phagocytic mechanisms in SLE patients causes the development of antibodies against these antigens leading to the formation of antigen- antibody complexes ( Immune complexes) which circulates throughout the body and gets deposited in various tissues causing systemic symptoms of SLE.

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6
Q

What are the systemic pathophysiological effects of antigen -antibody complex ( immune complex) deposition on various tissues in SLE?

A

These immune complex depositions triggers local inflammation such as the ones seen in lupus nephritis. Pancytopenia due to deposition on RBCs, WBCs and Platelets. In addition compliment depletion occurs due to high demand on complement system to destroy immune complex deposited cells.

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7
Q

what type of hypersensitivity reaction is antigen- antibody complex formation which is the core of SLE pathohysiology?

A

The antigen- antibody complex formation is Type 2 HSR. The Antigen- antibody complex deposition mediated pathological process are Type-3 HSR.

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8
Q

What are the signs of SLE ?

A
  • Nonscarring AlopeciaMalar rash Discoid rash * Photosensitivity * oro-nasal mucosal lesions. * Serositis Myocarditis * Libman-Sacks endocarditis. Non-erosive symmetrical polyarthritis.
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9
Q

What are the non- treatment requiring SLE nephritis subtypes ?

A

Type 01 messangial and type 02 messangial proliferative SLE nephritis have no symptoms or laboratory abnormalities. Therefore, they don’t require treatments.

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10
Q

What are the aggressive treatment requiring lupus nephritis types ?

A

Focal proliferative (III) and diffuse proliferative (IV).

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11
Q

What are the lab findings in Type 3 and 4 lupus nephritis ?

A

HTN, protein urea, active sediments, + Anti-dsDNA antibody, elevated Cr and urea, Low C3/C4.

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12
Q

What are the findings in type 5 or membranous lupus nephritis ?

A

Heavy protein urea and bland sediments. It needs to be actively treated.

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13
Q

What is the prognosis of type VI ( advanced sclerosing Lupus Nephritis )?

A

ESRD

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14
Q

What are the neuropsychiatric effects of SLE ?

A

Headache Seizures* Psychotic disorders* Mood disorders* Stroke* Peripheral neuropathies

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15
Q

What are the hematological laboratory findings seen in SLE?

A
  • Anaemia of chornic disease due to Renal disease associated erythropientin response insufficiency. * Iron deficiency anaemia* Hemolytic anaemia * Leukopenia and thromobocytopeania* In many cases pancytopenia
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16
Q

What are the typical antibodies seen elevated in SLE ?

A
  • ANA Anti-dsDNA * Anti-smith Antibody * Anti-phospholipid antibodies- Anti-cardiolipin, lupus anti-coagulant, Anti-β 2 glycoprotein 1 antibodies. Low C3 and C4 * Anti-histone antibody in drug induced lupus.
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17
Q

What is the sensitivity and specificity of ANA in SLE ?

A

95% sensitivity, but low specificity.

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18
Q

What is the sensitivity and specificity of Anti-dsDNA and anti-smith antibodies in SLE ?

A

Low sensitivity but high specificity with active disease

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19
Q

Anti-histone antibody is specific to what type of lupus ?

A

drug induced lupus.

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20
Q

What is the diagnostic criteria for SLE ?

A

It is predominantly a clinical diagnosis based on the clinical presentation consistent with SLE which is not explained by other conditions and an ANA titer > 1:80.

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21
Q

What are the initial laboratory work-ups in SLE ?

A
  • FBC* Serum creatinine* UA with microscopy* ANA
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22
Q

What is the general management of SLE ?

A
  • Limit sun exposure and use high factor sunblock.* Adequate intake of calcium and vitamin D
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23
Q

What is the management of mild SLE that presents with mucocutaneous lesions and arthritis only?

A
  • NSAIDs
  • Hydroxychloroquine (PLAQUENIL®)
  • Hydroxychloroquine (PLAQUENIL®) + NSAIDs*Occasional short course of low dosesteroids (e.g., 5 mg prednisolone).
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24
Q

What is the management of moderate SLE that presents with symptoms of mild SLE and serositis without nephritis ?

A

Hydroxychloroquine + Oral steroids (short term).

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25
Q

What is the management of severe SLE that presents with symptoms of moderate SLE and nephritis with severe symptoms?

A

Immunosuppressant such as:▪ Cyclophosphamide ▪ Azathioprine ▪ Methotrexate▪ Mycophenolate ▪ Rituximab *RUXIENCE

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26
Q

What is the pathogenesis of drug induced lupus ?

A

Many drugs can induce the formation of Ab-AG immune complex which can lead to similar symptoms as SLE rash, arthritis, serositis with notable absence of blood, kidney and braininvolvement.

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27
Q

what is the management of drug induced lupus ?

