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Immune Complex–Mediated Small Vessel Vasculitis


Presentation

  • Large ICs, formed when antibody and antigen are present in approximately equal proportions, are identified and removed easily by the reticuloendothelial system. In contrast, small ICs are formed in conditions of antibody excess.[musculoskeletalkey.com]
  • Clinical description Patients most commonly present during the fourth decade of life but onset during childhood has been described.[orpha.net]
  • IgA Vasculitis (Henoch-Schรถnlein Purpura) Henoch-Schรถnlein (HSP) usually presents in children younger than 5 years. It presents less frequently in adults.[ime.acponline.org]
  • Constitutional symptoms of fever, loss of appetite, and weight were present in 70% (9) of patients. Neurological deficits were present in 10 (83%) patients at the time of presentation.[jfmpc.com]
Pain
  • Snapshot A 5-year-old boy presents to the emergency room for abdominal pain and rash on his legs. He also reports having intermittent joint pains. A few weeks ago, he had an upper respiratory infection.[step1.medbullets.com]
  • Patients present with generalized urticarial eruptions located on the trunk, proximal extremities and face that are often associated with itching or pain and persist for more than 24 hours, with hyperpigmentation after resolution.[orpha.net]
  • It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/ arthralgia, abdominal pain, and renal disease.[amboss.com]
  • […] reported symptoms include mild fever, muscle pain, joint pain, or an overall feeling of discomfort. [6] [7] Additional symptoms depend on the cause of the vasculitis and if other organ systems are involved.[en.wikipedia.org]
  • C/F • Fixed annular wheals • 24 hr • pain and burning sensation rather then itching • hyperpigmentation on resolution • Systemic symptoms and signs 48.[de.slideshare.net]
Fever
  • It is characterized by itching, malaise, and a slight fever and by the presence of papules, vesicles, urticarial wheals, or small ulcers on the skin.[medical-dictionary.thefreedictionary.com]
  • Lymphocytic vasculitis may also be seen in rickettsial infections such as Rocky Mountain spotted fever.[jamanetwork.com]
  • Many patients develop their disease against a background of non‐specific symptoms, such as malaise, weight loss, fever and night sweats (so‐called B‐symptoms), which reflect constitutional symptoms.[academic.oup.com]
  • In general, patients with small vessel vasculitis present with fever, myalgias, arthralgias and malaise and many describe a prior “flu-like” illness.[propath.com]
  • Unexplained fever, weight loss. Unexplained proteinuria with or without casts.[de.slideshare.net]
Abdominal Pain
  • Snapshot A 5-year-old boy presents to the emergency room for abdominal pain and rash on his legs. He also reports having intermittent joint pains. A few weeks ago, he had an upper respiratory infection.[step1.medbullets.com]
  • Other systemic findings include gastrointestinal symptoms (abdominal pain, nausea, diarrhea, vomiting), musculoskeletal manifestations (arthritis and transient arthralgia affecting the hands, elbows, knees, ankles, and feet), ocular inflammation (episcleritis[orpha.net]
  • HSP is associated with purpura, arthritis, glomerulonephritis, and colicky abdominal pain. IgA deposition is found within blood vessel walls. Cryoglobulinemic vasculitis is most often associated with long-standing hepatitis C virus infection.[musculoskeletalkey.com]
  • It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/ arthralgia, abdominal pain, and renal disease.[amboss.com]
  • The patient subsequently started having abdominal pain and bloody diarrhea. Kidney function was normal at the time, but she had mild elevation of her liver enzymes. Her WSR was 58.[nature.com]
Colic
  • CT scan also showed spindle shape aneurysms at the origin of the celiac trunk (13 mm diameter), the superior mesenteric artery (16 mm diameter), splenic artery (11 mm) and left colic branch (1 cm), with spindle ectasia of both iliac arteries ( Figure[omicsonline.org]
Arthralgia
  • For example, patients with cutaneous disease and arthralgias but no major organ involvement may be managed with low-dose prednisone, hydroxychloroquine, or dapsone; whereas patients with major organ involvement, such as glomerulonephritis, may require[orpha.net]
  • It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/ arthralgia, abdominal pain, and renal disease.[amboss.com]
  • Vasculitis manifests in types II and III cryoglobulinemic patients with a triad of arthralgias, myalgias, and palpable purpura. Involvement of medium-sized vessels results in cutaneous ulcers, digital ischemia, and fixed livedo reticularis.[ime.acponline.org]
  • […] as a hyperviscosity syndrome Cryoglobulinemia is closely associated with what other disease Very strong association with hepatitis C infection (90% of cases) which is probably etiologic What is the classic clinical triad of cryoglobulinemia purpura arthralgias[quizlet.com]
  • CASE REPORT A 72-year-old man was admitted to our hospital with a 4-month history of skin lesions with myalgias, arthralgias, swelling on his extremities, and gait disturbance.[medicaljournals.se]
Red Eye
  • Scleral injection or "red eyes" can be noted. A slit lamp evaluation will likely be required for detailed evaluation of ocular vasculitis. Examining the ears, nose, and throat is particularly important for ANCA disease.[renalandurologynews.com]
Purpura
  • The most common types of IC-mediated vasculitis are hypersensitivity vasculitis, Henoch-Schönlein purpura (HSP), and mixed cryoglobulinemia.[musculoskeletalkey.com]
