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Vasculitis in Sjogren’s Syndrome

Posted on Fri, Mar 30, 2012

Vasculitis is considered to be a significant extraglandular manifestation of Sjögren’s syndrome (SS). While a specific type of vasculitis has not been universally associated with SS, several sub-types of vasculitis occur with considerable frequency in SS. These vasculitic sub-types have prognostic and therapeutic implications. This discussion is limited to the association of vasculitis with primary SS. Vasculitis, while not infrequently observed in secondary SS, often occurs via its association with the associated rheumatic disease (i.e. SLE).

More than 50 years ago, classification of vasculitic disorders was based on the caliber of vessels involved, but since that time, investigators discovered the relationship between immunological processes and specific kinds of vasculitis, and this has changed our way of thinking about and defining sub-types of vasculitis. However, terminology can remain in use long after its effectiveness, resulting in variable reports of vasculitis complicating SS.

The majority of vasculitic syndromes described in SS involve small vessels. A list of terms used to describe vasculitic syndromes complicating primary SS is shown in Table 1.

Table 1
Small and Medium Vessel Vasculitic
Syndromes Associated with SS

Small vessel

Benign hyperglobulinemic purpura of Waldenstrom


Cryoglobulinemic vasculitis

Hypergammaglobulinemic purpura (non-inflammatory)

Leukocytoclastic angiitis

Lymphocytic vasculitis

Mononuclear inflammatory vascular disease

Neutrophil inflammatory vascular disease

Palpable purpura

Schaumberg’s disease

Serum sickness vasculitis

Urticarial vasculitis


Medium Vessel

Granulomatous arteritis

Mononeuritis multiplex

Necrotizing vasculitis

Systemic necrotizing vasculitis

Management of vasculitis associated with SS

Hyperglobulinemic purpura may be managed using symptomatic therapy. Avoidance of tight fitting elastic on the lower extremities, elimination of long periods of standing and elevation of one’s legs while sitting are all helpful in reducing elevated hydrostatic pressure. Judicious use of topical corticosteroids (i.e. 1% hydrocortisone and others) may relieve itching as may the newer, non-sedating antihistamines which may be less drying than the traditional antihistamines. Mild to moderate cutaneous vasculitis may respond to a variety of agents including NSAIDs, colchicine, dapsone, hydroxychloroquine and methotrexate. Short courses of oral corticosteroid (prednisone) may be required.

The onset of numbness or other changes in sensation, pain and/or weakness of the extremities may be indicative of a more severe vasculitis involving the peripheral nerves and or muscles. In some cases, medium caliber vessels may be involved and other organs or systems may be affected as well, including the kidney, GI tract and central nervous system. These more severe forms of vasculitis require treatment with moderate to high doses of corticosteroids and immunosuppressive agents such as azathioprine and cyclophosphamide. Intravenous immunoglobulin has been successful in patients with peripheral neuropathy secondary to vasculitis. Rituximab has recently demonstrated efficacy for systemic necrotizing vasculitis and is under investigation for the treatment of extraglandular manifestations of SS. B cell depletion (rituximab therapy) is an attractive option for the management of SS particularly when vasculitis is associated with responsive forms of B cell NHL.

Information on Vasculitis in Sjögren’s Syndrome excerpted from an article by Steven C. Carsons, MD, Winthrop University Hospital, Vol. 28, Issue 2, The Moisture Seekers, published by the Sjögren’s Syndrome Foundation.

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