Leprosy reactions in postelimination stage: the Bangladesh experience.

BACKGROUND: Leprosy reactions are immunologically mediated conditions and a major cause of disability before, during and after multidrug therapy (MDT). Little data have been published on the epidemiology of leprosy reactions in Bangladesh. OBJECTIVES: To describe the pattern and prevalence of leprosy reactions in the postelimination stage. METHODS: A descriptive retrospective cross-sectional study was carried out in Chittagong Medical College Hospital using the registered records of patients in the period between 2004 and 2013. RESULTS: Of the 670 patients with leprosy, 488 (73.38%) were males and 182 (27.37%) were females. The prevalence of reaction was in 300 (44.78%) patients with a male:female ratio of 3.55 : 1. The age-specific cumulative reaction cases at >40 years were 115 (38.33%) among all age groups. The prevalence of reaction was found to be in 166 (55.33%) patients for the reversal reaction, 49 (16.57%) for the erythema nodosum leprosum (ENL) and 85 (28.33%) for the neuritis. Borderline tuberculoid was most common (106, 35.33%)in the reversal reaction group, while lepromatous leprosy was most common (37, 12.33%) in ENL group. More than half of the patients (169, 56.33%) had reactions at the time of presentations, while 85 (28.33%) and 46 (15.33%) patients developed reaction during and after MDT, respectively. The reversal reaction group presented with ≥six skin lesions in 96 (57.83%) patients and ≥two nerve function impairments (NFIs) in 107 (64.46%) patients. The ENL was present chiefly as papulo-nodular lesions in 45 (91.84%) patients followed by pustule-necrotic lesions in four (8.16%), neuritis in 33 (67.35%), fever in 24 (48.98%), lymphadenitis in six (12.24%), arthritis in five (10.20%) and iritis in two (4.08%). Bacterial index ≥3 had been demonstrated in 34 (60.71%) patients in ENL group. CONCLUSION: The incidence of leprosy reaction seemed to be more than three times common in borderline tuberculoid (52.33%) group than in lepromatous leprosy (14%) group. Reactions with NFI and disability still occur among multibacillary patients during and after MDT. Early detection and management of leprosy reaction are very important in preventing disability and deformity, and patients should be educated to undergo regular follow-up examinations. Developing reinforced new therapies to curb leprosy reactions is crucial for improving leprosy healthcare services.

First reported case of intraocular Gnathostoma spinigerum in Cambodia.

A live intraocular nematode was identified from a 37 year-old man presented with iritis, pain, redness, lacrimation, swelling, vision loss and intermittent blindness during many hours per day of the left eye. By using slit lamp examination, a worm was removed from iris in an ophthalmology outpatient department setting and sent to the Medical Microbiology Laboratory, Institut Pasteur du Cambodge. Gnathostoma spinigerum was identified, based on its typical morphology via microscopic examination. Based on our diagnosis, the patient was treated by oral albendazole and responded well to this therapy.

Ocular syphilis--back again: understanding recent increases in the incidence of ocular syphilitic disease.

PURPOSE: The clinical findings and outcomes of 12 cases of luetic uveitis are reported. METHODS: Review of clinical records. RESULTS: Patients included 10 men and 2 women; 7 were homosexual, 9 HIV-positive. Six patients presented a medical history suggestive of syphilis. All patients presented with iritis and vitritis. Visual acuity improved in 11 patients after treatment. CONCLUSIONS: Syphilis has reemerged in developed countries. This may be related to the post-AID S/HAART era, with a growing pool of HIV-positive men who oftenly practice unsafe sex. We underscore the importance of a high index of suspicion of ocular syphilis in patients with these characteristics.

Concordance of disease severity among family members with ankylosing spondylitis?