  • purpura Bowel angina Vessel wall granulocytes on biopsy What is the treatment for Henoch-Schonlein Purpura (HSP) Supportive care Tylenol or NSAIDS Severe cases should be hospitalized Prognosis: course usually self-limited & resolves w/in weeks to months[quizlet.com]
  • Immune complex vasculitis reviewed in this chapter includes IgA vasculitis (Henoch-Schönlein purpura) and cryoglobulinemic vasculitis.[uncch.pure.elsevier.com]
  • The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum 1990;33:1114–1121. PubMed CrossRef Google Scholar 7.[link.springer.com]
  • Treatment treat underlying HCV infection corticosteroids Henoch-Schonlein purpura (IgA Vasculitis) Please rate topic.[step1.medbullets.com]
Palpable Purpura
  • The classic cutaneous finding in small vessel vasculitis is palpable purpura, but a variety of other skin lesions may be found including pustules, vesicles, urticaria, and small ulcerations.[musculoskeletalkey.com]
  • What is the "classic" finding for virtually any type of small vessel vasculitis Palpable purpura Generally classified as immune complex (IC) mediated or antineutrophilic cytoplasmic antibody (ANCA) What are the immune-complex associated vasculitides 1[quizlet.com]
  • In the skin, palpable purpura, the most common cutaneous finding in IC-mediated vasculitis, results from extravasation of erythrocytes through damaged vessel walls ( Plate 82 ).[ime.acponline.org]
  • Clinical presentation Predominantly cutaneous vasculitis • Usual presentation is in the form of palpable purpura, urticaria, bullous ulcers or splinter haemorrhages. • Mainly limited to lower extremities. • Salient features are:• • • • Absence of systemic[de.slideshare.net]
  • It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/ arthralgia, abdominal pain, and renal disease.[amboss.com]
Palpable Purpura
  • The classic cutaneous finding in small vessel vasculitis is palpable purpura, but a variety of other skin lesions may be found including pustules, vesicles, urticaria, and small ulcerations.[musculoskeletalkey.com]
  • What is the "classic" finding for virtually any type of small vessel vasculitis Palpable purpura Generally classified as immune complex (IC) mediated or antineutrophilic cytoplasmic antibody (ANCA) What are the immune-complex associated vasculitides 1[quizlet.com]
  • In the skin, palpable purpura, the most common cutaneous finding in IC-mediated vasculitis, results from extravasation of erythrocytes through damaged vessel walls ( Plate 82 ).[ime.acponline.org]
  • Clinical presentation Predominantly cutaneous vasculitis • Usual presentation is in the form of palpable purpura, urticaria, bullous ulcers or splinter haemorrhages. • Mainly limited to lower extremities. • Salient features are:• • • • Absence of systemic[de.slideshare.net]
  • It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/ arthralgia, abdominal pain, and renal disease.[amboss.com]
Urticaria
  • The classic cutaneous finding in small vessel vasculitis is palpable purpura, but a variety of other skin lesions may be found including pustules, vesicles, urticaria, and small ulcerations.[musculoskeletalkey.com]
  • Allergic vasculitis allergic vasculitis A form of nonthrombocytopenic purpura due to a hypersensitivity vasculitis associated with urticaria, erythema, arthritis, gastrointestinal symptoms and renal involvement.[medical-dictionary.thefreedictionary.com]
  • Clinical presentation Predominantly cutaneous vasculitis • Usual presentation is in the form of palpable purpura, urticaria, bullous ulcers or splinter haemorrhages. • Mainly limited to lower extremities. • Salient features are:• • • • Absence of systemic[de.slideshare.net]
  • Symptoms Symptoms may include: New rash over large areas Purple-colored spots and patches on the skin Skin sores mostly located on the legs, buttocks, or trunk Blisters on the skin Hives ( urticaria ), may last longer than 24 hours Open sores with dead[health.ridgeviewmedical.org]
  • Urticaria can also be a manifestation of small-vessel vas-culitis. Unlike nonvasculitic allergic urticaria, vasculitic urticaria lasts more than one day and may evolve into purpuric lesions.[aafp.org]
Erythema
  • Rarer forms of this condition include hypocomplementemic urticarial vasculitis and erythema elevatum diutinum.[musculoskeletalkey.com]
  • Allergic vasculitis allergic vasculitis A form of nonthrombocytopenic purpura due to a hypersensitivity vasculitis associated with urticaria, erythema, arthritis, gastrointestinal symptoms and renal involvement.[medical-dictionary.thefreedictionary.com]
  • Less commonly, this histologic pattern may be seen in non-immunologically mediated processes, such as in the presence of bacterial toxins or malignant hypertension, or in more chronic disease states, such as granuloma faciale or erythema elevatum diutinum[jamanetwork.com]
  • The inflammatory infiltrate contributes to the raised nature and erythema of the lesions, while vessel damage and haemorrhage result in non-blancheable purpura.[oxfordmedicine.com]
  • Erythema caused by vasculitis does not blanche with diascopy because of extravasation of the red blood cells. Subcutaneous vasculitis presents as nodular lesions.[vin.com]
Confusion
  • Hypersensitivity angiitis, often confused with the pauci-immune form of vasculitis now termed microscopic polyangiitis (see Chapter 89 ), 1 was one of five disorders included in the original classification of the vasculitides in 1952. 2 This chapter focuses[musculoskeletalkey.com]
  • The term ‘pauci-immune’ used as an eponym for ANCA-associated vasculitis in a sense is confusing considering the presence of these autoantibodies in the blood, the influx of innate immune cells, and the severe damage that occurs to the glomerular capillary[karger.com]

