Systemic Vasculitis

Patients with primary systemic vasculitis should be treated in collaboration with expert centers. Patients with antibody associated systemic vasculitis (AAV) should be categorized according to European Vasculitis Study Group recommendations. A combination of cyclophosphamide (IV or oral) and glucocorticoids should be used to induce remission of generalized or severe AAV; methotrexate and glucocorticoids may be used for remission induction of localized or early systemic AAV. In severe disease, plasma exchange will improve renal survival. Azathioprine or methotrexate is appropriate for maintenance of remission.

Primary Small Vessel Vasculitis

Long term regular follow-up is recommended for patients with prior exposure to cyclophosphamide, and with persistent unexplained hematuria.

Large/Giant Vessel Arteritis

High dose glucocorticoids should be used to induce remission. Visual symptoms of giant cell arteritis should be treated with a short course of high dose IV glucocorticoids. Patients with giant cell arteritis, with previous cardiac, visual or ischemic symptoms should be treated with low-dose aspirin.

Eye Disease

Azathioprine and systemic corticosteroids should be included in the treatment regimen for patients with inflammatory eye disease affecting the posterior segment. Severe eye disease is most appropriately treated with either cyclosporine A or infliximab combined with azathioprine and corticosteroids.

Vascular Disease

Immunosuppressive agents (eg, corticosteroids, azathioprine, cyclophosphamide, cyclosporine) are recommended for the management of acute deep vein thrombosis. For the management of both pulmonary and peripheral arterial aneurysms, cyclophosphamide and corticosteroids are recommended. There are no controlled data on, or evidence of benefit from uncontrolled experience with, anticoagulants, anti-platelet or anti-fibrinolytic agents in the management of deep vein thrombosis or for the use of anticoagulation for the arterial lesions of Behçet's disease (BD).

Gastrointestinal Involvement

There is no evidence-based treatment that can be recommended for the management of gastrointestinal involvement of BD. Agents such as sulfasalazine, corticosteroids, azathioprine, TNF antagonists, or thalidomide should be tried first, before proceeding with surgery, except in emergencies.

Central Nervous System (CNS) Involvement

Corticosteroids, interferon-alpha, azathioprine, cyclophosphamide, methotrexate, and TNF antagonists may be appropriate for parenchymal involvement. For dural sinus thrombosis corticosteroids are recommended. Cyclosporine should only be used in patients with CNS involvement if necessary for treatment of intraocular inflammation.

Skin and Mucosa

Mucocutaneous involvement should be treated according to the dominant or co-dominant lesions present. Topical measures (ie, local steroids) should be the first-line of treatment for isolated oral and genital ulcers. Acne-like lesions are usually of cosmetic concern only. Thus, topical measures as used in acne vulgaris are sufficient. Colchicine should be used when the dominant lesion is genital ulcer or erythema nodosum. Leg ulcers might have different causes. For these ulcers, azathioprine, interferon-alpha and TNF antagonists may be considered in resistant cases.

Patient Education

The adverse effects of glucocorticoid therapy should be considered and discussed with the patient before therapy is started.

Dosing

The glucocorticoid dose depends on the underlying rheumatic disease, disease activity, risk factors and individual patient response. Comorbidities and risk factors for adverse effects should be evaluated and treated when therapy is first initiated. For prolonged treatment, the glucocorticoid dosage should be kept to a minimum and a glucocorticoid taper should be attempted in the case of remission or low disease activity.

Monitoring

Patients should be monitored for body weight, blood pressure, peripheral edema, cardiac insufficiency, serum lipids, blood and/or urine glucose, and ocular pressure depending on the individual patient's risk, glucocorticoid dose, and duration.

Patients receiving prednisone ≥7.5 mg daily for >3 months should receive supplemental calcium and vitamin D. Antiresorptive therapy with bisphosphonates to reduce the risk of glucocorticoid-induced osteoporosis should be based on risk factors, including bone mineral density.

Patients treated with glucocorticoids and concomitant NSAIDs should be given appropriate gastro-protective medication (eg, proton pump inhibitors, misoprostol).

Special Conditions

Patients on glucocorticoid therapy for >1 month, who will undergo surgery, must receive perioperative management with adequate glucocorticoid replacement to overcome potential adrenal insufficiency. Glucocorticoids during pregnancy have no additional risk for mother and child. Children receiving glucocorticoids should be checked regularly for linear growth and considered for growth hormone replacement in the case of growth impairment.

Risk Factors

Risk factors for hand osteoarthritis (OA) include: female gender, increasing age (>40 years), menopausal status, family history, obesity, higher bone density, greater forearm muscle strength, joint laxity, prior hand injury, and occupation or recreation-related usage.

Symptoms

Typical symptoms are pain on usage and only mild morning or inactivity stiffness affecting just one or a few joints at any one time; symptoms are often intermittent and target characteristic sites (DIPJs, PIPJs, thumb-base, index and middle MCPJs).

Clinical Presentation

Clinical hallmarks include Heberden's and Bouchard's nodes, and/or bony enlargement with or without deformity affecting characteristic target joints (DIPJs, PIPJs, thumb-base, and index and middle MCPJs).

Associated Risks/Subsets

Patients with polyarticular OA of the hand are at increased risk of OA of the knee, hip, and other common target sites and should be assessed and examined accordingly. Recognised subsets with different risk factors, associations and outcomes (requiring different assessment and management) include IPJ (with or without nodes), thumb-base, and erosive OA.

Diagnosis

The differential diagnosis for OA of the hand is wide. The most common conditions to consider are psoriatic arthritis; rheumatoid arthritis, gout, and hemochromatosis. Radiographs provide the gold standard for morphological assessment of OA of the hand. Blood tests are not required for diagnosis but may be required to exclude co-existent disease.