Clinical description Patients most commonly present during the fourth decade of life but onset during childhood has been described. [orpha.net]

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]

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]

Gastrointestinal complaints ranging from nausea to severe abdominal pain are present in one third of patients as well. Rarely, patients present signs of gastrointestinal bleeding. ANCA disease has a vast array of presentations. [renalandurologynews.com]

• 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]

  In general, patients with small vessel vasculitis present with fever, myalgias, arthralgias and malaise and many describe a prior “flu-like” illness. [propath.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]

  Unexplained fever, weight loss. Unexplained proteinuria with or without casts. [de.slideshare.net]

• Constitutional Symptom

  Constitutional symptoms including fever and weight loss were noticed by most of the (70%) patients. Neurological deficits were present in 83% of the study population. [jfmpc.com]

  Many patients present with constitutional symptoms and less specific signs. [els.net]

  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]

  Extrapulmonary Manifestations of Systemic Vasculitis Systemic vasculitis can cause nonspecific constitutional symptoms such as fever, weakness, and weight loss. [clinicaladvisor.com]

• Cough

  Cardiorespiratory manifestations include cough, dyspnea, pleural and pericardial effusions, and emphysema, with chronic obstructive pulmonary disease reported in 20-50% of patients. [orpha.net]

  […] the involved vessels. 1, 3, 7, 9 The kidneys are the most commonly affected organs in 90 percent of patients who have this type of vasculitis. 3, 9 Patients present with variable combinations of renal manifestations, palpable purpura, abdominal pain, cough [aafp.org]

  Image Copyright: Designua / Shutterstock Symptoms of small-vessel vasculitis include: General symptoms such as fever and weight loss Myalgia and arthralgia Purpura Renal involvement Respiratory symptoms such as cough and breathlessness Mononeuritis Gastrointestinal [news-medical.net]

  Chest X-ray should be performed on any patient with complaints of cough or hemoptysis when considering small vessel vasculitis. Diffuse alveolar hemorrhage can be seen as patchy infiltrates. [renalandurologynews.com]

  Careful review of preceding or accompanying symptoms - especially fever, abdominal pain, numbness or tingling of the hands or feet, presence of gross hematuria or bloody stools, and cough or shortness of breath - is important for prompt identification [rheumatologyadvisor.com]

• Dyspnea

  Cardiorespiratory manifestations include cough, dyspnea, pleural and pericardial effusions, and emphysema, with chronic obstructive pulmonary disease reported in 20-50% of patients. [orpha.net]

  Manifestations Constitutional Fever, weight loss, anorexia, general malaise Musculoskeletal Myalgia, arthralgia Skin Palpable purpura, urticaria Kidneys Proteinuria, hematuria, renal insufficiency, renal failure, necrotizing glomerulonephritis Respiratory tract Dyspnea [aafp.org]

  De Madrid, Spain, Tel: 34627313534, Email: [email protected] Received Date: Apr 04, 2016 / Accepted Date: Apr 25, 2016 / Published Date: Apr 28, 2016 A 63 years-old man was admitted with two weeks history of weakness, generalized arthralgias and mild dyspnea [omicsonline.org]

  Pulmonary involvement of disease may manifest as dyspnea, hemoptysis, chest pain or non productive cough. Sinus congestion, clear rhinorhea or bloody/crusty nasal discharge is common in MPA and GPA. [renalandurologynews.com]

  Maintenance Therapy After remission is achieved, the glucocorticoid dose should be tapered carefully with a goal dose of < 10 mg daily of prednisone by 6 months, with close monitoring of pulmonary parameters, including chest imaging, symptoms of dyspnea [clinicaladvisor.com]

• Arthralgia

  Diagnostic methods Diagnosis requires the presence of two major criteria (recurrent urticaria for > 6 months and hypocomplementemia) and at least two minor criteria (leukocytoclastic vasculitis on biopsy, arthralgia and arthritis, ocular inflammation, [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]

• Purpura

  (HSP) MC vasculitis in children with most cases occurring before 6 years of 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 [quizlet.com]

  The most common types of IC-mediated vasculitis are hypersensitivity vasculitis, Henoch-Schönlein purpura (HSP), and mixed cryoglobulinemia. [musculoskeletalkey.com]

  Immune complex vasculitis reviewed in this chapter includes IgA vasculitis (Henoch-Schönlein purpura) and cryoglobulinemic vasculitis. [uncch.pure.elsevier.com]

