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Evaluation of the patient with vasculitis

2026-04-16 - 11:51

Evaluation of the patient with vasculitis

Lazăr Călin -University of Medicine and Pharmacy „Iuliu Hațieganu” Cluj-Napoca

Condition	Anamnesis + Physical Examination	Laboratory Tests	Imaging	Response to Treatment
IgA Vasculitis (Henoch–Schönlein Purpura)	Necrotizing small-vessel vasculitis with predominantly IgA immune deposits.Palpable purpura (appearing mainly on the lower limbs and buttocks) plus at least one of the following:a. Gastrointestinal manifestations (abdominal pain, gastrointestinal bleeding, sometimes ileus or intussusception);b. Arthritis/arthralgia with subcutaneous edema on the dorsal surfaces of the hands and feet;c. IgA nephritis (proteinuria, hematuria, sometimes hypertension);d. Histopathological evidence (leukocytoclastic vasculitis with IgA or IgA nephritis).	Normal platelet count, positive fecal occult blood test (indicating gastrointestinal involvement), negative immunology (ANA, ANCA, RF), hematuria (>5 RBCs/field) and proteinuria (>0.3 g/24 hours or at least 2+ on dipstick), elevated serum IgA (in one-third of cases).Nephritis requires renal assessment, including determination of the glomerular filtration rate and serum levels of C3 and C4. In severe or persistent nephritis, renal biopsy is recommended.	Abdominal and renal ultrasound, brain MRI in cases of cerebral vasculitis.	Supportive treatment (hydration, avoidance of physical exertion), NSAIDs for arthritis/arthralgia,Antihypertensives (angiotensin-converting enzyme inhibitors). Corticosteroid therapy (Prednisone 1–2 mg/kg/day for 1–2 weeks) or pulse therapy (in severe forms) with Methylprednisolone 10–30 mg/kg/day, maximum 1 g/day for 3 days, in cases of cerebral vasculitis, pulmonary hemorrhage, or significant gastrointestinal involvement.For nephritis, corticosteroid therapy or immunosuppressants (Cyclophosphamide, Mycophenolate mofetil, etc.) with nephrologist approval. Recurrent or persistent forms – corticosteroid or immunosuppressive therapy (requires rheumatology consultation).
Kawasaki Disease	Medium-vessel vasculitis of early childhood (most often under 5 years of age), self-limiting in course, and the most common cause of acquired heart disease in children.Diagnosis in the complete form: high fever without an evident focus, persisting for ≥5 days, plus at least 4 of the following 5 criteria:a. Bilateral conjunctivitis without exudate;b. Non-vesicular rash, especially on the trunk; c. Palmar and plantar erythema and edema with desquamation after 10–14 days;d. Oropharyngeal changes (red, cracked lips; strawberry tongue; pharyngeal erythema);e. Unilateral cervical lymphadenopathy >1.5 cm.There are incomplete forms that require additional laboratory criteria.Possible systemic involvement: cardiovascular (tachycardia, murmurs, heart failure, myocardial infarction), pneumonitis, arthritis/arthralgia, gastrointestinal (gastroenteritis, hepatitis), renal (aseptic urethritis, nephritis), and central nervous system (aseptic meningitis, cranial nerve palsies, seizures, ataxia).	Initial evaluation requires a complete pediatric examination (including monitoring of vital signs), cardiologic assessment (ECG and echocardiography), and, if necessary, neurological evaluation, along with laboratory tests: complete blood count, platelet count, ESR, CRP, albumin, ALT, AST, bilirubin, electrolyte panel, creatinine, urinalysis, bacteriological tests for possible infectious foci, ferritin, and fibrinogen (in cases with suspected macrophage activation).Clinically incomplete forms may require treatment when associated with laboratory abnormalities: anemia, thrombocytosis >450,000/mm³, leukocytosis >15,000/mm³, elevated ALT, albumin ≤3 g/dl, or leukocyturia ≥10/field.	Echocardiography to detect aneurysms is part of the initial evaluation. In cases of active disease or coronary abnormalities, it should be repeated after 1 week; for the remaining patients, it should be repeated 2 weeks after IVIG administration and again 6–8 weeks after disease onset.	Treatment of choice: Intravenous immunoglobulin (IVIG) 2 g/kg single dose (preferably within the first 10 days from onset), in combination with Aspirin 30–50 mg/kg/day or 80–100 mg/kg/day (divided into 4 doses).After 48 hours of being afebrile, Aspirin is continued at an antiplatelet dose of 3–5 mg/kg/day (single daily dose) for 1–2 months (if no coronary abnormalities) or long-term (if abnormalities are present).If fever persists 36 hours after the first dose of IVIG, the infusion may be repeated. In cases of IVIG resistance (after 2 doses), pulse therapy with Methylprednisolone 10–30 mg/kg/day (maximum 1 g/day) for 3 days may be administered, followed by Prednisone 2 mg/kg/day for 7 days, then tapered over 2–3 weeks.Other indications for corticosteroid therapy: signs of hemophagocytosis, cardiogenic shock, or established aneurysms. Alternatives in tertiary centers for resistant forms include Infliximab and Heparin (for giant aneurysms).
