Medicine Marrow Pg No 467-476 (Rheumatology)
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Questions and Answers

Which of the following features is NOT associated with Limited SSc?

  • Calcinosis cutis
  • Sclerodactyly
  • Raynaud's phenomenon
  • Extensive fibrosis (correct)

    • Pulmonary artery hypertension associated with SSc has a poor prognosis due to increased mortality rates.

    True

    What skin manifestation is characterized by a 'salt and pepper' appearance in Diffuse SSc?

    Hypo and hyperpigmentation

    In patients with Diffuse SSc, _______ is typically decreased during pulmonary function tests.

    <p>DLCO</p> Signup and view all the answers

    Match the following features with their respective types of systemic sclerosis:

    <p>Type I = Early/Sine SSc (No skin changes) Type II = Not specified in the provided content Type III = Diffuse SSc with skin and organ involvement Pulmonary function test results = Normal FEV1, FVC; Decreased DLCO</p> Signup and view all the answers

    Which manifestation is characteristic of diffuse systemic sclerosis (SSC)?

    <p>Involvement of all parts of the body</p> Signup and view all the answers

    Systemic sclerosis generally has a good prognosis.

    <p>False</p> Signup and view all the answers

    What is the role of TGF-β in the pathogenesis of systemic sclerosis?

    <p>It promotes the transition of mesenchymal cells to myofibroblasts and contributes to fibrosis.</p> Signup and view all the answers

    In systemic sclerosis, capillary hypoxia leads to the release of ________, which is responsible for pulmonary arteriole hypertension.

    <p>TGF-β</p> Signup and view all the answers

    Match the following conditions with their respective characteristics:

    <p>Localized scleroderma = Involves only specific areas of the skin Eosinophilic fasciitis = Presents with groove sign Nephrogenic systemic fibrosis = Occurs due to gadolinium contrast exposure Vasculopathy in SSC = Leads to small vessel endothelial injury</p> Signup and view all the answers

    What is the primary treatment for renal crisis associated with scleroderma?

    <p>ACE inhibitors</p> Signup and view all the answers

    Renal crisis in scleroderma occurs in about 25% of patients.

    <p>False</p> Signup and view all the answers

    What two conditions are associated with thrombotic microangiopathy in scleroderma renal crisis?

    <p>Accelerated hypertension and renal failure</p> Signup and view all the answers

    The ____ phase of diffuse scleroderma is characterized by non-pitting edema.

    <p>edematous</p> Signup and view all the answers

    Match the following investigative findings to their corresponding conditions in scleroderma:

    <p>PFT showing DLCO ↓ = Interstitial lung disease (ILD) X-ray showing B/L basal subpleural reticular infiltrates = Pulmonary fibrosis Salt &amp; pepper appearance = Skin changes in scleroderma Thrombocytopenia = Renal crisis</p> Signup and view all the answers

    What is the primary age range associated with systemic sclerosis?

    <p>35 - 50 yrs</p> Signup and view all the answers

    Raynaud's Phenomenon is a late symptom of systemic sclerosis.

    <p>False</p> Signup and view all the answers

    Name one drug that can induce scleroderma.

    <p>Bleomycin</p> Signup and view all the answers

    The immune susceptibility marker associated with systemic sclerosis is ______.

    <p>HLA DR B1</p> Signup and view all the answers

    Match the HLA with the respective associated disease:

    <p>DR-3 = SLE, Sjogren's Syndrome DR-4 = Antiphospholipid Syndrome (APS), RA DR-5 = Systemic Sclerosis Silica = Also associated with SLE</p> Signup and view all the answers

    Which treatment is considered first-line for systemic sclerosis?

    <p>Calcium channel blocker</p> Signup and view all the answers

    Nonspecific Interstitial Pneumonia (NSIP) is associated with lung parenchyma destruction.

    <p>False</p> Signup and view all the answers

    What is the main distinguishing feature of Usual Interstitial Pneumonia (UIP) compared to NSIP?

    <p>Destruction of lung parenchyma</p> Signup and view all the answers

    In the treatment of interstitial lung disease associated with scleroderma, Nintedanib is combined with _____ for better results.

    <p>mycophenolate mofetil (mmF)</p> Signup and view all the answers

    Match the following treatments to their conditions:

    <p>Calcium channel blocker = Systemic Sclerosis Bosentan = Pulmonary arterial hypertension ACE inhibitors = Scleroderma renal crisis Steroids + methotrexate = Joints + muscle involvement</p> Signup and view all the answers

    What is a requirement for a diagnosis of Antiphospholipid Syndrome relating to pregnancy?

    <p>One miscarriage after the 10th week</p> Signup and view all the answers

    Experiencing three miscarriages before the 10th week qualifies as a criterion for Antiphospholipid Syndrome.

    <p>True</p> Signup and view all the answers

    Name a vascular event that is associated with Antiphospholipid Syndrome.

