First-degree Family Members Of Patients Research Articles

The Investigation of Death Anxiety and Spiritual Well-Being Levels of Family Members of Patients Admitted to Intensive Care Unit.

This study aimed to investigate the death anxiety (DA) and spiritual well-being (SWB) levels of first-degree family members of patients hospitalized in the intensive care unit (ICU). The data of this descriptive correlational type of study were collected through a faceto-face interview and survey with 308 family members who came to visit family members treated in the ICU of a public hospital in the western province of Turkey. Results were analyzed with SPSS software version 22. The average Death Anxiety Scale (DAS) score of the family members is 7.99 (3.15), which is above the middle value (min/max; 0-15), and the average Spiritual Well-Being Scale (SWBS) score is 121.83 (12.91), which is relatively high (min/max; 29-145). A positive, weak, and significant correlation existed between DAS and SWBS mean scores (r=0.20; P<0.05). As a result, the DA levels of family members increase with the thought of losing their patients in the ICU. During this period, family members of patients need spirituality more than ever to cope with increasing DA. In this study, a positive and significant correlation was found between the DA levels and SWB levels of the family members. According to this result, as family members' DA increases, their spiritual needs also increase.

Optimizing Screening for Early Disease Detection in Familial Pulmonary Fibrosis (FLORIS): A Prospective Cohort Study Design.

Background: Familial pulmonary fibrosis (FPF) can be defined as pulmonary fibrosis in two or more first-degree family members. The first-degree family members of FPF patients are at high risk of developing FPF and are eligible for screening. Reproducible studies investigating risk factors for disease are much needed. Methods: Description of the screening study protocol for a single-center, prospective cohort study; the study will include 200 asymptomatic, first-degree family members of patients with FPF who will undergo three study visits in two years. The primary objective is determining the diagnostic value of parameters for detection of early FPF; the secondary objectives are determining the optimal timing of the screening interval and gaining insight into the natural history of early FPF. The presence of interstitial lung disease (ILD) changes on high-resolution computed tomography of the chest is indicative of preclinical ILD; the changes are determined at baseline. The comparison between the group with and without ILD changes is made for clinical parameters (pulmonary function, presence of digital clubbing, presence of Velcro-like crackles, blood count, liver- and kidney-function testing, patient-reported cough and dyspnea score) and exploratory parameters. Discussion: This study will be the first large-size, prospective, longitudinal cohort study for yearly screening of asymptomatic family members of FPF patients investigating the diagnostic value of parameters, including lung function, to detect early FPF. More effective screening strategies could advance early disease detection.

A Multi-diagnostic Screening Study of the Prevalence and Risk Factors of Echinococcosis in First-degree Family Members of Infected Patients Undergoing Surgical Treatment

Background: Echinococcosis is a zoonotic infection caused by Echinococcus tapeworms. About 60% of patients remain asymptotic until severe stages. Early diagnosis of the infection in high-risk populations is of medical importance. Objectives: This study was designed to investigate the prevalence of echinococcosis among first-degree family members of the infected patients who underwent surgical treatment. Methods: A cross-sectional study was conducted to screen the first-degree family members of 96 patients who underwent surgical intervention at Imam Reza hospital, Mashhad, Iran, from September 2016 to March 2017. All family members were invited by phone calls to perform the serologic test, ultrasound examination, and chest X-ray test. Results: Forty-six (47.9%) patients participated in the study. A total of 114 family members performed the screening tests. Seven participants from five families (6.14%) were found to be infected in ultrasound examination (five females with a mean age of 36 years). There were no significant differences between the infected and non-infected groups concerning age (P = 0.921) and gender (P = 0.457). Conclusions: Screening the first-degree family members of patients with a confirmed echinococcosis infection results in the early diagnosis of infected patients, which is economically significant, especially in endemic areas.