Workup

  • Viral workup was negative for EBV, CMV, Hepatitis A, B, and C, HSV, & parvovirus. Immunologic studies were positive for C-ANCA, ANA, smooth muscle antibodies, and elevated IgA and IgG levels.[nature.com]
  • Thus, the first step in the workup of a patient with suspected vasculitis is to exclude other diseases that produce clinical manifestations that can mimic vasculitis (Table 10-3).[what-when-how.com]
  • IgA vasculitis is more likely to present with abdominal pain, bloody urine, and joint pain. [11] In the case that the cause is not obvious, a reasonable initial workup would include a complete blood count, urinalysis, basic metabolic panel, fecal occult[en.wikipedia.org]
  • DIAGNOSTIC WORKUP Laboratory investigations • • • • • • Full blood count with differential white cell count Markers of inflammation: ESR,CRP Electrolytes and hepatic transaminases, glucose Urinalysis for protein and blood Blood cultures (if pyrexial)[de.slideshare.net]
  • DAH in conjunction with other manifestations of systemic vasculitis (see section below on common findings) should trigger a workup to establish a diagnosis.[clinicaladvisor.com]
Hyperviscosity
  • […] vasculitides is caused by immunoglobulins (antibodies) that precipitate out of blood at low temperatures ( What type of cryoglobulins cause small vessel vasculitis Types II & III Type I is associated with hematologic syndromes which usually manifest as a hyperviscosity[quizlet.com]
  • Such patients most commonly present with hyperviscosity and thrombosis, but vasculitic symptoms can occur.[ime.acponline.org]

Treatment

  • The epidemiology, cause, distinctive pathophysiologic mechanisms, unique clinical features, and approaches to treatment are discussed separately for each condition. Treatments are also summarized in Table 91-1.[musculoskeletalkey.com]
  • […] and prognosis Treatment options in severe forms usually include methylprednisolone /- cyclophosphamide.[radiopaedia.org]
  • Management and treatment There is no specific treatment and management requires tailored therapy with steroids and immunosuppressives.[orpha.net]
  • […] age Henoch-Schonlein Purpura (HSP) is closely associated with what other disease What are the diagnostic criteria for Henoch-Schonlein Purpura (HSP) need 2/4: Age 20 years Palpable purpura Bowel angina Vessel wall granulocytes on biopsy What is the treatment[quizlet.com]
  • Large vessel vasculitis involvement was considered treatment resistant, thus high dose steroids treatment was maintained and a second line immune-suppression therapy with rituximab was proposed.[omicsonline.org]