A

stop drug, NSAIDs,topical steroids.

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28
Q

What are the laboratory evidence of drug induced lupus ?

A

+ANA and Anti-HistoneAntibodies.

29
Q

What is lupus-antiphospholipd syndrome ?

A

It is marked by antibodies against phospholipids in isolation or in combination with SLE with symptoms similar to both such as Coagulation defect, Livedo reticularis, Obstetric (recurrent miscarriages), Thrombocytopaenia.

30
Q

What is the Dx marker of Lupus – Antiphospholipid Syndrome?

A

persistent elevation in antiphospholipid antibodies

31
Q

What is the Tx of Lupus – Antiphospholipid Syndrome?

A

Aspirin for prophylaxis for asymptomatic patients and Warfarin for patients with stroke or renal infarcts.

32
Q

what is the epidemiology of systemic sclerosis (scleroderma) ?

A

It is a rare condition with female predominance.

33
Q

What is the pathophysiology of scleroderma ?

A

Genetic factors, viral infections such as CMV, parvovirus B-19, certain chemical exposure, and certain medications can trigger the formation of immune complexes similar to that of SLE. These immune complexes cause T cell mediated endothelial injuryand fibroblast activation causing vascular and skin fibrosis and tissue ischemia.

34
Q

What are the antibodies produced by the B cells in systemic sclerosis ?

A
  • Anti-SCL 70* Anti-RNA polymerase III (ARA)* Anti-Centromere (ACA)
35
Q

What are the dermatological manifestations of scleroderma ?

A

*Initially, distal extremities (hands) become doughy or swollen
* Eventually, skin becomes tight, shiny, smooth and stiff
* Ascension of effects from distal to proximal.
* Sclerodactyly and Calcinosis
* Ulcerations and Claw-shaped hand
* Raynaud Phenomenon

36
Q

What are the facial manifestations of scleroderma ?

A
  • Microstomia* Beaked nose* Telangiectasia
37
Q

What are the systemic manifestations of Scleroderma ?

A

GI: GERDPLum: Pulmonary hypertension and fibrosis.Renal: Scleroderma renal crisis

38
Q

What is the presentation of limited systemic sclerosis ?

A

It affects face, hand ,and feet with organ damage occurring later in the course of the disease. The Anti-centromere antibody is specific to limited scleroderma. CREST syndrome is classically seen in Limited scleroderma.

39
Q

What are the components of CREST syndrome ?

A
  • Calcinosis* Raynaud Phenomenon (usually 1st sx)* Esophageal Dysmotility* Sclerodactyly* Telangiectasia
40
Q

What is diffuse scleroderma ?

A

It affects arms, legs, trunk and face with organ damage occurring early on in the course of the disease. Anti-SCL 70 and Anti-RNA polymerase III (ARA) antibodies are specific to diffuse scleroderma.

41
Q

How to evaluate for organ damage in systemic sclerosis ?

A
  • Blood pressure – evidence of renal impairment and secondary HTN* Upper endoscopy – evidence of GORD* FBC – anaemia secondary to malabsorption d/t GI effects* Pulmonary function tests – evidence of restrictive airway disease
42
Q

What is the medical management of scleroderma ?

A
  • To delay disease progression immunosuppresents such as Methotrexate, Mycophenolate and Cyclophosphamide.* For GERD PPIs * For Raynaud phenomenon: CCB* For pain NSAIDs* For HTN ACE inhibitors.
43
Q

what is Sjogren Syndrome?

A

It is an autoimmune exocrine glandular disease with systemic involvement.The exact aetiology is not known. There is often genetic and family Hx present.

44
Q

What is the epidemiology of Sjogren Syndrome?

A

approximately 3% of the global population is affected with 0.5 to 1% of the Irish population affected. It is nine times more common in woman than in men with a mean onset age range of 40 to 60.

45
Q

What is the triad of Sjogren Syndrome?

A

Keratoconjuntivtis sica, Xerostomia and RA.

46
Q

Sjogren Syndrome without arthritis is called ?

A

Sicca syndrome.

47
Q

What percentage of RA patients have Sjogren Syndrome?

A

20 to 50%

48
Q

What percentage of SLE patients have Sjogren Syndrome?

A

10 to 25%

49
Q

What are the anti-bodies specific to sjogren’s syndrome ?

A

Anti-SSA (Ro) and Anti-SSB (La) in gland biopsy and blood.

50
Q

What is the pathophysiology of Sjogren’s disease ?

A

It is a lymphocytes mediated autoimmune disease in which Anti-SSA(Ro) and Anti-SSB (La) antibodies produced by the B lymphocytes destroy Minor salivary glands, Lacrimal glands and joints. In addition there is also T cell mediated type 4HSR occurs in the pathogenesis of Sjogren’s disease. These dual antibody and T-cell mediated immune response together causes exocrine glandular fibrosis and impaired secretory function.