Causes Of Desertion Of Diabetes Preventive Programs Aimed At Relatives Of Patients With Type 2 Diabetes

Purpose: First-degree family members of patients with type 2 diabetes have a higher risk of developing diabetes. Attendance in a diabetes prevention program is essential to implement prevention strategies. However, many relatives drop out. Identifying the causes behind the program abandonment is necessary to improve attendance adherence. Methods: We reviewed six different databases for causes of treatment abandonment in prevention programs for type 2 diabetes published between 1980 and 2020. Eleven articles met inclusion criteria and varied in sample size, ethnicity, gender, age of the participating patients, and length of follow-up. Causes of abandonment were categorized into social, biological, lifestyle, and emotional. Results: Sample sizes of the selected studies ranged from 143 to 128,520 subjects. The main barriers were age, economic difficulties, ignorance about risk factors, family history of diabetes, living in public housing, marital status, comorbidities, and low consumption of fruits and vegetables. Physicians' attitudes toward prediabetes screening were also a barrier. Conclusion: People at risk of developing diabetes face multiple barriers to preventing the development of this disease. During the research, the variety of reasons causing dropouts in prevention programs was great and diverse.

The Effects of Family Psychoeducation Courses on Depression, Anxiety and Family Functioning in the Caregivers of Schizophrenic Patients

Background: Schizophrenia is one of the most serious psychiatric diseases that affects the patient’s family members in addition to the patient himself. This disease can lead to depression and anxiety in the family members of the patient and even affect their functioning. Objectives: The present study was conducted to investigate the effect of a short-term family psychoeducation course on the depression and anxiety family functioning in caregivers Methods: In this before-after clinical trial, 163 first-degree family members of patients with schizophrenia were invited to participate in a short-term FPE course. A total of 65 of this group attended the first training session. Prior to the first session, the beck depression inventory (BDI), the beck anxiety inventory (BAI), and the family assessment device (FAD) were completed for the participants based on a family functioning model. Six months after the end of the FPE course, the 36 subjects who had completed the initial questionnaires and fully attended the sessions were invited to complete the questionnaires again. A total of 31 subjects completed the questionnaires again at this stage. The data were analyzed by SPSS-20 software using Mann–Whitney and Wilcoxon rank tests. Results: The mean age of the participants in both stages of questionnaire completion was 48.64 ± 11.85 years. Among them, 16 (51.6%) were female, and 15 (48.4%) were male. Also, six (19.4%) subjects had an education above high school, and mothers were the most frequent participating family members with a frequency of 11 (35.5%). The anxiety and depression scores of these subjects were 10.8 ± 5.14 and 6.9 ± 2.45 at baseline, respectively. These scores decreased significantly after six months to 5.03 ± 2.48 and 4.40 ± 1.9, respectively. In addition, among family functioning, Role and Behavioral control was significantly improved (P-value &lt; 0.05). Conclusions: The results of this study showed that depression and anxiety levels decreased in the family members of the examined patients six months after an FPE course. This effect can be further investigated through studies conducted with control groups. These findings suggest that the integration and institutionalization of FPE programs in the healthcare system are essential for improving the status of patient caregivers with severe psychiatric illnesses and their families.

Diagnosis, family screening, and treatment of inherited arrhythmogenic diseases in Europe: results of the European Heart Rhythm Association Survey.

The spectrum of inherited arrhythmogenic diseases (IADs) includes disorders without overt structural abnormalities (i.e. primary inherited arrhythmia syndromes) and structural heart diseases (i.e. arrhythmogenic ventricular cardiomyopathy, hypertrophic cardiomyopathy). The aim of this European Heart Rhythm Association (EHRA) survey was to evaluate current clinical practice and adherence to 2015 European Society of Cardiology Guidelines regarding the management of patients with IADs. A 24-item centre-based online questionnaire was presented to the EHRA Research Network Centres and the European Cardiac Arrhythmia Genetics Focus Group members. There were 46 responses from 20 different countries. The survey revealed that 37% of centres did not have any dedicated unit focusing on patients with IADs. Provocative drug challenges were widely used to rule-out Brugada syndrome (BrS) (91% of centres), while they were used in a minority of centres during the diagnostic assessment of long-QT syndrome (11%), early repolarization syndrome (12%), or catecholaminergic polymorphic ventricular tachycardia (18%). While all centres advised family clinical screening with electrocardiograms for all first-degree family members of patients with IADs, genetic testing was advised in family members of probands with positive genetic testing by 33% of centres. Sudden cardiac death risk stratification was straightforward and in line with current guidelines for hypertrophic cardiomyopathy, while it was controversial for other diseases (i.e. BrS). Finally, indications for ventricular mapping and ablation procedures in BrS were variable and not in agreement with current guidelines in up to 54% of centres.