OBJECTIVE: The heritability of disease activity and function in ankylosing spondylitis (AS) have been estimated at 0.51 and 0.63 (i.e., 51% and 63%), respectively. We examined the concordance of disease severity among family members in terms of disease activity, function, radiological change, prevalence of iritis, and juvenile onset. METHODS: Disease activity and functional impairment due to AS were studied using the Bath AS Disease Activity Index (BASDAI) and Functional Index (BASFI) self-administered questionnaires; radiographic involvement was measured using the Bath AS Radiology Index (BASRI) scale. Familial correlation of BASDAI and BASFI was assessed in 406 families with 2 or more cases, using the program PAP. Parent-child and sibling-sibling concordance for iritis and juvenile AS were also studied in these families. Heritability of radiological disease severity based on the BASRI was assessed in 29 families containing 60 affected individuals using the program SOLAR. RESULTS: Correlations between parent-child pairs for disease activity and function were 0.07 for both. Correlations between sibling pairs for disease activity and function were 0.27 and 0.36, respectively. The children of AS parents with iritis were more likely to develop iritis [27/71 (38%)] than children of non-iritis AS parents [13/70 (19%)] (p = 0.01). Parents with JAS were more likely to have children with JAS [17/30 (57%) compared to non-JAS parents 34/111 (30%)] (p = 0.002). The heritability of radiological disease severity based on the BASRI was 0.62. CONCLUSION: While correlation in severity between parent and child is poor, siblings do resemble each other in terms of severity, supporting the findings of segregation studies indicating significant genetic dominance in the heritable component of disease activity. Significant parent-child concordance for iritis and juvenile disease onset suggest that there are genetic risk factors for these traits independent of those determining the risk of AS itself. The finding of significant heritability of radiological change (BASRI) provides support using an objective measure for the observed heritability of the questionnaire-assessed disease severity scores, BASDAI and BASFI.

The interrelationship between sex, susceptibility factors, and outcome in ankylosing spondylitis and its associated disorders including inflammatory bowel disease, psoriasis, and iritis.

OBJECTIVE: To examine the evidence that families, where the mother has disease, carry more heritable factors and investigate the effect of maternal/paternal inheritance on phenotypic expression of disease in terms of (a) severity and outcome and (b) additional co-disorders. The children of women with ankylosing spondylitis (AS) develop the disease more often than the children of men. This suggests that either women with disease carry more susceptibility factors than men or that the uterine environment/breast feeding may play a role in AS. METHODS: The number of second degree relatives (i.e., grandparent, aunt/uncle) was calculated for those index patients with a mother with disease as opposed to a father. Outcome measures were compared and prevalence of secondary disorders (i.e., psoriasis, iritis, inflammatory bowel disease) was examined in patients with an AS mother as opposed to an AS father. RESULTS: The affected offspring of maternal cases had more second degree relatives with disease [20% vs 9%, respectively, p = 0.012, odds ratio (OR): 2.3, 95% confidence interval (CI): 1.2, 4.5] than did children of affected men. The affected children of a mother with AS were comparable in terms of disease activity, function, and radiology to children of a father with disease. Inflammatory bowel disease was more prevalent among children of AS mothers than AS fathers (15% vs 5%, respectively, p = 0.009, OR: 2.9, 95% CI: 1.3, 6.3). Psoriasis was less prevalent among sons of AS mothers than among sons of AS fathers (9% vs 22%, respectively, p = 0.03, OR: 0.4, 95% CI: 0.2, 0.9). CONCLUSION: The inherited susceptibility load is strongly linked to the sex of the parent with AS. Women with disease carry higher heritability (which is associated with inflammatory bowel disease) than do men. There is a male sex impact on susceptibility to psoriasis (when AS is present). However, there is no evidence that the susceptibility load has an effect on outcome or severity of disease (as measured by disease activity, function, and radiology), or that outcome is influenced by transmission of maternal as opposed to paternal factors.

Small-incision manual extracapsular cataract surgery in Ghana, West Africa.

PURPOSE: To evaluate the results of small-incision manual extracapsular cataract extraction surgery (ECCE) in a district hospital in West Africa. SETTING: Margret Marquart Catholic Hospital, Ghana, West Africa. METHODS: This prospective study consisted of 200 eyes of 193 patients who had small-incision manual ECCE between January 1999 and May 2000. For comparison, the charts of 32 patients (32 eyes) operated on between July and December 1998 using a limbal incision (control group) were retrospectively analyzed. Outcome measures included intraoperative and postoperative complications, postoperative visual acuity, and refractive astigmatism. RESULTS: In the small-incision ECCE group, self-sealing wounds were achieved in 129 eyes (64.5%). Vitreous loss occurred in approximately 3% of eyes in both the small-incision and control groups. The final visual acuities were similar between the 2 groups, with more than 90% of eyes in both groups achieving a final best corrected visual acuity of at least 20/60. Eyes in the small-incision group had faster visual recovery (P <.001), a lower incidence of fibrinous iritis (P =.02), and were more likely to have round pupils (P <.001) than eyes in the control group. The main complication of small-incision surgery was moderate corneal edema, which persisted until at least the 1-week visit in 14 eyes (7%). At the most recent visit, 1 eye in the small-incision group (0.5%) had bullous keratopathy. CONCLUSION: In a district hospital in West Africa, small-incision manual ECCE surgery yielded faster visual rehabilitation and had a lower incidence of fibrinous iritis than standard ECCE surgery.

