The impact of gastroesophageal reflux disease symptoms in scleroderma: effects on sleep quality.

Systemic scleroderma/sclerosis (SSc) is an autoimmune connective tissue disease, which can lead to esophageal motor dysfunction and gastroesophageal reflux disease (GERD). Nocturnal GERD symptoms may be associated with sleep disturbances, which in turn can drastically affect well-being and fatigue levels. We hypothesized that GERD symptoms would be associated with poorer sleep in patients with SSc. Rheumatologist established SSc patients completed the following questionnaires: the UCLA scleroderma clinical trial consortium gastrointestinal tract instrument (GIT) 2.0 questionnaire; the Pittsburgh sleep quality index (PSQI); the fatigue severity scale (FSS); the multidimensional gastrointestinal symptom severity index (GSSI). Poor sleep quality was defined by a PSQI total score >5. Questionnaires were completed by 287 patients [mean (SD) age = 59 (14) years; female = 243]. Poor sleep quality was identified in 194 (68%) patients. Patients with poor sleep quality reported less sleep time and increased fatigue compared to those with normal sleep scores. SSc patients with poor sleep had significantly higher GIT Reflux scores (P < .001), and poor sleep was more frequent in those with moderate/severe versus mild/no heartburn on GISSI (P < .001). Narcotic and antidepressant use was significantly more frequent in SSc patients with poor sleep quality. Multivariable logistic regression supported the association between GERD symptoms and poor sleep after controlling for age, sex, and body mass index (BMI) (2.53, 95% confidence interval (CI) 1.52-4.25; P < .001). The association remained after controlling for narcotic and antidepressant use (2.20, 95% CI 1.29-3.73; P < .001). SSc patients who reported GERD symptoms were also more likely to report poor sleep quality. Future studies should examine mechanisms underlying nocturnal GERD symptoms in SSc patients, and the impact of improved GERD symptom control on sleep quality.

Associations of antibodies to native DNA with HLA-DRw3. A possible major histocompatibility complex-linked human immune response gene.

We examined the incidence of B lymphocyte (HLA-DRw) alloantigens in patients who exhibited elevated antibody titers to native DNA irrespective of their diagnosis. We found a statistically significant (P less than or equal to 0.0001) association between HLA-DRw3 and the presence of antibodies to native DNA not only in patients with a diagnosis of systemic lupus erythematosus but in other patients who did not share that diagnosis. This association supports the existence of a human immune response gene linked to the HLA complex. These data suggest that the hypothesis of an association between HLA and disease operating through disease susceptibility antigens or genes might be invalid and supports an alternative hypothesis, that HLA and disease associations are a manifestation of an immune response gene that controls the production of specific antibodies in any of several disease states.

Jaw claudication in primary systemic amyloidosis.

Amyloid deposits in the temporal artery were observed 40 years ago, but the presence of vascular ischemic symptoms in patients with amyloidosis has been infrequently recognized. We examined 22 patients who had typical jaw claudication with biopsy-proven primary amyloidosis. In none was vasculitis a contributing cause of the claudication. However, two patients were misdiagnosed initially as having temporal arteritis and polymyalgia rheumatica and were treated with corticosteroids, which resulted in significant toxicity. Subsequent temporal artery biopsy revealed extensive amyloid deposits in both patients. Jaw claudication was associated with other ischemic vascular symptoms, such as arm or calf claudication. The median survival for the subset of patients with amyloidosis and jaw claudication was 42 months, and that for the entire group of patients with amyloidosis was 12 months. Appropriate staining of temporal artery biopsy specimens is necessary for the correct diagnosis in such cases.

Histopathologic features of the L-tryptophan-related eosinophilia-myalgia (fasciitis) syndrome.

Study of 18 biopsy specimens in 11 patients with L-tryptophan-related eosinophiliamyalgia (fasciitis) syndrome showed hyaline sclerodermoid changes. Dermal scleroderma was found in eight of nine punch biopsy specimens and eight of nine excisional biopsy specimens. Fascial scleroderma was found in eight excisional biopsy specimens. One specimen obtained by excision had no fascia present. Eleven biopsy specimens showed edema of the dermis, and 13 showed dilated lymphatic structures; thus, the clinical picture of edematous sclerosis was confirmed. Mucinous fasciitis was present in five excisional biopsy specimens, in conjunction with a large number of macrophages in four. Dermal mucinosis was present in 11 biopsy specimens. Lymphocytic and macrophage inflammation was minimal in 14 biopsy specimens and pronounced in only 4. Plasma cells were present in eight cases. Eosinophils were present in substantial numbers in three biopsy specimens and only occasionally in four. Eosinophilic spongiosis was observed in one patient. Lymphocytic inflammation was noted around a single muscle spindle and around large nerve trunks in three patients. No relationship was established between these pathologic features and the duration or dose of tryptophan, prednisone treatment, or duration of symptoms. Pathologic features of the L-tryptophan syndrome consist of hyaline sclerodermoid collagen in the dermis, the septa, and the fascia. Edema, focal mucinosis, and macrophage inflammation may be features that identify this event.

Scleroderma and L-tryptophan: a possible explanation of the eosinophilia-myalgia syndrome.

Scleroderma developed in six women who were taking L-tryptophan. Fasciitis and morphea were most common, but one patient had pleural effusion, hypertension, and signs of cardiac and kidney failure. In five patients the biopsy findings were characteristic of scleroderma; the sixth patient had Crohn's disease and developed fasciitis; her biopsy specimen showed inflammatory arteritis. All patients' conditions improved after cessation of their L-tryptophan intake, initiation of corticosteroid therapy, or both. These findings confirm previous data that show altered tryptophan-kynurenine metabolism in some patients with scleroderma and fasciitis, particularly with tryptophan loading.

Whipple's arthritis: direct detection of Tropheryma whippelii in synovial fluid and tissue.

We describe 2 patients presenting with polyarthritis in whom the synovial fluid (1 patient) or synovial tissue (1 patient) was positive for Tropheryma whippelii, the Whipple's disease-associated bacillus, when examined by polymerase chain reaction (PCR) and DNA sequencing. Histopathologic findings were consistent with articular Whipple's disease in the synovial fluid of 1 patient and the synovial tissue of the other. In both patients, bowel mucosal specimens were negative for Whipple's disease features by histologic and PCR methods. One patient was positive for T whippelii in the peripheral blood. Control synovial fluid specimens from 40 patients with other arthritides, including Lyme arthritis, were negative. Sequencing of a 284-basepair region of the 16S ribosomal RNA gene confirmed that the sequence is closely related to the known T whippelii sequence. Both patients responded to treatment with antibiotics.

Polymyositis associated with jejunoileal bypass.

We report a patient with polymyositis who had a functioning jejunoileal bypass. Although many connective tissue syndromes have been reported with small bowel bypass, the association of polymyositis is unusual even though similar pathogenetic mechanisms might be involved.