Experience and Outcome of Patients with Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) over 8 Years

Introduction: NLPHL is an uncommon variant of Hodgkin lymphoma (HL) accounting for 5% incidence of all HL diagnoses. NLPHL represents a more indolent disease than classical HL, and is therefore managed uniquely. Unlike classical Hodgkin lymphoma (cHL), NLPHL shows CD20 immunoreactivity. Given the rarity of the disease, optimal management is unclear.Here we present our retrospective clinical experience of nine cases of NLPHL diagnosed over the last 8 years at our institution.Results: The median age at diagnosis was 45 years. Eight of the nine patients were males. The youngest patient was 21 years old and the oldest patient at diagnosis was 58 years old. One patient had Stage I, six had stage II and two patients had Stage-III disease. No patient had bone marrow involvement and only two patients had B symptoms. Interestingly, two sets of brothers diagnosed at different intervals were identified among this group of patients.Three out of seven patients with Stage I/II disease received radiation as the primary modality of treatment. Four patients received chemotherapy only and two patients received combined modality treatment( chemotherapy followed by radiation). Only the youngest patient received R-CVP (rituximab, cyclophosphamide, vincristine, and prednisone) to avoid anthracycline related long term toxicity. A total of four patient received R-CHOP, two of these also received involved site radiation therapy (ISRT).One patient received six cycles of AVD (doxorubicin, vincristine and dacarbazine).All the patients achieved complete response (CR) and durable remission except one patient. That particular patient had stage III NLPHL at diagnosis and was treated with R-CHOP initially. Three years later on relapse, he had T-cell rich large B cell lymphoma (TCRBCL) and is currently on a clinical trial with pembrolizumab and remains in complete remission.Conclusions: As has been the experience with other institutions, the majority of the cases of NLPHL at our institution have presented with early stage disease. Treatments have included R-CHOP regimens in most of the cases with durable remission in the majority. Although R-CVP is not the standard regimen for NLPHL, the one young patient who had stage III disease at diagnosis and received R-CVP, achieved CR and remains disease free five years later. This highlights the importance of non-anthracycline chemotherapy regimens as an option for particularly young or older frail patients.NLPHL occurs with higher than expected frequency among first-degree family members of patients with NLPHL as demonstrated in the Finnish Cancer Registry study reported in JCO 2013. Interestingly, in our small cohort, there were two sets of brothers identified with NLPHL, diagnosed metachronously. Unlike cHL, late relapse can occur as well as a propensity to transform to an aggressive B-cell Non-Hodgkin lymphoma. DisclosuresNo relevant conflicts of interest to declare.

Serrated polyposis syndrome: Diagnosis and management

Serrated polyposis syndrome is a clinical diagnosis characterized by a few large right-sided colorectal serrated polyps, multiple small serrated polyps, or a combination of both. Colorectal polyps and cancers develop via the methylator (or serrated) carcinogenic pathway. Patients with SPS have an increased risk of CRC compared to the general population throughout their lifetime and adequate surveillance and intervention are necessary. Cancer develops mainly via serrated polyp intermediates, but adenomas can also be present. The goals of management are to prevent the development of colorectal cancer and death from colorectal cancers. Colonoscopy beginning at age 40 at the latest, with polypectomy is the mainstay of clinical management. Surgery is utilized to treat colorectal cancer or decrease its risk. First-degree family members of patients with SPS are also at increased risk of colorectal cancer compared to the general population and increased colonoscopic screening is indicated.

The immunophenotypic fingerprint of patients with primary antibody deficiencies is partially present in their asymptomatic first-degree relatives.

