Practical guidelines for the early diagnosis of Sjögren's syndrome in primary healthcare.

Primary care physicians can play a crucial role by recognising Sjögren's syndrome (SS) in the early stages identifying those patients with the greatest probability of being diagnosed with SS. SS has a very specific epidemiological profile at presentation (female aged 30-50 years), which may aid an early diagnosis. Although the disease may be expressed in many guises, there are three predominant clinical presentations that should be considered as key clues to increased clinical suspicion (multiple symptoms of dryness, asthenia-polyalgia syndrome and systemic organ-specific manifestations). The physical examination may provide important clues to systemic involvement (parotid gland enlargement, skin lesions suggestive of purpura or annular erythema, respiratory crackles, arthritis, neurological sensory or motor deficits). Simple laboratory studies may be very useful in reinforcing the clinical suspicion of SS, and the triad of cytopenia, raised erythrocyte sedimentation rate and high serum gamma globulin levels is a very specific "biological" pattern suggesting SS. A solid clinical suspicion of SS requires both the patient reporting sicca symptoms and objective evidence that these symptoms are associated with dysfunction of the lachrymal and salivary glands. Ultrasonography of the parotid glands, a non-invasive method, may be a major advance in the diagnostic approach to SS in primary care. Primary care physicians must be considered essential members of the multidisciplinary team in charge of the follow-up of SS patients, due to their key role in the continuum of patient care and their cross-sectional knowledge of common diseases that frequently coexist in patients with SS.

[Correspondence between primary and secondary healthcare providers about patients with cancer; how can it be improved?] / Correspondentie tussen eerste en tweede lijn over patiënten met kanker.

OBJECTIVE: To explore the correspondence between primary and secondary healthcare providers about patients with lung, breast or colorectal cancer. DESIGN: Qualitative research. METHOD: We collected the medical files of 50 patients with lung, breast or colorectal cancer by purposive sampling and selected the correspondence-related items from them. These concerned referral letters from primary to secondary caregivers and letters from specialists. A qualitative content analysis of these documents was performed. In addition, 4 general practitioners, 4 oncologists and 1 nurse specialist were interviewed. RESULTS: We analysed 50 referral letters and 369 letters from specialists. Content could be divided into 6 main themes in the referral letters, and it was noticeable that highly relevant information regarding the past medical history was often mixed with less relevant information. The same was true for the medication list and case history to a certain extent. We could distinguish 9 themes in the letters from specialists. All the letters from specialists did include information about the current treatment, but information about treatment intent (curative or palliative) or alternative treatment options was rarely available. Interviews with the healthcare providers confirmed these findings. CONCLUSION: The study findings indicate that referral letters and specialist correspondence are not sufficiently tailored to the needs of the recipient.

Correspondence between primary and secondary care about patients with cancer: A qualitative mixed-methods analysis.

Cancer care is complex and involves many different healthcare providers, especially during diagnosis and initial treatment, and it has been reported that both general practitioners and oncology specialists experience difficulties with interdisciplinary communication. The aim of this qualitative study was to explore information sharing between primary and secondary care for patients with lung, breast or colorectal cancer. A qualitative content analysis of 50 medical files (419 documents) was performed, which identified 70 correspondence-related items. Six main topics were identified in most referral letters from primary to secondary care, but it was particularly notable that highly relevant information regarding the past medical history was often mixed with less relevant information. To lesser extents, the same held true for the medication list and presenting history. In the letters from specialists, nine topics were identified in most letters. Although information about actual treatment was always present, only limited detail, if any, was given about the intent of the treatment (curative or palliative) or the treatment alternatives. Interviews with nine healthcare providers confirmed these issues. These findings indicate that neither the initial referral nor the specialist correspondence is tailored to the needs of the recipient.

Health-related quality of life, employment and disability in patients with Sjogren's syndrome.

OBJECTIVE: To compare health-related quality of life (HR-QOL), employment and disability of primary and secondary SS (pSS and sSS, respectively) patients with the general Dutch population. METHODS: HR-QOL, employment and disability were assessed in SS patients regularly attending the University Medical Center Groningen (n = 235). HR-QOL, employment and disability were evaluated with the Short Form-36 questionnaire (SF-36) and an employment and disability questionnaire. Results were compared with Dutch population data (matched for sex and age). Demographical and clinical data associated with HR-QOL, employment and disability were assessed. RESULTS: Response rate was 83%. SS patients scored lower on HR-QOL than the general Dutch population. sSS patients scored lower on physical functioning, bodily pain and general health than pSS patients. Predictors for reduced HR-QOL were fatigue, tendomyalgia, articular involvement, use of artificial saliva, use of anti-depressants, comorbidity, male sex and eligibility for disability compensation (DC). Employment was lower and DC rates were higher in SS patients compared with the Dutch population. CONCLUSION: SS has a large impact on HR-QOL, employment and disability.

Sjögren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity?

OBJECTIVE: To report 8 patients with Sjögren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases. METHODS: The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated. RESULTS: Among the 8 patients with a clinical diagnosis of SS, 6 fulfilled the American-European Consensus Group criteria for SS. All of the patients were women in whom SS had been diagnosed at a median age of 47 years (range 30-61 years) and amyloid had been diagnosed at a median age of 60 years (range 42-79 years). The presence of the immunoglobulin light chain type of amyloid (AL amyloid) was confirmed in 4 patients. In 3 of these 4 patients as well as 2 other patients, a light chain-restricted plasma cell population was observed near the amyloid deposits. Progression to systemic amyloidosis was not observed in any patient during a median followup of 3.5 years. CONCLUSION: SS should be considered in patients with cutaneous amyloidosis. The combination of cutaneous amyloidosis and SS appears to be a distinct disease entity reflecting a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS.

The future of biologic agents in the treatment of Sjögren's syndrome.

The gain in knowledge regarding the cellular mechanisms of T and B lymphocyte activity in the pathogenesis of Sjögren's syndrome (SS) and the current availability of various biological agents (anti-TNF-alpha, IFN- alpha, anti-CD20, and anti-CD22) have resulted in new strategies for therapeutic intervention. In SS, various phase I and II studies have been performed to evaluate these new strategies. Currently, B cell-directed therapies seem to be more promising than T cell-related therapies. However, large, randomized, placebo-controlled clinical trials are needed to confirm the promising results of these early studies. When performing these trials, special attention has to be paid to prevent the occasional occurrence of the severe side effects.