Prognosis

  • Prognosis The prognosis for HUV patients is variable and is influenced primarily by the severity of pulmonary, cardiac and renal disease. When present, pulmonary disease is the major cause of death. Acute laryngeal edema can be life-threatening.[orpha.net]
  • HSP has an excellent prognosis, usually resolving within one month when not complicated by significant renal disease.[amboss.com]
  • […] for Henoch-Schonlein Purpura (HSP) need 2/4: Age 20 years Palpable purpura Bowel angina Vessel wall granulocytes on biopsy What is the treatment for Henoch-Schonlein Purpura (HSP) Supportive care Tylenol or NSAIDS Severe cases should be hospitalized Prognosis[quizlet.com]
  • Prognosis The prognosis of patients with cutaneous vasculitis depends on the underlying syndrome and the presence of end-organ dysfunction. Patients with disease that primarily affects the skin and/or the joints have a good prognosis.[emedicine.medscape.com]
  • Other described manifestations (which are non-specific as individual features) include 9 : regions of ground-glass attenuation pulmonary consolidation thickening of bronchovascular bundles honeycombing : features of pulmonary fibrosis 11 Treatment and prognosis[radiopaedia.org]

Etiology

  • The various etiologies causing vasculitis include drugs, infections, malignancies, and serum sickness.[jfmpc.com]
  • Most cases are of unknown etiology.[vin.com]
  • Etiology HUV generally occurs sporadically but occurrence in siblings has been described.[orpha.net]
  • III Type I is associated with hematologic syndromes which usually manifest as a hyperviscosity syndrome Cryoglobulinemia is closely associated with what other disease Very strong association with hepatitis C infection (90% of cases) which is probably etiologic[quizlet.com]
  • The etiology is unknown, but there may be an association with reactions to medications, and eosinophils are often present within the lesions. Histologically, giant cell phlebitis is characterized by granulomatous destruction of muscular veins.[uscapknowledgehub.org]

Epidemiology

  • The epidemiology, cause, distinctive pathophysiologic mechanisms, unique clinical features, and approaches to treatment are discussed separately for each condition. Treatments are also summarized in Table 91-1.[musculoskeletalkey.com]
  • Epidemiology Sex: More common in children 90% of cases 10 years old Peak incidence : 6 years References: [1] [2] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • Race Hypersensitivity vasculitis is reported most often in the white population, but epidemiologic studies are not available to assess whether hypersensitivity vasculitis is associated with any specific ethnic group or skin type.[emedicine.medscape.com]
  • Summary Epidemiology Prevalence is unknown but less than 200 cases have been reported in the literature. Women are more frequently affected than men (female to male ratio of 8:1).[orpha.net]
  • J Am Acad Dermatol 26: 441–448 PubMed Google Scholar Nordborg E (2000) Epidemiology of biopsy-positive giant cell arteritis: an overview.[link.springer.com]
Sex distribution
Age distribution

Pathophysiology

  • The epidemiology, cause, distinctive pathophysiologic mechanisms, unique clinical features, and approaches to treatment are discussed separately for each condition. Treatments are also summarized in Table 91-1.[musculoskeletalkey.com]
  • Nydegger UE (2007) Immune complex pathophysiology. Annals of the New York Academy of Sciences 1109: 66–83. Rich RR (1992) Immune complex diseases.[els.net]
  • ., yellow fever, cholera ) References: [3] Pathophysiology Hypothesized pathophysiological mechanism: exposure to allergen /antigen (e.g., infection, drugs) stimulation of IgA production deposition of IgA immune complexes in vascular walls (e.g., in the[amboss.com]
  • Abstract Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis.[karger.com]
  • This finding on histological examination is termed “leukocytoclastic vasculitis”. [5] Considering the wide range of potential causes leading to cutaneous small vessel vasculitis, there are subtle variations in the underlying pathophysiology for each cause[en.wikipedia.org]

Prevention

  • Possible Complications Complications may include: Lasting damage to the blood vessels or skin with scarring Inflamed blood vessels affecting the internal organs Prevention DO NOT take medicines which have caused an allergic reaction in the past.[health.ridgeviewmedical.org]
  • How can cutaneous small vessel vasculitis be prevented? Flares of cutaneous small vessel vasculitis can be minimised by rest, compression and elevation of lower legs.[dermnetnz.org]
  • Prevent complications such as infection (PPD, Pneumovax, and other immunizations; trimethoprim–sulfamethoxazole prophylaxis if on high dose prednisone), osteoporosis, and atherosclerosis (control blood pressure and lipids).[irheuma.com]
  • Treatment • Goal: Reduce the symptoms and to prevent visual loss • If clinical suspicion is high, treatment should NOT be delayed for biopsy • • • • • • Glucocorticoids Methotrexate Infliximab Azathioprine Imatinib mesylate Surgery 25.[de.slideshare.net]
  • However, anti-TNF-α treatment by a single intraperitoneal injection of anti-murine TNF-α antibody TN3 attenuated, but did not prevent, the LPS-mediated aggravation of anti-MPO IgG-induced glomerulonephritis.[karger.com]

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