The etiology of primary antibody deficiencies is largely unknown. Beside rare monogenic forms, the majority of cases seem to have a more complex genetic basis. Whereas common variable immunodeficiency has been investigated in depth, there are only a few reports on milder primary antibody deficiencies such as idiopathic primary hypogammaglobulinemia and IgG subclass deficiency. We performed flow cytometric immunophenotyping in 33 patients with common variable immunodeficiency, 23 with idiopathic primary hypogammaglobulinemia and 21 with IgG subclass deficiency, as well as in 47 asymptomatic first-degree family members of patients and 101 unrelated healthy controls. All three groups of patients showed decreased memory B- and naïve T-cell subsets and decreased B-cell activating factor receptor expression. In contrast, circulating follicular helper T-cell frequency and expression of inducible T-cell co-stimulator and chemokine receptors were only significantly altered in patients with common variable immunodeficiency. Asymptomatic first-degree family members of patients demonstrated similar, albeit intermediate, alterations in naïve and memory B- and T-cell subsets. About 13% of asymptomatic relatives had an abnormal peripheral B-cell composition. Furthermore, asymptomatic relatives showed decreased levels of CD4+ recent thymic emigrants and increased central memory T cells. Serum IgG and IgM levels were also significantly lower in asymptomatic relatives than in healthy controls. We conclude that, in our cohort, the immunophenotypic landscape of primary antibody deficiencies comprises a spectrum, in which some alterations are shared between all primary antibody deficiencies whereas others are only associated with common variable immunodeficiency. Importantly, asymptomatic first-degree family members of patients were found to have an intermediate phenotype for peripheral B- and T-cell subsets.

EPA-1769 – Imaging genetic risk for schizophrenia

The genetic risk for schizophrenia is estimated to range between 60% and 80%. These estimates are based on family-studies. Patients with schizophrenia have changes in brain structure and function, Since brain structure and function are considerably heritable and include genes implicated in schizophrenia, these measures are interesting to provide phenotypic characterization of risk variant carriers. Indeed, it was found that first-degree family members of patients share some of the gray matter changes with the probands, and in particular those with increased genetic burdening such as in discordant twins of patients (Moran et al, 2013). A study in monozygotic and dizygotic twins discordant for schizophrenia revealed that local cortical thickness changes as well as smaller white matter volume in schizophrenia are for a considerable part heritable, including genes overlapping with the genetic risk for the disease (Hulshoff Pol et al, 2012). To determine whether the smaller white matter volume as a candidate endophenotype for schizophrenia reflects changes in white matter microstructure and network properties is an important next step. Brain measures may provide clinically relevant risk scores in the future. The feasibility to use a classification model based on magnetic resonance brain images (Schnack et al, in preparation), and to include family related contextual information (Mandl et al, 2013) to measure risk for schizophrenia will be discussed.

Clinical, immunologic, and genetic features of familial systemic sclerosis

To examine whether the affected first-degree relatives within multicase systemic sclerosis (SSc; scleroderma) families are concordant for autoantibody profile, disease type, and HLA class II haplotypes and whether clinical expression and serologic characteristics of familial SSc differ from those of sporadic SSc. Seven hundred ten SSc families from the Scleroderma Family Registry and DNA Repository (Scleroderma Registry) were examined, and 18 multicase families were identified. SSc cases and their first-degree family members underwent serologic testing for different autoantibodies. The disease type and various disease features were abstracted from the available medical records. Additionally, HLA class II typing was performed on the multicase SSc sibpairs. The observed SSc-specific antibody concordance within each multicase SSc family was statistically more common than expected by chance alone (P = 0.007). The autoantibody profile and disease features of familial and sporadic SSc cases did not differ significantly. The frequency of autoantibody positivity was not different between unaffected first-degree family members of patients from multicase versus singleton SSc families. HLA haplotype sharing between SSc sibpairs was significantly more common than expected (P = 0.011). The affected members within multicase SSc families tend to be concordant for SSc-specific autoantibodies and HLA haplotypes; familial SSc does not appear to be a unique disease subset.