51
Q

What are the exraglandular manifestations of Sjogren’s disease ?

A

Systemic symptoms: Fever, fatigue, Myalgia and weight lossCNS: Depression, NeuropathyCVS: pericarditis and CVDblood: Anemia, leukopenia, Hypogamaglobulinemia, and monoclonal gamopathy, MALT lymphoma. GI: dysphagia, gastritis, celiac disease.Skin: Xerosis cutis, Raynud’s and purpuras. Plum: RLD, ILD, and NSIPendocrine glands: thyroiditis.Joints: Arthralgia +/- Inflammatory arthritis.

52
Q

What is the clinical presentation of Sjogren’s syndrome?

A

The most common presentation is Daily symptoms of dry mouth or dry eyes x 3 months with or without arthralgia

53
Q

What are the methods to examine salivary gland function?

A
  • Quantitative salivary gland scintigraphy* Whole sialometry – measures amount of salivaproduced over a given time period* Ultrasound or MRI to look for gland enlargement* Lip salivary gland biopsy
54
Q

What are the methods to examine lacrimal gland function?

A

Schirmer test – measures amount of tear production. * Ocular surface staining – look for ulceration of cornea and conjunctiva. Tear break-up time.

55
Q

What are the laboratory findings in Sjogren’s syndrome ?

A
  • decreased WBC, increased globulins and ESR with normal CRP. * Autoantibodies are ANA, RF, Anti-SSA (Ro) and Anti-SSB (La).
56
Q

what is the confirmatory diagnostic requirement in Sjogren’s disease ?

A

+ Anti SSA (Ro) or Anti-SSB (La) and Lips salivary gland biopsy showing lymphocytic infiltration and fibrosis of the gland.

57
Q

What is the Tx of mild Sjogren’s that presents with secretory dysfunctions only ?

A

Pilocarpine and artificial tears.

58
Q

What is the Tx of moderate to severe Sjogren’s that presents with major salivary gland enlargement and systemic organ involvements?

A

treat the glandularmegaly with PO steroids. For systemic organ involvement DMARDs such as Hydroxychlroquine or methotrexate.

59
Q

What is the treatment of severe Sjogren’s disease ?

A

Biologic DMARDs such as Rithuximab or cyclophosphamide.

60
Q

What are the main types of idiopathic inflammatory myopathies ?

A
  • Polymyositis* Dermatomyositis* Inclusion body myositis* Necrotizing autoimmune myopathy
61
Q

What is the pathophysiology of Polymyositis & Dermatomyositis?

A

In Polymyositis & Dermatomyositis, the CD8+ T lymphocytes detects the normal MHC class-01 self antigens of the normal cells as autoantignes and binds to it causing apoptosis. In addition, the B cells also prouces antiboids againts these autoantigens such as t-RNA synthateses (JO-01), MI2 nuclear protien, and SRP. These immune responses causes muscle inflmmation and elevation in msucle enzymes such as CK and aldolace which manifest as sysmptoms of PM and DM.

62
Q

What are the antibodies in Polymyositis & Dermatomyositis?

A
  • tRNA synthetase (Jo-1)* MI-2 nuclear protein* Signal Recognition Particle (SRP)
63
Q

What is the clinical presentation of Polymyositis ?

A

Proximal muscle weakness andwasting as well as tenderness to palpation. Pharyngeal muscles may be involved which may present as dysphonea and dysphagia.

64
Q

What is the clinical presentation of Dermatomyositis?

A
  • Same muscle findings as PM* Skin manifestations* Heliotrope rash* Malar rash* Shawl sign* V sign* Gottron’s papules
65
Q

What are the laboratory findings in PM ?

A

Elevated muscle enzymes such as CK and Aldolase. * Elevated ESR and CRP Anti SRP and Anti- Jo-1 antibodies.* EMG consistent with myopathy *(CD8+) lymphocyticinfiltration of the endomysium.

66
Q

What are the laboratory findings in DM?

A

Elevated muscle enzymes such as CK and Aldolase. * Elevated ESR and CRP EMG consistent with myopathyAnti- Jo-1 and Anti-Mi 2. *(CD4+) lymphocyticinfiltration of the perimysium.

67
Q

What is the Tx of PM ?

A
  • High-dose steroids (up to 80mg prednisolone per day) fortapering down after one year* Immunosuppressants* Methotrexate* Azathioprine* Mycophenolate
68
Q

What is the Tx of DM ?

A
  • Same as PM with addition ofthe following for skinmanifestations* Avoid sun* Protective clothing* Hydroxychloroquine for skin lesions

PAMS: Orthopaedics and Rheumatology (17 decks)

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