Podcast
Questions and Answers
Which autoimmune disease is characterized by rough and cracked lateral and palmar areas of the fingers?
Which autoimmune disease is characterized by rough and cracked lateral and palmar areas of the fingers?
What is a common systemic symptom associated with dermatomyositis?
What is a common systemic symptom associated with dermatomyositis?
Which antibody is specifically associated with dermatomyositis and presents with Gottron’s papules and heliotrope rash?
Which antibody is specifically associated with dermatomyositis and presents with Gottron’s papules and heliotrope rash?
What is a distinguishing feature between dermatomyositis and polymyositis?
What is a distinguishing feature between dermatomyositis and polymyositis?
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Which characteristic of dermatomyositis is associated with anti-histidyl-tRNA-synthetase antibodies?
Which characteristic of dermatomyositis is associated with anti-histidyl-tRNA-synthetase antibodies?
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In dermatomyositis, where is the deposition of C5b-9 (MAC) commonly found?
In dermatomyositis, where is the deposition of C5b-9 (MAC) commonly found?
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What is the characteristic microscopic pathology of Henoch-Schonlein Purpura?
What is the characteristic microscopic pathology of Henoch-Schonlein Purpura?
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Which symptom is NOT typically associated with Henoch-Schonlein Purpura?
Which symptom is NOT typically associated with Henoch-Schonlein Purpura?
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What is the common age group affected by Henoch-Schonlein Purpura?
What is the common age group affected by Henoch-Schonlein Purpura?
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Which antibody is typically elevated in p-ANCA (MPO-ANCA) associated vasculitides?
Which antibody is typically elevated in p-ANCA (MPO-ANCA) associated vasculitides?
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What type of vasculitis is characterized by skin lesions progressing from blanching macules to petechiae to palpable purpura in the buttocks and legs?
What type of vasculitis is characterized by skin lesions progressing from blanching macules to petechiae to palpable purpura in the buttocks and legs?
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What is the usual course of Henoch-Schonlein Purpura in terms of resolution?
What is the usual course of Henoch-Schonlein Purpura in terms of resolution?
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What is the primary cause of systemic vasculitides?
What is the primary cause of systemic vasculitides?
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Which of the following is a general symptom of systemic vasculitides resulting from the inflammatory response?
Which of the following is a general symptom of systemic vasculitides resulting from the inflammatory response?
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What is the primary complication of giant cell arteritis (GCA) if left untreated?
What is the primary complication of giant cell arteritis (GCA) if left untreated?
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What is the recommended therapy for giant cell arteritis (GCA)?
What is the recommended therapy for giant cell arteritis (GCA)?
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Which of the following is a characteristic finding in the histopathology of giant cell arteritis (GCA)?
Which of the following is a characteristic finding in the histopathology of giant cell arteritis (GCA)?
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What is the primary mechanism by which systemic vasculitides lead to organ damage?
What is the primary mechanism by which systemic vasculitides lead to organ damage?
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Which of the following is a characteristic of Polymyalgia Rheumatica (PMR)?
Which of the following is a characteristic of Polymyalgia Rheumatica (PMR)?
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What is the prevalence of Polymyalgia Rheumatica (PMR) in people over 50 years old?
What is the prevalence of Polymyalgia Rheumatica (PMR) in people over 50 years old?
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Which of the following is a characteristic of Takayasu Arteritis?
Which of the following is a characteristic of Takayasu Arteritis?
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Which of the following is a potential complication of Takayasu Arteritis?
Which of the following is a potential complication of Takayasu Arteritis?
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What is the characteristic histopathological finding in Polymyalgia Rheumatica (PMR)?
What is the characteristic histopathological finding in Polymyalgia Rheumatica (PMR)?
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Which of the following is a characteristic of Polyarteritis Nodosa?
Which of the following is a characteristic of Polyarteritis Nodosa?
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Which disease is characterized by fibrinoid necrosis of the arterial wall and thrombotic occlusion of the lumen?
Which disease is characterized by fibrinoid necrosis of the arterial wall and thrombotic occlusion of the lumen?
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What is the hallmark histological feature seen in Buerger's Disease?