A prospective cohort study on the absolute incidence of venous thromboembolism and arterial cardiovascular disease in asymptomatic carriers of the prothrombin 20210A mutation

AbstractThe prothrombin 20210A mutation has been associated with an increased risk for venous thromboembolism (VTE) and arterial cardiovascular disease. The risks for asymptomatic carriers of this mutation have thus far been studied only in case-control and retrospective cohort studies. Here we present the results of the first prospective observational study in asymptomatic first-degree family members of patients with either VTE or premature atherosclerosis and the prothrombin 20210A mutation. We included 464 individuals (236 carriers) with a total follow-up duration of 1816 years (943 years for the carriers). The annual incidence of a first VTE was 0.37% (95% CI, 0.08-1.08) for carriers and 0.12% (95% CI, 0.00-0.69) for noncarriers (HR, 3.1; 95% CI, 0.3-29.6). The annual incidence of a first arterial cardiovascular event was 0.56% (95% CI, 0.18-1.31) for carriers and 0.73% (95% CI, 0.27-1.58) for noncarriers (adjusted HR, 0.7; 95% CI, 0.2-2.5). We conclude that the absolute incidence of a first VTE or arterial cardiovascular event is low; therefore, the clinical implications of carriership of the prothrombin 20210A mutation are limited, and routinely testing all first-degree relatives of probands with this mutation does not appear to be justified.

The Complex Genetics of Common Variable Immunodeficiency

Immunoglobulin (lg)A deficiency and common variable immunodeficiency (CVID) are the most common primary immunodeficiency disorders in North America and Europe. These diseases appear to comprise a familial spectrum of immunodeficiency that ranges from partial IgA deficiency to a complete absence of serum immunoglobulin. The CVID phenotype is typically acquired and can spontaneously revert to IgG and IgM sufficiency. Family studies suggest the presence of at least two susceptibility loci within the major histocompatibility complex on the short arm of chromosome 6: one located near the class II region and the other located near the junction between the class III and class I regions. Inheritance of these susceptibility genes may yield an additive risk for the development of immunodeficiency. First-degree family members of patients with CVID are at risk throughout their lives for the development of these diseases and should be monitored with a high index of suspicion.

The complex genetics of common variable immunodeficiency.

Immunoglobulin (Ig)A deficiency and common variable immunodeficiency (CVID) are the most common primary immunodeficiency disorders in North America and Europe. These diseases appear to comprise a familial spectrum of immunodeficiency that ranges from partial IgA deficiency to a complete absence of serum immunoglobulin. The CVID phenotype is typically acquired and can spontaneously revert to IgG and IgM sufficiency. Family studies suggest the presence of at least two susceptibility loci within the major histocompatibility complex on the short arm of chromosome 6: one located near the class II region and the other located near the junction between the class III and class I regions. Inheritance of these susceptibility genes may yield an additive risk for the development of immunodeficiency. First-degree family members of patients with CVID are at risk throughout their lives for the development of these diseases and should be monitored with a high index of suspicion.

Family members of patients with selective IgA deficiency should be routinely screened for primary immunodeficiency

Rationale Although the pathogenesis of selective IgA deficiency (SIgAD) is poorly understood, inheritance patterns suggest it is autosomal dominant. Despite this fact, first-degree family members of patients diagnosed with SIgAD are not routinely screened for primary immunodeficiency, due in part to lack of symptomatology. We wish to present a case in which two asymptomatic adult children of a 45 year old white female with SIgAD were screened and found to have both IgA deficiency and anti-IgA antibodies. Methods Bloodwork was first performed on the mother, and later on her children, and consisted of quantitative total serum Ig levels (IgM, IgG, IgA), serum IgE, IgG subtypes, anti-IgA antibody levels, humoral immune panel (diphtheria antitoxoid antibody, tetanus antitoxoid antibody, and a 6 serotype pneumococcal antibody panel), and a CBC with WBC count differential. Results The mother's bloodwork revealed a total serum IgA level of <13mg/dL (normal 81 to 463mg/dL). Her son, age 20, had an IgA level of <14mg/dL with a borderline low IgM level of 39mg/dL (normal 48 to 271mg/dL), and her daughter, age 22, had an IgA level of 33mg/dL, suggesting a relative deficiency of serum IgA. Additionally, each child had detectable anti-IgA antibodies of the IgG isotype. All other remaining lab results were unremarkable. Conclusions In order to decrease the morbidity and mortality of patients with SIgAD and/or carriers of anti-IgA antibodies, first-degree family members of individuals diagnosed with SIgAD should be routinely screened for primary immunodeficiency, regardless of their level of symptomatology.

