eMedicine Specialties > Pediatrics: General Medicine > Rheumatology
Vasculitis and Thrombophlebitis: Treatment & Medication
Updated: Nov 23, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Most patients with vasculitis or thrombophlebitis who are not acutely ill may be evaluated on an outpatient basis.
- Admit patients with hypertension, mental status changes, pulmonary hemorrhage, or those who are acutely ill for diagnostic workup and stabilization.
- Admit patients with thrombophlebitis for evaluation and anticoagulation. Catheter-directed thrombolysis by an experienced physician may decrease morbidity due to postphlebitic syndrome.
- Treatment goals are to decrease acute inflammation of blood vessels and to maintain adequate perfusion of skin and vital organs, while limiting the side effects of potentially toxic therapies.
- Individualize treatment based on the organs affected and the overall condition of the patient. In general, corticosteroids are administered to control acute symptoms and laboratory evidence of systemic inflammation. After control is achieved, attempts may be made to taper over a month. For patients with renal or CNS involvement, immunosuppression with cyclophosphamide, azathioprine, methotrexate, or tumor necrosis factor blockade is used. Use of immunosuppressants and biological agents may enable reduction in steroid dose. The care of these patients should be centralized where possible to optimize diagnosis and treatment. Morbidity due to cumulative corticosteroid dose as well as toxicity from immunosuppression must be weighed in the long-term plan of care.
- Anticoagulation is indicated for any patient with a thrombotic episode and an underlying hypercoagulable state. The protocols set forth by Andrew in 1994 are useful to achieve therapeutic heparin and warfarin levels.11
- Base treatment of systemic vasculitis on the severity and distribution of end-organ involvement. Always seek infectious etiologies; patients with vasculitis secondary to hepatitis B respond to conventional therapy but become chronic viral carriers, and many progress to hepatic cirrhosis and esophageal varices.
Surgical Care
For patients with thrombophlebitis, removal of any local intravascular instrumentation is recommended. Placement of Greenfield filters is not recommended for patients with hypercoagulability.
- Stenting of stenotic vessels is increasingly used. Balloon dilatation has also been used to improve renovascular flow.
- Restenosis is an issue; 78% of patients with Takayasu arteritis undergoing angioplasty develop recurrent stenoses. Of patients undergoing surgical bypass procedures, 36% experience restenosis.
- Large vessels may require Gore-Tex or other synthetic replacements. Surgery should be postponed until the inflammatory disease is optimally controlled. However, despite normal clinical and laboratory findings, patients may have progression of disease and active inflammatory lesions found at the time of surgery.
- Patients with Wegener granulomatosis may develop subglottic stenosis; these lesions are also amenable to balloon dilatation.
Consultations
- Pediatric rheumatologist
- Pediatric nephrologist for patients with renal involvement
- Vascular surgeon or interventional radiologist
- Pediatric cardiologist, as indicated
- Pediatric neurologist, as indicated
Diet
- Prescribe a low-sodium diet if the patient is hypertensive.
Activity
- Activity may be performed as tolerated. Patients taking anticoagulants should not participate in contact sports.
Medication
Immunosuppression is achieved using corticosteroids and, for patients with renal or CNS involvement, with cyclophosphamide as induction therapy. Monthly pulse doses of cyclophosphamide are associated with a higher incidence of relapse. Following induction, azathioprine or methotrexate has been used as maintenance to reduce toxicity from cyclophosphamide. Anecdotally, treatment with etanercept, infliximab, or rituximab (anti-CD20) may be used.
With the heterogeneity of vasculitis syndromes, response to a given agent may vary. Tumor necrosis factor (TNF) blockade has been helpful in Takayasu arteritis but has not been useful in Wegener granulomatosis. A multicenter, double-blind randomized controlled study revealed no benefit and greater complication rate with etanercept in Wegener granulomatosis.12
Intravenous immunoglobulin (IVIG) and antiplatelet doses of aspirin are standard of care for Kawasaki disease. Large doses of glucocorticoids have been used in treatment resistant Kawasaki disease. A subset of patients may respond to IVIG (1 g/kg body weight; treatment failures receive 2 g/kg). This strategy attempts to decrease the amount of IVIG used. Patients who respond to the lower dose have no worse outcome but may represent a subset of patients with milder disease.
Corticosteroids
These agents have potent immunosuppressive activity with rapid onset of action.
Prednisone (Deltasone, Sterapred)
Used to control acute symptoms and laboratory evidence of inflammation. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.
Adult
5-60 mg/d PO qd or divided bid to normalize symptoms and laboratory parameters; taper possible by 2 wk in some entities, as symptoms resolve
Pediatric
1-2 mg/kg/d PO to control acute symptoms and laboratory evidence of inflammation; after achievement of control, attempts may be made to taper within 4 wk in some entities (varies)
Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI bleeding or ulceration
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Cushingoid side effects, including hypertension, hirsutism, moon facies, and striae; adrenal suppression, osteoporosis, pseudotumor cerebri, and increased appetite
Immunosuppressants
These agents help control inflammatory signs and symptoms.