Takayasu Arteritis	Granulomatous panarteritis affecting the aorta and its main branches, and sometimes the pulmonary arteries.Diagnosis: angiographic abnormalities of the aorta or pulmonary arteries plus one of the following:a. Pulse deficit or claudication;b. Blood pressure discrepancy between limbs (>10 mmHg);c. Vascular bruits over major arteries; d. Arterial hypertension (above the 95th percentile for age);e. Elevated inflammatory markers (ESR, CRP).Nonspecific onset (fever, weight loss, myalgia, arthralgia, symptoms due to hypertension), followed by the occlusive phase: involvement of the aortic arch (brachiocephalic ischemia – headache, syncope, vertigo, hearing loss, visual disturbances, cerebrovascular accidents); subclavian involvement (pain or heaviness in the upper limbs, hypotrophy, diminished pulses, skin ulcerations, or livedo reticularis); thoracoabdominal involvement (systolic bruits, pulse and blood pressure discrepancies, renovascular hypertension, mesenteric infarctions); pulmonary involvement (dyspnea, pleurisy, pulmonary hypertension).	Elevated CRP and ESR, anemia, thrombocytosis, negative ANA and ANCA.Renal assessment and monitoring in cases of renal artery stenosis.Target organ monitoring (ECG, echocardiography, ophthalmologic examination, etc.).	Doppler ultrasound (carotid and renal arteries)Conventional arteriography, CT, or MRI.	Diagnosis and treatment in specialized tertiary centers.Induction: systemic corticosteroid therapy plus Cyclophosphamide and/or Methotrexate.Maintenance: Methotrexate, Azathioprine, Mycophenolate mofetil.Refractory cases: biological therapies (anti-TNF, anti-IL6).Antiplatelet and/or anticoagulant therapy.
Polyarteritis Nodosa (PAN)	Necrotizing vasculitis of medium/small vessels. Diagnosis: demonstration of necrotizing vasculitis (histopathologically) or aneurysms/stenosis/occlusion of medium or small vessels (angiographically), plus at least one of the following:a. Cutaneous involvement (purpura, livedo reticularis, cutaneous nodules, skin or deep ulcers of the digits or other peripheral tissues – nose/auricle, necrosis, gangrene);b. Myalgia;c. Arterial hypertension;d. Peripheral sensory neuropathies or mononeuritis multiplex;e. Ischemic vascular nephropathy (hematuria, proteinuria, renal failure). Associated findings include fever, weight loss, and sometimes gastrointestinal (hemorrhage, infarction), cardiovascular manifestations, or orchitis.There is a cutaneous form with predominantly skin involvement.	Leukocytosis, thrombocytosis, and elevated ESR/CRP values.ANA and ANCA negative.Renal assessment (proteinuria, hematuria, urea, creatinine, glomerular filtration rate) in cases of renal involvement.Histopathological examination with biopsy of the affected organs.	ECG and echocardiography in cases of cardiac involvement.Angiography (MRI or CT)	Diagnosis and treatment in tertiary centers, especially for the systemic form.Systemic form:Acute phase – high-dose corticosteroid therapy plus CyclophosphamideMaintenance – low-dose corticosteroids plus Azathioprine.Cutaneous form: NSAIDs, corticosteroids.Long-term prophylaxis with Penicillin in cases with evidence of streptococcal infection.
Granulomatosis with Polyangiitis (formerly Wegener’s Granulomatosis)	Within the group of ANCA-associated vasculitides, there is a localized form (mainly affecting the respiratory tract) and a systemic, multiorgan form.1. General manifestations: fever, weight loss;2. ENT involvement: purulent or bloody rhinorrhea, nasal crusting, nasal septum perforation, saddle-nose deformity, chronic otitis media, chronic sinusitis, dysphonia, laryngeal stenosis;3. Respiratory manifestations: cough, hemoptysis, chest pain, dyspnea;4. Rapidly progressive glomerulonephritis: hematuria, proteinuria, hypertension, renal failure;5. Cutaneous: nodules, ulcerations;6. Neurological: peripheral neuropathy, cerebral vasculitis;7. Cardiovascular: pericarditis, thrombosis;8. Ocular: uveitis, orbital granulomas.	Anemia, leukocytosis, thrombocytosis, and markedly elevated ESR/CRP values.ANCA antibodies variably positive (by immunofluorescence and ELISA): proteinase 3, myeloperoxidase.Assessment and monitoring of renal function.Histopathological examination of lesions.	Chest CT, MR angiography (for thrombosis), echocardiography (for pericarditis)	Diagnosis and treatment in specialized centers: high-dose corticosteroid therapy (pulse therapy), Cyclophosphamide, Methotrexate, Azathioprine, and more recently Rituximab and Mycophenolate mofetil.Antiplatelet therapy, prophylaxis of opportunistic infections.

Monitoring of vasculitis includes: physical examination, anthropometric indices, neurological and musculoskeletal assessment, measurement of acute-phase reactants (leukocytes, CRP), and functional evaluation of the affected organs. For monitoring, the Pediatric Vasculitis Activity Score (PVAS) can be used both at initial assessment and during follow-up visits, while persistent involvement (over 3 months) is evaluated using the Paediatric Vasculitis Damage Index (PVDI). Both scores cover 9 domains: general, cutaneous, mucosal/ocular, ENT, respiratory, cardiovascular, gastrointestinal, renal, and central nervous system manifestations.