Ankylosing spondylitis: interaction between genes, joints, age at onset, and disease expression.

OBJECTIVE: Ankylosing spondylitis (AS) is a chronic inflammatory disorder with symptom onset generally occurring in the late teens/mid-twenties. In women, a younger age at onset enhances disease susceptibility in the next generation. We examined the influence of age at symptom onset on phenotypic expression. METHODS: Patients were divided into cohorts according to age of symptom onset. The primary outcome measure was radiological progression (by Bath AS Radiology Index, BASRI). Secondary measures were disease activity (Bath AS Disease Activity Index, BASDAI), function (Bath AS Functional Index, BASFI), numbers undergoing AS related surgery, and percentage with secondary disorders. RESULTS: Age at onset had no significant effect on radiological progression (young onset vs late onset, 8.0, 8.6, respectively) disease activity (young vs late, 4.4, 4.4), need for non-hip surgical intervention (9%, 8%, respectively), or prevalence of secondary disorders (iritis, 40%, 41%; psoriasis, 20%, 19%; inflammatory bowel disease, 7.5%, 8.9%). By contrast, there was a striking increase in prevalence of total hip replacement in those with juvenile onset (18%, 8%, respectively; p < 0.001). Regardless of age at onset, spinal progression determined radiologically was greater in those with hip arthritis compared to those without (young onset hip involvement vs non-hip involvement, 9.7 (2.4), 7.2 (3.0) (p < 0.001); late onset hip involvement vs non-hip involvement, 10.1 (2.5), 7.1 (3.0), respectively]. Function deteriorates with age (young onset vs late onset, 3.7, 4.5, respectively; p < 0.01). CONCLUSION: (1) Hip disease (young or late onset) is a major prognostic marker for longterm severe disease (patients with hip disease have a spinal score increased by 2.5-3 points or 35-40% more change). (2) Hip involvement is more prevalent among patients with young age at onset. (3) Young onset patients without hip involvement do not have more severe disease. Thus, age at onset, itself, does not influence disease severity. (4) Since hip involvement and not age at onset is associated with worse outcome, patients with a young age at onset may be assumed to have an increased susceptibility load (i.e., genetic component or environmental trigger) rather than more severity genes. The lack of association between severity and age at onset implies that the determinants of susceptibility and severity are independent.

Inflammatory eye, skin, and bowel disease in spondyloarthritis: genetic, phenotypic, and environmental factors.

OBJECTIVE: To explore the nature of the interrelationship between inflammatory disease of the spine/joints, skin, eye, and bowel [i.e., ankylosing spondylitis (AS), psoriasis, iritis, inflammatory bowel disease (IBD)]. METHODS: The study used 4 approaches: (1) analysis of the prevalence of secondary disorders within the AS individual (chi-square and matched pair analysis); (2) study of the temporal relationship between the onset of the different conditions; (3) evaluation of the prevalence of disease among first degree relatives; and (4) influence of secondary disorders on outcome of AS. RESULTS: 1. Among 3287 patients with AS, more than expected had either spondylitis associated with multiple co-disorders or pure AS (with no co-diseases); fewer than expected had AS plus a single co-disease (chi-square = 32.2, p < 0.001). In a matched pair analysis, patients with AS and a secondary disorder were more likely to have an additional concomitant disease, e.g., IBD-AS (n = 335) patients had a higher prevalence of iritis [45.4% vs 36.7%; OR 1.4 (1.1-2.0)] or psoriasis [23.9% vs 14.3%; OR 1.9 (1.3-2.8)] than controls. 2. Among our database subjects, the symptomatic onset of the spinal disease precedes or is contemporaneous with gut, skin, and eye involvement (matched pair t test, p < 0.001). 3. Patients with multiple disorders predict the highest prevalence of co-diseases (i.e., psoriasis, IBD, iritis, or AS) within family members, followed by those AS patients with only IBD, psoriasis, or iritis in descending order. 4. Both psoriasis and IBD increase severity in terrms of function and disease activity of AS in the patient. Radiological change is greatest for those AS subjects with iritis. CONCLUSION: There is a striking overlap within patients and family members of rheumatological, dermatological, and gastroenterological diseases. The susceptibility genes of these co-disorders appear to overlap with each other and with AS: 1. A patient with 2 inflammatory conditions is at an increased risk of developing an additional related inflammatory disorder. 2. Those with enteropathic spondylarthritis would appear to carry the greatest genetic load in terms of first degree relatives developing inflammatory conditions (including psoriasis and iritis that are not seen in the index IBD-AS patient). 3. The secondary disorders do not precede AS (arguing against psoriasis and IBD allowing for an environmental conduit to pathogenic triggers in AS). The susceptibility factors for these inflammatory conditions may be additive or have a synergistic effect on each other. There is evidence for a shared gene hypothesis.

