Medicine Marrow Pg No 507-516 (Rheumatology)
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Questions and Answers

What is a major criterion for diagnosing Behcet's Disease?

  • Recurrent genital ulceration
  • Eye lesions
  • Positive pathergy test
  • Recurrent oral ulceration (correct)

    • Behcet's Disease spares the kidney.

    True

    What is the most affected age group for Behcet's Disease?

    30-50 years

    In Behcet's Disease, the presence of at least one major criterion and one minor criterion is required for __________.

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

    Match the following manifestations with their corresponding criteria:

    <p>Recurrent oral ulceration = Major criterion Genital ulceration = Minor criterion Eye lesions = Minor criterion Positive pathergy test = Minor criterion</p> Signup and view all the answers

    What type of inflammation is associated with Behcet's disease?

    <p>Non necrotizing inflammation</p> Signup and view all the answers

    Granulomas are present in Behcet's disease.

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

    Name one HLA associated with Behcet's disease.

    <p>HLA-B</p> Signup and view all the answers

    Behcet's disease is classified under ______ vasculitis.

    <p>large vessel</p> Signup and view all the answers

    Match the following features to either Takayasu arteritis or Behcet's disease:

    <p>Panarteritis = Behcet's disease Granulomas = Takayasu arteritis Panniculitis = Both Takayasu arteritis and Behcet's disease Obliterative phlebitis = Behcet's disease</p> Signup and view all the answers

    What percentage of patients with Henoch-Schonlein Purpura present with joint problems?

    <p>70%</p> Signup and view all the answers

    Patients with Henoch-Schonlein Purpura have a high risk of Chronic Kidney Disease.

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

    What are the predominant joint symptoms in Henoch-Schonlein Purpura?

    <p>Knee joint pain</p> Signup and view all the answers

    In Henoch-Schonlein Purpura, 50% of patients experience abdominal angina and __________ pain.

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

    Match the following complications with Henoch-Schonlein Purpura.

    <p>Abdominal angina = Colicky pain Intussusception = Ileoileal (most dangerous) Bowel ischemia = Hemorrhage (Bleeding PR) Scrotal swelling = Complication of GIT</p> Signup and view all the answers

    Which of the following is a symptom included in the Meltzer triad of Cryoglobulinemia?

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

    Cryoglobulinemia includes mononeuritis multiplex as a potential neurological complication.

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

    What is the treatment for Type 2 and Type 3 Cryoglobulinemia if HCV is negative?

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

    The presence of ____ cryoglobulins indicates a ↓C3/C4 in the serum.

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

    Match the following treatments with their corresponding types for Cryoglobulinemia:

    <p>PLEX = Type 1 (High IgM) Rituximab = Type 2/3 with negative HCV Cyclophosphamide = Type 1 (High IgM) Treatment for HCV = Type 2/3 with positive HCV</p> Signup and view all the answers

    Which of the following is a characteristic of Immune Complex Mediated Vasculitis?

    <p>Present necrotizing inflammation</p> Signup and view all the answers

    Henoch-Schonlein Purpura is classified as a secondary vasculitis.

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

    Name one condition classified under ANCA vasculitis.

    <p>Any of the following: Granulomatosis with polyangiitis, Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis.</p> Signup and view all the answers

    Cryoglobulinemia Associated vasculitis is a type of ______ vasculitis.

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

    Match the following conditions with their classification:

    <p>Henoch-Schonlein Purpura = Primary vasculitis Rheumatoid Arthritis = Secondary vasculitis Systemic Lupus Erythematosus = Secondary vasculitis Cutaneous Leukocytoclastic Angiitis = Single organ vasculitis</p> Signup and view all the answers

    Which of the following conditions is most likely to present with pulmonary infiltrates or nodules?

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

    Microscopic Polyangiitis is associated with a higher prevalence of glomerulonephritis than Churg-Strauss Syndrome.

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

    Which ANCA-associated condition is characterized by peripheral nervous system involvement rated as '++'?

    <p>Microscopic Polyangiitis</p> Signup and view all the answers

    In the differential diagnosis table, Churg-Strauss Syndrome shows a high tendency for __________ disease.

