Complications

Complication
Timeframe
Likelihood
short term
high

Can include sweating, mild abdominal pain, headache, nausea and gastrointestinal upset, urinary frequency, chills and influenza-like symptoms, rhinitis, flushing, increased lacrimation, and palpitations.[114]

Despite frequency of these side effects, they rarely appear to restrict patient compliance or therapy.[118]

long term
low

Forty times higher risk than that of the general population. Most have been marginal zone B-cell neoplasms arising in diverse extra-nodal and nodal sites.[11] Predictors of the development of lymphoma include: chronic antigenic stimulation by exoantigen or autoantigen; molecular oncogenic events such as microsatellite instability, loss of the B-cell cycle control, and the forced overproduction of specific B-cell biological stimulators; presence of palpable purpura, low C4, and mixed monoclonal cryoglobulinaemia.[121]

Cyclophosphamide pulse therapy rather than daily administration is recommended given the high frequency of lymphoma in Sjogren syndrome.[122]

Non-Hodgkin's lymphoma

long term
low

Presents as an enlarging mass, diagnosed on biopsy. Most non-life-threatening.

Mucosal associated lymphoid tissue (MALT) lymphoma is about 80 times more common among Sjogren syndrome patients than a matched population without the disease. Such lymphomas are effectively treated with low-dose oral methotrexate, and are rarely life-threatening. In the absence of a known cause for this severe/fatal complication, the use of methotrexate or even TNF-alpha antagonists to reduce hyperactivation of autoreactive B cells, when present, might be preventative treatment of lymphoproliferation. However, this in theory could favour other kinds of lymphoproliferation that do not have any common characteristic with SS-associated lymphomas.[123]

Non-Hodgkin's lymphoma

long term
low

Osteoporosis, diabetes, cardiovascular effects, and mood disruption are possible.

Sjogren syndrome patients have more problems with corticosteroid side effects than lupus patients. There is acceleration of periodontal disease and oral candidiasis.[11]

Hydroxychloroquine, methotrexate, and azathioprine are used to help taper the dose of corticosteroids.[11]

long term
low

Primary Sjogren syndrome has been associated with an increased risk of cerebrovascular events and myocardial infarction.[124][125][126]​​​​

Evidence suggests that the highest risk of cardiovascular events is found in patients with both anti-Ro/SSA and anti-La/SSB antibodies.[127]

variable
high

An inflammation of the meibomian sebaceous glands in the eyelid, resulting from dry eyes. Treatment consists of cleaning the eyelids, use of warm compresses, and treatment with an antibiotic.[77]

variable
high

Frequent exposure to preservatives in tear substitutes leads to toxicity to the ocular surface, and removal of the mucous layer. Use of methylcellulose inserts and lubricating ointment can cause blurring of vision.[77]

Tear substitutes with preservatives should not be used more than 4 times daily. Preservative-free tear substitutes can be used.

Methylcellulose inserts and lubricating ointment should be reserved for night-time use.

variable
medium

Topical antifungal treatment followed by systemic antifungal agents (for persistent or recurring episodes).

Wearing dentures overnight should be discouraged in order to prevent candidiasis. Dentures should be cleaned with chlorhexidine.

Individuals with the hyperplastic variant of Candida infection should have a biopsy of a mouth lesion undertaken, due to the risk of malignant transformation.

variable
medium

Symmetrical sensorimotor polyneuropathy, symmetrical sensory neuropathy, autonomic neuropathy, and cranial neuropathy can occur.

variable
medium

Anaemia of chronic disease is possible and may worsen fatigue; routine monitoring of Hb is indicated.

variable
medium

Complications of dry eyes.

variable
medium

The prevalence of depression in patients with primary Sjogren syndrome has been estimated at 33% to 38%.[119][120]​ This may be due to the stress of chronic illness or the effect of pro-inflammatory cytokines on neural pathways.[120]

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