Immune-related systemic manifestations of inflammatory bowel disease. A prospective study of 792 patients.

The prevalence of immune-related systemic manifestations associated with inflammatory bowel disease varies widely in different studies. This prospective hospital study examines the prevalence and clinical aspects of these manifestations. A total of 792 patients, 449 with Crohn's disease and 343 with ulcerative colitis, were followed up from diagnosis to 20 years. Data related to systemic manifestations and to the extent and severity of intestinal lesions were obtained at diagnosis and during follow-up. Extraintestinal manifestations related to the activity of intestinal inflammation appeared at least once in 25.8% of the patients. The cumulative probability of extraintestinal manifestations increased from 12%-30% during the 20-year follow-up; it was significantly higher in patients with Crohn's disease (p < 0.01). Arthritis (p < 0.001) and erythema nodosum (p < 0.01) were more frequent in Crohn's disease. A significant positive association of arthritis with skin, eye, and mouth complications was observed (p < 0.001). Bowel-related systemic manifestations appeared frequently together in the same patient (p < 0.001). Patients with any of these manifestations ran a higher risk of developing another one. Autoimmune manifestations were associated with both Crohn's disease and ulcerative colitis.

Iritis in children with varicella.

A prospective study that included 82 consecutive children seen over 2 years by five pediatricians was undertaken to determine the incidence of iritis in children with chickenpox. Twenty-one (25%) children without pox on their lids were found to have iritis. Follow-up of children with objective evidence of iritis revealed no long-term sequelae.

Extracolonic diagnoses in ulcerative colitis: an epidemiological study.

In a population-based study of 1274 patients with ulcerative colitis (UC), the overall prevalence of extracolonic diagnoses was 21%. Seventy percent of patients with extracolonic diagnoses had extensive colitis. Patients without extracolonic diagnoses had only 28% extensive colitis (p less than 0.001), compared with 37% in the entire test population. The prevalence was higher in familial UC (p less than 0.05). The extracolonic diagnoses could be classified into two major groups, activity related and autoimmune, with regard to the extent and activity of UC and to the effects of medical and surgical treatment. Three hundred sixty-four diagnoses were distributed in 271 UC patients. It is concluded that extracolonic diagnoses are less frequent in an unselected patient group. However, extracolonic diagnoses are associated with the extent of UC at the time of diagnosis and with familial UC, and they can be classified as either activity related or autoimmune, with characteristic combinations of several extracolonic diagnoses occurring in 25% of the 271 patients.

Incidence and prevalence of juvenile chronic arthritis: a population survey.

In a population based epidemiological survey of juvenile chronic arthritis (JCA), performed in Western Sweden in 1983, an incidence of 12/100,000 was found. The estimated prevalence was 56/100,000. Subgroup distribution showed a preponderance of mono- and pauciarticular forms. The peak age of onset was between 0 and 4 years of age. Girls predominated over boys in a ratio of 3:2. Overall, 30% were antinuclear antibody (ANA) positive, 9% rheumatoid factor (RF) positive, and eye involvement occurred in 10% of the children. The results suggest differences in population based studies of JCA compared with previously reported hospital based series.

Studies on onchocerciasis in the United Cameroon Republic. IV. A four-year follow-up of six rain-forest and six savanna villages. The incidence of ocular lesions.

After an interval of four years the same observers re-examined six rain-forest and six savanna villages, using the same standardized techniques. The results of these surveys have previously been reported by area, i.e. rain-forest and savanna. The present paper analyses the incidence of ocular changes by village. In the rain-forest the concentrations of microfilariae in the skin were similar in the six villages and there was, in general, little difference in the incidence and/or deterioration of ocular lesions between these villages. In the savanna the corresponding quantities were much higher in the three more heavily infected villages compared with the three less heavily infected ones. The implications of these findings for the control of blindness due to onchocerciasis in the savanna are discussed.