    <p>Upper airway</p> Signup and view all the answers

    Match the ANCA-associated vasculitides to their respective glomerulonephritis ratings:

    <p>Wegener's Granulomatosis = +++ Glomerulonephritis Microscopic Polyangiitis = +++ Glomerulonephritis Polyarteritis Nodosa = - Glomerulonephritis Churg-Strauss Syndrome = ++ Glomerulonephritis</p> Signup and view all the answers

    Which feature has a rating of '-' for Polyarteritis Nodosa?

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

    Skin purpura is commonly associated with Churg-Strauss Syndrome.

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

    What does the abbreviation 'CSS' most likely represent in this context?

    <p>Churg-Strauss Syndrome</p> Signup and view all the answers

    Which of the following is a danger sign of small vessel vasculitis?

    <p>CNS vasculitis</p> Signup and view all the answers

    Weight loss greater than 4kg is a criterion for diagnosing Polyarteritis Nodosa (PAN).

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

    What is the first investigation recommended for diagnosing small vessel vasculitis?

    <p>MR Angiogram</p> Signup and view all the answers

    In cutaneous PAN, venules are ______.

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

    Match the symptoms to their respective classifications:

    <p>DAH = Signs against diagnosis of PAN RPGN = Symptoms against diagnosis of PAN Testicular pain = ACR criterion for PAN Livedo reticularis = ACR criterion for PAN</p> Signup and view all the answers

    Which of the following is considered a pathognomonic finding for the diagnosis of oral aphthous ulcers?

    <p>Recurrent painful ulcers</p> Signup and view all the answers

    Genital ulcers in Behcet's disease are typically more recurrent than oral ulcers.

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

    Name one common site where oral aphthous ulcers are found.

    <p>Lips, buccal mucosa, tongue, or soft palate</p> Signup and view all the answers

    A positive pathergy test indicates hypersensitivity to ______.

    <p>long scratch or intradermal saline injection</p> Signup and view all the answers

    Match the following skin lesions with their characteristics:

    <p>Erythema nodosum = Painful, pretibial pigmented papule Pyoderma gangrenosum = Rapidly progressive ulcerative lesion Pseudofolliculitis = Inflammatory reaction resembling infected hair follicles Papulopustular lesions = Common type of skin lesion in Behcet's disease</p> Signup and view all the answers

    What type of immunoglobulins are involved in cryoglobulinemia classification Type a?

    <p>Polyclonal IgG</p> Signup and view all the answers

    What is a characteristic skin lesion associated with Henoch-Schönlein Purpura?

    <p>Cutaneous palpable purpura</p> Signup and view all the answers

    Cryoglobulinemia primarily affects young males.

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

    Henoch-Schönlein Purpura only affects children under the age of 5.

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

    Name one associated disease of Type 1 cryoglobulinemia.

    <p>Multiple myeloma</p> Signup and view all the answers

    In which anatomical area is the purpura most commonly distributed in Henoch-Schönlein Purpura?

    <p>Extensor aspect of the lower limb, especially the buttocks.</p> Signup and view all the answers

    Cryoglobulinemia can lead to _______ due to medium vessel involvement.

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

    Henoch-Schönlein Purpura is characterized by positive immunofluorescence and a ______________ inflammatory infiltrate in small vessels.

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

    Match the following clinical manifestations of cryoglobulinemia with their relevant systems:

    <p>Non-thrombocytopenic palpable purpura = Skin Arthralgia = Joints MPGN = Kidneys HCV involvement = Gastrointestinal Tract</p> Signup and view all the answers

    Match the following characteristic features of Henoch-Schönlein Purpura with their descriptions:

    <p>Non-thrombocytopenic palpable purpura = Universal manifestation in patients Age group = Primarily affects children, but can affect adults Neutrophilic segmental inflammation = Type of inflammation observed in the dermis Crops pattern = Lesions appear in clusters</p> Signup and view all the answers

    Study Notes

    Behcet's Disease

    • T-cell mediated inflammation predominates, with Th1 cells playing a greater role than Th17 cells.
    • Behcet's disease is a chronic, relapsing, multisystem autoimmune disease.
    • The most affected age group is 30-50 years old, with severe disease more common in young males.
    • The kidneys are typically spared.

    Clinical Manifestations

    • Sites of involvement include the oral cavity, eyes, genitals, vasculature, joints, gastrointestinal tract, nervous system, lungs, and heart (rare).
    • Pulmonary artery vasculitis/aneurysm is a feature of Takayasu arteritis, not Behcet's disease.