    <p>Venous or arterial thrombosis</p> Signup and view all the answers

    Antiphospholipid Syndrome can manifest during pregnancy as ________ delivery.

    <p>premature</p> Signup and view all the answers

    Match the criteria for Antiphospholipid Syndrome with their descriptions:

    <p>One miscarriage ≥ 10 weeks = A valid criterion for diagnosis ≥ 3 miscarriages before 10 weeks = A valid criterion for diagnosis Premature delivery = A valid criterion for diagnosis Venous/arterial thrombosis = A vascular event associated with APS</p> Signup and view all the answers

    Which antibody is associated with Diffuse SSc?

    <p>Both B and C</p> Signup and view all the answers

    Self-limiting and symmetric symptoms are characteristic of Limited SSc.

    <p>False</p> Signup and view all the answers

    What is the primary investigation used for Diffuse SSc?

    <p>Nail fold capillaroscopy</p> Signup and view all the answers

    The ANA pattern associated with Limited SSc is __________.

    <p>Centromere</p> Signup and view all the answers

    Match the following features with their respective type of SSc:

    <p>Good prognosis = Diffuse SSc Long standing and severe = Limited SSc Short lasting Raynaud's phenomenon = Diffuse SSc Critical limb ischemia = Limited SSc</p> Signup and view all the answers

    Which of the following complications are more likely associated with Limited SSc?

    <p>Critical limb ischemia</p> Signup and view all the answers

    Raynaud's phenomenon in Diffuse SSc is typically long lasting and severe.

    <p>False</p> Signup and view all the answers

    What symptom is often SAW in Limited SSc?

    <p>Severe limb ischemia</p> Signup and view all the answers

    What is the most common cause of death in Systemic Sclerosis (SSc)?

    <p>Pulmonary manifestations</p> Signup and view all the answers

    Anti-centromere antibodies are associated with the limited type of systemic sclerosis.

    <p>True</p> Signup and view all the answers

    What is a significant feature of the diffuse type of systemic sclerosis regarding skin involvement?

    <p>Rapidly progressive skin involvement</p> Signup and view all the answers

    In patients with systemic sclerosis, severe __________ phenomenon is common in limited types.

    <p>Raynaud's</p> Signup and view all the answers

    Match the antibodies with their respective types of systemic sclerosis:

    <p>Anti-centromere = Limited SSc Antitopoisomerase-1 (Anti Scl-70) = Diffuse SSc Anti-RNA polymerase-3 = Diffuse SSc Anti-Pm/Scl-70 = Overlap</p> Signup and view all the answers

    Which of the following is a manifestation associated with small intestinal bacterial overgrowth?

    <p>Esophageal hypomotility</p> Signup and view all the answers

    Cardiac manifestations in systemic sclerosis can include restricted cardiomyopathy.

    <p>True</p> Signup and view all the answers

    What is a common digestive issue in systemic sclerosis related to fibrosis?

    <p>Esophageal hypomotility</p> Signup and view all the answers

    The hallmark skin change in diffuse systemic sclerosis includes __________.

    <p>tendon friction rubs</p> Signup and view all the answers

    Which antibody is associated with a higher risk of malignancy in systemic sclerosis?

    <p>Anti-RNA polymerase-3</p> Signup and view all the answers

    Study Notes

    Limited SSc

    • Limited SSc is characterized by minimal fibrosis, featuring CREST syndrome, with Calcinosis cutis, Raynaud's phenomenon, Esophagitis, Sclerodactyly, and Telangiectasia.
    • Extensive fibrosis and ILD indicate against Limited SSc.

    Diffuse SSc

    • Diffuse Systemic Sclerosis (SSc) involves significant inflammation and fibrosis.
    • Skin manifestations include hypo and hyperpigmentation (salt and pepper appearance), face tightening leading to microstomia, pursed lips, puckered mouth, and mask facies.
    • Bilateral symmetrical skin thickening is a characteristic feature.
    • Early/Sine SSc (Type I) lacks skin changes while Type II and Type III Diffuse SSc further classify different stages.
    • Pulmonary function tests (PFT) reveal normal FEV1, FVC, and FEV1/FVC ratios but a decreased DLCO.
    • Cardiac and renal involvement are common, with a dry, tight skin due to loss of body oil.
    • Hair loss and intense itching are associated with hyperpigmentation.
    • Pulmonary artery hypertension carries a poor prognosis due to increased mortality, which can be asymptomatic.
    • Images showcase various body parts with shaded areas and facial expressions consistent with SSc features, illustrating different clinical subtypes and their aspects.

    Systemic Sclerosis

    • Systemic Sclerosis (SSc) is characterized by inflammation (++) and fibrosis (+), while vascular involvement involves vasculopathy or small vessel endothelial injury due to anti-endothelial antibodies.