What is the hallmark histological feature seen in Buerger's Disease?
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Which condition is NOT associated with ANCA antibodies?
Which condition is NOT associated with ANCA antibodies?
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Which disease predominantly affects young heavy smokers and may co-exist with Raynaud's phenomenon?
Which disease predominantly affects young heavy smokers and may co-exist with Raynaud's phenomenon?
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Which disease is characterized by the classical triad of kidney involvement, nasopharyngeal ulcers, and lung nodular densities?
Which disease is characterized by the classical triad of kidney involvement, nasopharyngeal ulcers, and lung nodular densities?
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Which condition primarily affects vessels of the respiratory tract and is strongly associated with asthma and eosinophilia?
Which condition primarily affects vessels of the respiratory tract and is strongly associated with asthma and eosinophilia?
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What is the classic symptom seen in Kawasaki Disease that spares the limbus?
What is the classic symptom seen in Kawasaki Disease that spares the limbus?
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Which disease results in aneurysm and myocardial infarction if left untreated?
Which disease results in aneurysm and myocardial infarction if left untreated?
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What is the main histological finding in Microscopic Polyangiitis?
What is the main histological finding in Microscopic Polyangiitis?
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What differentiates Churg-Strauss Syndrome from other small vessel vasculitides?
What differentiates Churg-Strauss Syndrome from other small vessel vasculitides?
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Study Notes
Dermatomyositis (DM)
- Characterized by mechanic's hand, which is a rough and cracked skin on the lateral and palmar areas of the fingers
- Telangiectasia and vasculopathic changes, including dilated capillary loops in the nailfolds and gum
- Systemic symptoms include fever, malaise, weight loss, arthralgia, and Raynaud's phenomenon
- Other manifestations include:
- Arthralgia, synovitis, or deforming arthropathy with subluxation in the interphalangeal joints
- Oesophagus: dysphagia and gastrointestinal symptoms
- Lung: pulmonary dysfunction due to weakness of the thoracic muscles
- Subcutaneous calcifications and joint contractures, especially in children
- Cardiac disturbances and malignancy in adults
Pathogenesis of DM
- Involves CD4+ T-cells and B-cells in the perimysial connective tissue and around blood vessels
- Deposition of C5b-9 (MAC) in capillary vessels
- Autoantibodies, including:
- ANA (40-60% of patients)
- Anti-Mi2 antibodies (25% of patients)
- Anti-helicase antibodies (implicated in nucleosome remodelling)
- Anti-Jo1 antibodies (associated with interstitial lung disease, arthritis, and "mechanic's hands")
- Anti-P155/P140 antibodies (associated with paraneoplastic and juvenile cases of DM)
Polymyositis (PM)
- Characterized by bilateral proximal muscle weakness, similar to DM but without a rash
- May also have gastrointestinal, skin, cardiac, and peripheral neuropathy symptoms
- Microscopic pathology: granulomatous vasculitis and p-ANCA (MPO-ANCA) positive
- Treatment: supportive therapy and glucocorticoids
Henoch-Schonlein Purpura
- Characterized by IgA vasculitis and immune complex-mediated vasculitis, often following upper respiratory tract infection in young children
- Presentation: skin lesions on the buttocks and legs, progressing from blanching macules to petechiae to palpable purpura
- Other symptoms: oedema, polyarthritis, colicky abdominal pain, and haematuria
- May have associated IgA nephropathy
- Diagnosis: clinical, as lab results are often normal
- Treatment: supportive therapy and corticosteroids for gastrointestinal symptoms
Vasculitides
- p-ANCA (MPO-ANCA) and c-ANCA (PR3-ANCA) associated with different types of vasculitides
- General symptoms: fever, fatigue, weight loss, and specific symptoms depending on the affected organ
- Endothelial cells are attacked by immune cells, leading to direct damage and organ symptoms