Cyclophosphamide (Cytoxan)
Used as first-line therapy for severe systemic vasculitides such as Wegener granulomatosis and for steroid-refractory disease. Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.
Adult
1-2 mg/kg PO qd
0.5-1 g/m2 IV pulse every mo
Pediatric
Administer as in adults; has also been used at the lower dosage q2-3wk
Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity; watch for additive effects with other drugs inducing leukopenia
Documented hypersensitivity; severely depressed bone marrow function
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Hemorrhagic cystitis, syndrome of inappropriate antidiuretic hormone secretion, and hypertension; ovarian failure increases with age (>50% in patients >35 y) as does male sterility; neutropenia; nadir may occur between 7-14 d after IV dose
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.
Adult
1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d; not to exceed 100-150 mg/d PO
Pediatric
Initial dose: 2-5 mg/kg/d PO/IV
Maintenance dose: 1-2 mg/kg/d PO/IV; not to exceed 100-150 mg/d PO
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Documented hypersensitivity; low levels of serum thiopurine methyl transferase (TPMT)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Leukopenia; increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check TPMT level prior to therapy and monitor liver, renal, and hematologic function; pancreatitis rarely associated
Anticoagulants
These agents provide immediate and long-term treatment of vascular thrombosis.
Heparin
Augments activity of antithrombin III and prevents conversion of fibrinogen to fibrin. Does not actively lyse but is able to inhibit further thrombogenesis. Prevents reaccumulation of clot after spontaneous fibrinolysis.
Provide as continuous heparin infusion to maintain aPTT at 1.5 times the control.
Adult
Initial dose: 40-170 U/kg IV
Maintenance infusion: 18 U/kg/h IV
Alternatively, 50 U/kg/h IV initially, followed by continuous infusion of 15-25 U/kg/h and increase dose by 5 U/kg/h q4h prn using aPTT results
Pediatric
Initial dose: 50-100 U/kg IV
Maintenance infusion: 15-25 U/kg/h IV; increase dose by 2-4 U/kg/h q6-8h prn using aPTT results
Digoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, ASA, dextran, dipyridamole, and hydroxychloroquine may increase heparin toxicity
Documented hypersensitivity; subacute bacterial endocarditis; active bleeding; history of heparin-induced thrombocytopenia; severe thrombocytopenia; intracranial hemorrhage
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Heparin-induced thrombocytopenia may occur within hours of starting or restarting heparin therapy; in neonates, preservative-free heparin is recommended to avoid possible toxicity (gasping syndrome) by benzyl alcohol, which is used as preservative; use caution in severe hypotension and shock; monitor for bleeding in peptic ulcer disease, menstruation, increased capillary permeability, and when administering IM injections
Enoxaparin (Lovenox)
Low molecular weight heparin. Augments activity of antithrombin III and prevents conversion of fibrinogen to fibrin. Does not actively lyse but is able to inhibit further thrombogenesis. Prevents reaccumulation of clot after spontaneous fibrinolysis.
Advantages include intermittent dosing and decreased requirement for monitoring. Heparin anti–factor Xa levels may be obtained if needed to establish adequate dosing.
Adult
1 mg/kg SC bid
Pediatric
0.5-1 mg/kg SC bid
Platelet inhibitors or PO anticoagulants such as dipyridamole, salicylates, aspirin, NSAIDs, sulfinpyrazone, and ticlopidine may increase risk of bleeding
Documented hypersensitivity; subacute bacterial endocarditis; active bleeding; history of heparin-induced thrombocytopenia; severe thrombocytopenia; intracranial hemorrhage
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Possible accelerated osteoporosis with long-term use; risk of heparin-induced thrombocytopenia
Warfarin (Coumadin, Coumarin)
Interferes with hepatic synthesis of vitamin K–dependent coagulation factors.
Adult
5-10 mg PO qd; titrate after 2-5 d to maintain INR at 2.5-3.5 following a thrombotic episode; 2-10 mg/d usual dose
Pediatric
0.05-0.34 mg/kg/d PO
Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, PO contraceptives, and sucralfate; medications that may increase anticoagulant effects of warfarin include PO antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac
Documented hypersensitivity; severe liver or renal disease; active bleeding; peptic ulcer disease; malignant hypertension; pregnancy
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Response to PO anticoagulants may be markedly decreased in biliary obstruction because of reduced vitamin K absorption and may be decreased in hepatitis and cirrhosis because of decreased production of vitamin K–dependent clotting factors; do not switch brands once desired therapeutic range is achieved; discontinue use at least 3 d prior to invasive surgical procedure and check PT/INR
Immunomodulators
IVIG is used as first-line therapy for Kawasaki disease; it decreases risk of coronary artery aneurysms.