    Etiology of SSc

    • An orphan disease with unknown causes, affecting individuals between 35 to 50 years of age with a female to male ratio of 5:1.
    • HLA DR B1, II (DR5) suggests immune susceptibility.
    • Viruses like EBV, CMV, and Parvovirus B19 are implicated.
    • Drug-induced scleroderma can occur due to vinyl chloride, bleomycin, pentazocine, contaminated L-tryptophan, and silica, with the strongest association with silica.

    Very Early Systemic Sclerosis (VESS)

    • VESS is defined as a stage of systemic sclerosis lasting 3-5 years.
    • Two-thirds of these cases eventually evolve into SSc.

    Raynaud's Phenomenon

    • A key early symptom of systemic sclerosis.
    • Triggered by cold, stress, or vibration, it manifests in three phases: pallor, cyanosis, and redness on rewarming.
    • Pain and tightness of digits, along with puffy hands, are additional symptoms.
    • Nail fold capillaroscopy is used for investigation.

    HLA Associations with Diseases

    • HLA DR-3 is associated with SLE and Sjogren's Syndrome.
    • HLA DR-4 is linked with Antiphospholipid Syndrome (APS) and RA.
    • HLA DR-5 is associated with SSc.
    • Silica is also linked with SLE.

    Joint Manifestations in Diffuse and Limited SSc

    • Diffuse SSc*

    • Arthritis and myositis are common, caused by inflammation.

    • Limited SSc*

    • Acral osteolysis, where fingers fall off, is a prominent feature.

    • Notably characterized by severe and diffuse Raynaud's phenomenon.

    Other Manifestations of Systemic Sclerosis

    • Esophageal hypomotility due to fibrosis or vasculopathy.
    • Small intestinal bacterial overgrowth and pneumatosis intestinalis are potential complications.

    Disease Progression of Systemic Sclerosis

    • Pulmonary manifestations, particularly Interstitial lung disease (ILD), are the leading cause of death.
    • Cardiac and renal manifestations are additional concerns.

    Antibodies and Types of Systemic Sclerosis

    • Anti-centromere antibody is associated with Limited SSc, featuring CREST syndrome, pulmonary hypertension, restricted cardiomyopathy, thrombotic microangiopathy, and scleroderma renal crisis.
    • Antitopoisomerase-1 (Anti Scl-70) is linked to Diffuse SSc with similar features, including pulmonary hypertension, restricted cardiomyopathy, thrombotic microangiopathy, and scleroderma renal crisis.
    • Anti-RNA polymerase-3 is also tied to Diffuse SSc, manifesting as tendon friction rubs, gastric antral vascular ectasia (GAVE), rapidly progressive skin involvement, joint contractures, tendon friction rubs, scleroderma renal crisis (more common with Anti RNA polymerase 3 than Anti-topoisomerase), and malignancy in the lung or breast.
    • Anti-Pm/Scl-70 and Anti-Ku are associated with overlap syndromes.

    Scleroderma: NSIP > UIP

    • Two types of interstitial lung disease (ILD) associated with scleroderma:
      • NSIP (Nonspecific Interstitial Pneumonia): No lung parenchyma destruction, Ground Glass Opacities, responds to steroids and mycophenolate mofetil, leading to a better prognosis.
      • UIP (Usual Interstitial Pneumonia): Lung parenchyma destruction, Honey combing, cystic changes, traction bronchiectasis, no effective treatment.

    Treatment of Systemic Sclerosis (SSc)

    • First-line treatment: Calcium channel blocker (CCB) +/- phosphodiesterase (PDE)-5 inhibitors (e.g., sildenafil).
    • Second-line therapy: Bosentan, an endothelin antagonist.
    • Skin involvement: Mycophenolate mofetil (mmF).
    • Interstitial Lung Disease (ILD): Nintedanib + mmF for non-responsive cases.
    • Joints and muscle involvement: Steroids + methotrexate.
    • Scleroderma Renal Crisis: ACE inhibitors.

    Diffuse vs Limited SSc

    • Diffuse SSc: Fine speckled ANA pattern, Antitopoisomerase and Anti-RNA PIII antibodies, self-limiting and symmetrical symptoms, good prognosis, normal nailfold capillaroscopy.
    • Limited SSc: Centromere ANA pattern, Anti-centromere antibody, severe, life-threatening symptoms with critical limb ischemia, ulcers, and gangrene, long-standing and severe, predominant vasculopathy, positive for critical limb ischemia, abnormal nailfold capillaroscopy.
    • Note:* This table provides a detailed comparison between Diffuse and Limited SSc, highlighting key differences in their characteristics.

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    Description

    Test your understanding of Limited and Diffuse Systemic Sclerosis (SSc) in this informative quiz. Explore the characteristics, symptoms, and classifications of SSc, including the unique features of CREST syndrome and the stages of Diffuse SSc. Perfect for medical students and anyone interested in autoimmune disorders!

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