- Immune cells accidentally target endothelial cells due to antigen mimicry (e.g., MPO, PR3)
Giant Cell Arteritis (GCA)
- Formerly known as temporal arteritis
- Granulomatous arteritis of large to small-sized arteries, mainly arteries in the head (extracranial branches of carotids)
- Commonest primary systemic vasculitis in individuals over 50 years old
- Symptoms: fever, fatigue, weight loss, headaches, visual disturbances, facial pain, and scalp tenderness
- Complication: 25% of untreated patients go blind
- Therapy: steroids
Polymyalgia Rheumatica (PMR)
- An inflammatory disorder that causes sudden bilateral neck, shoulder, and low back muscle pain and stiffness
- May be associated with malaise, tiredness, weight loss, depression, and fever
- Elevated ESR and CRP
- Marked improvement with steroids
- Prevalence: 1:133 in individuals over 50 years old
- Often seen with giant cell arteritis
Takayasu Arteritis
- Granulomatous vasculitis that mainly affects the aorta and its branches
- Symptoms: weak or no pulse in the upper extremities, visual and neurologic symptoms, and marked weakening of the pulses in the upper extremities
- Segmental and patchy granulomatous inflammation
- Asian women are more commonly affected
- Symptoms are vague, varied, and perplexing
- Low-grade fever, ischemia, and infarction of affected tissues and organs
- Treatment: steroids
Polyarteritis Nodosa (PAN)
- Segmental transmural necrotizing inflammation of small- to medium-sized arteries
- Every layer of the artery is affected
- Transmural inflammation of the arterial wall with a mixed infiltrate of neutrophils, eosinophils, and mononuclear cells
- Fibrinoid necrosis and thrombotic occlusion of the lumen
- Later, fibrous (occasionally nodular) thickening of the vessel wall that can extend into the adventitia
Kawasaki Disease
- Acute febrile, usually self-limited illness of infants and children (boys more commonly affected)
- Mucocutaneous symptoms and cervical lymph node enlargement
- Large, medium, and small arteries are affected, mostly coronary arteries
- Without treatment, there is a 25% risk of heart complications
- Classic symptoms: conjunctivitis, rash, adenopathy, strawberry tongue, and fever
- Anti-endothelial and smooth muscle antibodies
- Treatment: IVIG
Buerger's Disease / Thromboangiitis Obliterans
- Affects medium and small vessels
- Epidemiology: heavy smokers, males, 20-40 years old
- Pathogenesis: clots in vessels supplying fingers and toes
- Patients develop ulcers on fingers and toes
- Often co-exists with Raynaud's phenomenon
- Intermittent claudication may lead to gangrene and digit auto-amputation
- Superficial nodular phlebitis
- Histology: segmental thrombosing vasculitis
- Treatment: smoking cessation
Small Vessel Vasculitides
- ANCA-associated:
- Microscopic polyangiitis
- Granulomatosis with polyangiitis (Wegener's granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Mediated by immune complexes:
- IgA vasculitis (Henoch-Schonlein purpura)
Microscopic Polyangiitis (MPA)
- Leukocytoclastic or hypersensitivity vasculitis
- Transmural, necrotizing vasculitis of small vessels
- Organs involved: skin, mucous membranes, lungs, GI, kidneys, heart, and brain
- Is (MPO-ANCA) perinuclear ANCA / anti-myeloperoxidase
- Treatment: glucocorticoids and cyclophosphamide
Granulomatosis with Polyangiitis (Wegener's Granulomatosis)
- Classical clinical triad: kidney, nasopharynx, and lungs
- Kidney: focal necrotizing glomerulonephritis
- Nasopharynx: chronic pain from sinusitis, bloody mucus from ulcers
- Lungs: pneumonitis, haemoptysis, cough, and dyspnea
- D Biopsy: focal necrotizing vasculitis, granulomatous change, and PR3-ANCA (anti-proteinase 3/c-ANCA) positive
- Rapidly fatal if left untreated
- Treatment: glucocorticoids and cyclophosphamide
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
- Small vessel necrotizing vasculitis
- Typically affects vessels of the respiratory tract
- Strongly associated with asthma, eosinophilia, and pulmonary infiltrates
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Description
Test your knowledge on the manifestations of diabetes mellitus, including 'mechanic's hand', telangiectasia, vasculopathic changes, systemic symptoms, arthralgia, Raynaud’s phenomenon, and gastrointestinal symptoms.