Immune globulin, intravenous (Sandoglobulin, Gamimune, Gammar-P)
Multiple mechanisms. May absorb superantigens or toxins in Kawasaki disease. May saturate available Fc receptors. May block cytokines, cytokine receptors, or both. May absorb complement activation products. May down-regulate immunoglobulin synthesis. Blocks Fc receptors on macrophages. Suppresses inducer T and B cells and augments suppressor T cells. Blocks complement cascade. May increase CSF IgG (10%).
Adult
2 g/kg IV over 2-5 d
Pediatric
2 g/kg IV infusion over 6-10 h for Kawasaki disease
Increases toxicity of live virus vaccine (MMR); do not administer within 3 mo of vaccine
Documented hypersensitivity; IgA deficiency; IgE/IgG anti-IgA antibodies
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Premedication with acetaminophen and diphenhydramine to be considered; possible reduction of adverse effects of flushing, fever, hypotension, and aseptic meningitis
Antibiotics
These agents play a role in the prophylaxis against relapse in Wegener granulomatosis (although an infectious etiology has not been identified).
Trimethoprim-sulfamethoxazole (Bactrim, Septra)
As Pneumocystis carinii prophylaxis. This drug may delay flare in patients with Wegener granulomatosis.
Dihydrofolate reductase inhibitor in combination with sulfonamide.
Adult
1 DS tab (160 mg TMP/800 mg SMZ) PO 3 times/wk
Pediatric
<2 months: Contraindicated
>2 months: 10 mg/kg/d, based on TMP, PO 3 times/wk; not to exceed 320 mg TMP
May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration of diuretics increases incidence of thrombocytopenia purpura in elderly patients; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
Documented hypersensitivity; megaloblastic anemia due to folate deficiency; porphyria; age <2 mo
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not use during last trimester of pregnancy because of potential toxicity to newborn (eg, jaundice, hemolytic anemia, kernicterus)
Dosage adjustments (adult adjustments)
CrCl (mL/min) 80-50: Recommended IV dose q18h
CrCl 50-10: Recommended IV dose q24h
CrCl <10: Not recommended
HD: 4-5 mg/kg after HD
During peritoneal dialysis: 0.16-0.8 g q48h
Discontinue at first appearance of skin rash or sign of adverse reaction; frequently obtain CBC counts; discontinue therapy if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, give 5-15 mg/d leucovorin); caution in folate deficiency (eg, chronic alcoholics, elderly persons, those receiving anticonvulsant therapy, those with malabsorption syndrome); hemolysis may occur in G-6-PD deficient individuals; patients with AIDS may not tolerate or respond to TMP-SMZ; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); give fluids to prevent crystalluria and stone formation; may cause Stevens-Johnson syndrome, toxic epidermal necrolysis, agranulocytosis, and aplastic anemia
Anti-inflammatory agents
These agents are used to decrease inflammation of blood vessels and to maintain adequate perfusion of skin and vital organs.
Methotrexate (Rheumatrex, Folex)
Antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction. Effects may also be mediated by adenosine via the inhibition of aminoimidazole carboxamide ribonucleotide (AICAR) transformylase, leading to increased release of adenosine.
Adjust dose gradually to attain satisfactory response.
Adult
0.3 mg/kg/wk PO/IM; not to exceed 20 mg/dose
Pediatric
0.3 mg/kg/wk PO/SC, titrate upwards to 1 mg/kg/wk; not to exceed 30 mg/dose (although higher doses up to 50 mg have been used over shorter treatment periods); alternatively, standard range is 10-20 mg/m2/wk
PO aminoglycosides may decrease absorption and blood levels of concurrent PO methotrexate (MTX); charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX; indomethacin and phenylbutazone can increase MTX plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity of MTX; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor CBC counts monthly and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occurs; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs, including salicylates, has not been tested)
More on Vasculitis and Thrombophlebitis |
| Overview: Vasculitis and Thrombophlebitis |
| Differential Diagnoses & Workup: Vasculitis and Thrombophlebitis |
 Treatment & Medication: Vasculitis and Thrombophlebitis |
| Follow-up: Vasculitis and Thrombophlebitis |
| Multimedia: Vasculitis and Thrombophlebitis |
| References |
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Further Reading
Keywords
vasculitis, thrombophlebitis, superficial venous thrombosis, Henoch-Schönlein purpura, Kawasaki disease, infantile polyarteritis nodosa, polyarteritis nodosa, Takayasu's arteritis, Takayasu arteritis, temporal arteritis, Wegener granulomatosis, treatment, diagnosis, symptoms