    Diagnostic Criteria

    • Diagnosis is based on the International Study Group for Behcet's Disease (ISG) criteria or the International Criteria for Behcet's Disease.
    • ISG Criteria: At least one major criterion and one minor criterion, or a score of 24 or more.
      • Major Criterion: Recurrent oral ulceration (at least 3 times in 12 months).
      • Minor Criteria: Recurrent genital ulceration, eye lesions (anterior or posterior uveitis, retinal vasculitis), skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform nodules in post-adolescent patients), and a positive pathergy test.
    • International Criteria for Behcet's Disease: A score of 24 or more.
      • Oral Aphthosis, Genital Aphthosis, and Ocular Lesions: Each receives one point.
      • Skin lesions, Neurological Manifestations, Vascular manifestations, and Positive pathergy test: Each receives one point.

    Behcet's vs Takayasu Arteritis

    Feature Takayasu arteritis Behcet's disease
    Characteristic features Panarteritis Panarteritis
    Predominant cell Lymphocyte
    Granulomas Present Absent
    Neutrophil Absent Absent
    Inflammation Non-necrotizing inflammation Non-necrotizing inflammation
    Other features Panniculitis Panniculitis; Obliterative phlebitis

    Arteritis with Granuloma

    • Large vessel vasculitis:
      • Temporal arteritis
      • Takayasu arteritis
    • EGPA
    • Rheumatoid vasculitis
    • Cogan's syndrome

    Arteritis without Granuloma

    • Behcet's disease
    • MPA

    Risk Factors

    • HLA associations:
      • HLA-B (Fragmented product of 85)
      • HLA-A26, HLA-B27, HLA-B57

    Joint Involvement

    • 70% of patients present with joint problems.
    • 15% of patients initially present with joint symptoms.
    • Features include:
      • Predominant knee joint involvement
      • Large joint involvement
      • Migratory pattern
      • Non-deforming arthritis
      • Oligoarthritis
    • Difficulty walking due to pain.
    • Differential diagnosis: Acute rheumatic fever arthritis.

    Gastrointestinal Tract

    • 50% of patients experience abdominal angina and colicky pain.
    • Complications include:
      • Hemorrhage (bleeding per rectum)
      • Bowel ischemia
      • Scrotal swelling
      • Intussusception: Ileoileal (most dangerous).

    Renal Involvement

    • 20-50% of patients experience renal issues.
    • Asymptomatic microhematuria is common.
    • No risk of Chronic Kidney Disease (CKD).
    • In adults:
      • Type 2 RPGN (Rapidly progressive glomerulonephritis)
      • Risk of End-Stage Renal Disease (ESRD)
      • High relapse rate

    Oral Aphthous Ulcers

    • Mandatory for diagnosis: Recurrent (at least 3 times per year) painful ulcers.
    • Common sites include the lips, buccal mucosa, tongue, and soft palate.
    • Erythematous papules/pustules develop into shallow necrotic ulcers that heal without scarring.

    Painless Ulcers Over Hard Palate

    • Consider a differential diagnosis of SLE or Dermatomyositis:
      • SLE can involve the oral cavity, while Dermatomyositis does not.
      • Dermatomyositis can cause a painful rash, whereas SLE has a painless rash.

    Genital Ulcers

    • More deeply situated and thus more painful than oral ulcers.
    • Less recurrent than oral ulcers.
    • Heals with scarring.
    • Spares the glans penis and urethra.
    • Most common sites: Scrotum and labia.
    • Extragenital involvement is rare, with the buttocks being the most common site.

    Positive Pathergy Test

    • Hypersensitivity to long scratches or intradermal saline injections.
    • Performed by oblique skin puncture with a 25-gauge needle.
    • Papule/pustule formation occurs after 24-48 hours.
    • Low sensitivity (60%) and high specificity (80%).

    Cutaneous Manifestations

    • All types of skin lesions are seen, including:
      • Erythema nodosum (painful, pretibial, pigmented papules)
      • Pyoderma gangrenosum
      • Pseudofolliculitis
    • Papulopustular lesions are the most common.
    • Site of lesions can vary.

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    Description

    Explore the complexities of Behcet's disease, a chronic autoimmune condition characterized by T-cell mediated inflammation. This quiz covers its clinical manifestations, affected demographics, and diagnostic criteria based on international standards. Test your knowledge on this multisystem disorder.

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