  Adult onset Henoch-Schönlein purpura: Case report and review of literature. Conn Med. 2015;79:81–5. 2 Yang YH, Yu HH, Chiang BL. The diagnosis and classification of Henoch-Schönlein purpura: an updated review. [biochemia-medica.com]

  Treatment treat underlying HCV infection corticosteroids Henoch-Schonlein purpura (IgA Vasculitis) Please rate topic. [step1.medbullets.com]

• Palpable Purpura

  […] with most cases occurring before 6 years of 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 [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]

  Henoch-Schönlein Purpura • • • • • Also referred to as anaphylactoid purpura characterized by – palpable purpura (most commonly distributed over the buttocks and lower extremities) – arthralgias – gastrointestinal signs and symptoms Glomerulonephritis [de.slideshare.net]

  Purpuric lesions are not always palpable to the touch, and the existence of palpable purpura does not necessarily imply an IC-mediated pathophysiology; pauci-immune forms of vasculitis such as granulomatosis with polyangiitis (GPA) (formerly Wegener’s [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]

• Erythema

  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]

  Rarer forms of this condition include hypocomplementemic urticarial vasculitis and erythema elevatum diutinum. [musculoskeletalkey.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]

• Urticaria

  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]

  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]

  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]

• Hyperpigmentation

  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]

  " on " Hyperpigmentation ") A portion of cases may be persistent or recurrent. [en.wikipedia.org]

  Following resolution, purpuric lesions may leave postinflammatory hyperpigmentation, particularly if repeated bouts occur (see Figure 91-3F ). [musculoskeletalkey.com]

  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]

  Patchy alopecia, scaling, erythema and hyperpigmentation are seen. Lesions typically involve the pinnae, face, feet and tip of the tail often occurring over pressure points. [vin.com]

• Withdrawn

  New lesions may appear almost daily until treatment is initiated or a trigger withdrawn. Successive flares can occur at various intervals, interspaced from days or weeks to sometimes years. [rheumatologyadvisor.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]

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]

• Fibrinoid Necrosis

  Biopsy of a lesion showed leukocytoclastic vasculitis, with perivascular predominantly neutrophilic infiltrate and fibrinoid necrosis (negative immunofluorescence study). Figure 3. [rheumatologyadvisor.com]

  necrosis – Crescent formation – Absent/paucity of Ig/C3/C4 deposits 35. [de.slideshare.net]

  Leukocytoclastic vasculitis is characterized by the inflammation of small vessels, mainly post-capillary venules showing leukocytoclasis (destructive fragmentation of the nucleus of a dying cell) of infiltrating neutrophils, fibrinoid necrosis of the [synapse.koreamed.org]

  Immunofluorescent stains reveal complement, immunoglobulins, and fibrinogen deposited in the vessel walls, usually the venules, and histologically the vessels show fibrinoid necrosis and inflammation. [humpath.com]

[…] criteria for cutaneous leukocytoclastic vasculitis need 3/5: Age > 16 years old Temporal use of possible offending medication Palpable purpura Maculopapular rash Skin biopsy showing neutrophilic infiltration around arterioles or venules What is the treatment [quizlet.com]

Management and treatment There is no specific treatment and management requires tailored therapy with steroids and immunosuppressives. [orpha.net]

[…] and prognosis Treatment options in severe forms usually include methylprednisolone +/- cyclophosphamide. [radiopaedia.org]

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]

Topical treatments Topical treatments such as emollients or corticosteroid creams are sometimes prescribed and may transiently alleviate pruritus when present. The efficacy of such treatments is not supported by any data and is highly variable. [rheumatologyadvisor.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 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]

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]

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]

[…] include autoimmune conditions and malignancies, usually hematologic (related to the blood). [5] [6] Most Common Etiologies in Adults [5] [6] Cause Frequency Examples Idiopathic 45-55% Cause unknown Infection 15-20% Staphylococcus and Streptococcus spp [en.wikipedia.org]

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]

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]

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]

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 skin, GI tract, joints, kidneys ) → activation of complement [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]

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]

For example, combination therapy with cyclophosphamide and methylprednisolone is offered to those with renal involvement in Wegener's granulomatosis to prevent progression to end stage renal disease, 32 while even co‐trimoxazole is sufficient treatment [ncbi.nlm.nih.gov]