Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease.

Objective: Systemic sclerosis (SSc) related mortality and morbidity remains high. Immunosuppressive therapy is considered most effective when immune activity and inflammation but not fibrosis still dominates the disease process. This study evaluated long-term intensified immunosuppression combined with therapeutic plasma exchange (TPE) in early-onset progressive SSc-related interstitial lung disease (ILD). Methods: The study cohort consisted of 161 SSc patients, with a median follow-up time of 8.9 years. The standardized mortality rate (SMR) and overall survival was calculated in patients with and without cardiopulmonary involvement. We used a standardized, pragmatic, non-randomized approach to treat 24 consecutive early progressive SSc-ILD patients with intensified immunosuppressive therapy, including plasma exchange. Outcome measurements were event-free survival (EFS), pulmonary function and safety profile. The outcome was compared with the analyzed data from the other SSc-ILD patients, who did not fulfill the inclusion criteria, and instead were treated with estimated optimal care (EOc). Results: The age-adjusted SMR of all 161 SSc patients was 3.0 (CI95%; 0.32-5.68). EFS at 10 years was 49.9% in the intensified treatment group and 43.3% in the EOc group (p = 0.106). Improvement of the percentage of predicted forced vital capacity (%pFVC) and percentage of predicted diffusing capacity for carbon monoxide (%pDLco) in the intensified treatment group was +10.1% respectively +3.6%, compared to a decrease of respectively 10.8% and 7% in the EOc (p < 0.001 resp. p = 0.019). Safety analysis showed 1 death (female patient, over 75 years of age), due to pneumosepsis, in the intensified treatment group. Conclusion: Intensified and long-lasting immunosuppression combined with TPE is safe in early severe systemic sclerosis and is associated with improved EFS and pulmonary function as compared to the outcome in the variable but EOc group. Our findings warrant larger studies for confirmation.

Schnitzler's syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström's disease.

Schnitzler's syndrome is an auto-inflammatory disorder which is characterized by two mandatory features: an urticarial rash and a monoclonal gammopathy. Although the pathophysiology of this syndrome is not yet fully understood, a role for interleukin-1 seems apparent. While this presumed link between interleukin-1 and the monoclonal gammopathy is not yet elucidated, a mutual factor in pathophysiology however seems likely. Here we present a novel hypothesis of a shared pathophysiologic mechanism between Schitzler's syndrome and monoclonal gammopathy.

[An aphasic man with high fever; difficulty with diagnostic procedure]. / Een afatische man met hoge koorts; moeilijkheden bij het stellen van de diagnose.

A 73-year-old man, with known motor aphasia presented with high fever, dyspnoea, and vomiting. Only after several days it appeared that these signs were due to a sepsis as a consequence of a phlegmon of the neck, caused by an aspired part of his set of false teeth. The delay before diagnosis was due to the fact that adequate medical history taking was difficult because of the aphasia and attempts at non-verbal communication were poorly understood. The localization of the corpus alienum was a second pitfall, because it could not be visualized during physical examination or on the initial chest X-ray. The increased risk of colonization of dentures with pathogenic micro-organisms in nursing home residents may have played a role in the severe course of this infection. This case illustrates the diagnostic problems that may exist in patients who are unable to communicate adequately.

Response shift in the measurement of quality of life in hearing impaired adults after hearing aid fitting.

UNLABELLED: REASON FOR THE STUDY: Response shift is the change in the meaning of one's self-evaluation of a target construct, like quality of life (QOL). The objective of this study was to investigate whether response shift in the measurement of generic and specific QOL occurred in persons with a relatively mild health condition. For this purpose hearing impairment was used as a research model. MAJOR FINDINGS: Response shift effects were observed in the scores on the dimensions of hearing related QOL. In the scores on overall hearing related QOL, and in the scores on the generic control items, no response shift occurred. CONCLUSIONS: This study showed that response shift effects can take place in a relatively mild condition as well. The occurrence of response shift in QOL ratings over time could have large implications for the estimation of the effectiveness of medical interventions and for the use of these estimations in cost-effectiveness analyses. After a successful treatment the conventional change could be an underestimation of the effectiveness of the treatment, although it has also been argued that psychological adaptation is a welcome capacity of human beings, and that then-test changes do no justice to this capacity.