ABSTRACT
OBJECTIVES: To study anal sphincter morphology, anal sphincter pressure, and rectoanal inhibitory reflex (RAIR) in patients with systemic sclerosis (SSc) complicated by anal incontinence (AI) and to investigate possible risk factors for AI in SSc. METHOD: Nineteen SSc patients with severe AI were investigated using anal endosonography, anal manometry, and rectal manovolumetry. To determine risk factors for AI, disease characteristics of SSc patients with AI were compared with those of 95 SSc patients without AI; there were five matched SSc patients without AI for each SSc patient with AI. RESULTS: The mean (SD) internal sphincter thickness was 1.3 (0.46) mm in patients with AI, which was thinner (p < 0.001) than reference data from healthy individuals whose internal sphincter measured 2.2 (0.45) mm, whereas the external sphincter thickness did not differ. The mean (SD) resting pressure in AI patients was lower than the reference data from healthy individuals [60 (22) vs. 94 (29) mmHg, p < 0.002] whereas the squeeze pressure did not differ. Centromeric antibodies and features of vascular disease [i.e. the presence of pulmonary arterial hypertension (PAH), digital ulcers, pitting scars, or the need for iloprost infusions] were associated with AI whereas fibrotic manifestations [i.e. modified Rodnan skin score (mRss), the diffuse cutaneous SSc (dcSSc) subset, or low vital capacity (VC)] were not. CONCLUSIONS: SSc patients with AI have a thin internal anal sphincter and a low resting pressure. Risk factors for AI among SSc patients are centromeric antibodies and vascular disease, which supports the hypothesis that gastrointestinal involvement in SSc is in part a vascular manifestation of the disease.
Subject(s)
Anal Canal/physiopathology , Familial Primary Pulmonary Hypertension/complications , Fecal Incontinence/epidemiology , Fecal Incontinence/physiopathology , Scleroderma, Systemic/complications , Ulcer/complications , Vascular Diseases/complications , Adult , Aged , Antibodies/blood , Case-Control Studies , Centromere/immunology , Comorbidity , Endosonography , Female , Fingers , Humans , Male , Manometry , Middle Aged , Rectum/physiopathology , Retrospective Studies , Risk Factors , Scleroderma, Systemic/physiopathologyABSTRACT
OBJECTIVE: Chronic renal disease other than scleroderma renal crisis (SRC) is not well documented in systemic sclerosis (SSc). We examined the occurrence of decreased glomerular filtration rate (GFR) in a large consecutive SSc cohort and analysed whether it was related to SSc or could be related to other causes. METHODS: During 1983-2004 GFR was measured by chromium-51-ethylenediaminetetraacetic acid (51Cr-EDTA) or iohexol clearance in 461 patients with SSc according to the American College of Rheumatology (ACR) criteria [356 with limited cutaneous SSc (lcSSc) and 105 with diffuse cutaneous SSc (dcSSc)] and the measurements were repeated once a year. Decreased GFR was defined as GFR<70% of the age-adjusted values. SRC was diagnosed in 4/360 lcSSc (1.1%) and in 10/115 dcSSc (8.7%). These patients were excluded from further analyses. RESULTS: At the latest follow-up at a median duration of 7.7 (range 0.5-54) years, decreased GFR was found in 39 lcSSc (11%) and nine (8.6%) dcSSc patients. Among the 48 SSc patients with GFR< 70p% (percentage of predicted value = p%), hypertension was diagnosed in 29 (60%) and cardiac involvement in 25 (52%). Different nephropathies were found in eight (19%) patients by renal biopsy. Fifteen patients with decreased GFR were followed up for > or = 4 years and no progress was seen in 11/15. CONCLUSIONS: A minority of patients with SSc develop renal dysfunction other than SRC. Decreased GFR was associated with other manifestations such as hypertension and cardiac involvement indicating possible pre-renal causes.
Subject(s)
Antibodies, Antinuclear/blood , Cause of Death , Glomerular Filtration Rate/physiology , Scleroderma, Systemic/mortality , Scleroderma, Systemic/physiopathology , Adult , Age Factors , Aged , Aged, 80 and over , Antibodies, Antinuclear/immunology , Cohort Studies , Disease Progression , Female , Follow-Up Studies , Humans , Kidney Function Tests , Male , Middle Aged , Probability , Reference Values , Retrospective Studies , Risk Assessment , Scleroderma, Systemic/complications , Severity of Illness Index , Sex Factors , Statistics, Nonparametric , Survival Analysis , Time Factors , Young AdultSubject(s)
Immunosuppressive Agents/therapeutic use , Myocarditis/etiology , Still's Disease, Adult-Onset/complications , Still's Disease, Adult-Onset/drug therapy , Adult , Fibrosis/pathology , Humans , Male , Methotrexate/therapeutic use , Myocarditis/pathology , Myocardium/pathology , Prednisolone/therapeutic useABSTRACT
OBJECTIVE: To examine plasma levels of calcitonin gene related peptide (p-CGRP) in patients with systemic sclerosis (SSc) and pulmonary hypertension (PH). MATERIAL AND METHODS: Twenty nine patients with SSc, 10 with diffuse form, 18 with limited form and one with overlapping systemic lupus erythematosus were examined. Twelve patients displayed normal systolic pulmonary artery pressure (PAPsyst) < or =30 mm Hg and 17 increased PAPsyst >30 mm Hg. Eight patients had isolated PH without interstitial lung disease (ILD) and nine had PH and ILD (secondary PH). PAPsyst was measured non-invasively by Doppler cardiography. CGRP was determined by radioimmunoassay. RESULTS: Patients with PH had higher p-CGRP than patients with normal pressure. A positive relation was found between p-CGRP and PAPsyst and between p-CGRP and erythrocyte sedimentation rate (ESR), particularly in patients with isolated PH. CONCLUSION: In patients with SSc p-CGRP correlates with pulmonary pressure and with ESR. Whether CGRP reflects disease activity or is released secondary to pulmonary vasoconstriction needs to be investigated further.