High innate production of interleukin-10 and tumor necrosis factor-α contributes to susceptibility for non-paraneoplastic Lambert–Eaton myasthenic syndrome

Non-paraneoplastic Lambert–Eaton myasthenic syndrome (LEMS) is an antibody-mediated autoimmune disorder, in which genetically determined interleukin-10 (Il-10) and tumor necrosis factor-α (TNF-α) could play a role in the susceptibility for the disease. Therefore, we analyzed the production of Il-10 and TNF-α after whole-blood stimulation in first-degree family members of patients with LEMS without malignancy, as a measure of innate production in the patients. Thirty-six first-degree family members of 10 patients and 80 healthy controls were studied. Both Il-10 ( p=0.037) and TNF-α ( p=0.0016) production were increased in the family members, but had no relation with the severity of LEMS or HLA-B8DR3 carriership. Our findings suggest that high innate production of Il-10 and TNF-α is a susceptibility factor for non-paraneoplastic LEMS.

Antibody Screening in a Population of Children

The first large-scale (secondary) intervention trials have been initiated in first-degree family members of patients with insulin-dependent diabetes mellitus (IDDM). Within a few years, data from these studies may suggest that intervention is possible, thereby opening similar approaches in the general population. However, before large-scale intervention studies can be initiated, several problems need to be solved. One of these problems is the lack of knowledge on the natural course of β-cell autoimmunity. This review analyses this and other issues related to population-based prediction for IDDM. At present, no long-term follow-up studies are available in large-sized populations, but data show that prediction in the general population is both technically feasible and likely to have sufficient power to be useful in prevention trials. More data need to be generated, not only to determine which markers are most likely to give good prediction but also to obtain knowledge on the natural course, psychosocial impact and cost-effectiveness of screening.

Mortality in young first-degree relatives of patients with familial adenomatous polyposis

Mortality and cancer deaths among young family members of patients with familial adenomatous polyposis (FAP) were investigated. The subjects were 1764 members of 628 families with FAP registered at the authors' Polyposis Registry. They consisted of first-degree relatives of patients with FAP, excluding the propositus. These 1764 subjects were born between 1960 and 1978. Their survival times and causes of death were certified mainly by the National Family Registry and death certificates. Relative mortality rates were calculated based on the calendar year, age sex, and cause-specific death rate of Japanese people in the Vital Statistics, (Ministries of Health and Welfare, Japan. Vital statistics, Japan, Vol. 1960-1990. Tokyo: Statistics and Information Department, Minister's Secretariat, Ministry of Health and Welfare.) 1960-1990, Japan. There were 69 deaths before the age of 25 years. Deaths from malignant tumor were observed in 20 cases. Overall relative mortality rate in the subject group younger than age 20 years was 1.48. Relative cancer mortality in the age group between 1 and 4 years was significantly higher, because the relative mortality from hepatoblastoma in this age group was 176. Relative cancer mortality rate was significantly higher among males 15-19 years of age (15.4) and 20-24 years of age (male, 33.3; female, 150) due to colorectal cancer. Hepatoblastoma was a specific cancer in the 1- to 4-year-old age group in first-degree family members of patients with FAP. The incidence of colorectal cancer considerably increased, starting around the age of 20 years.

Plasma Norepinephrine Response to Standing in Patients With Pheochromocytoma or Medullary Carcinoma of the Thyroid

We determined the effect of a lying/standing test on 22 patients with medullary carcinoma of the thyroid (MCT), seven patients with pheochromocytoma (six of whom also had MCT), six healthy first-degree family members of patients with MCT, and nine normal subjects who did not have a family history of MCT. The purpose of the study was to determine if the patients with MCT had an altered noradrenergic response to standing and to determine if this test would be useful in screening MCT patients for the presence of pheochromocytoma. All of the patients with MCT, as well as all of the healthy family members, had normal urinary excretion of catecholamines and their metabolites. Plasma norepinephrine (NE) concentrations, plasma cortisol levels, and blood pressure (BP) were determined during the lying/standing test. The plasma NE concentration showed the expected increase with a change in posture; the plasma cortisol concentration did not change. There was no significant difference in the plasma NE, plasma cortisol, and BP response in the four study groups. Only one of the seven patients with pheochromocytoma had a hypertensive episode in response to standing. We conclude that there is probably a normal noradrenergic response to standing in patients with MCT, and the standing/lying test, as performed in this study, is of limited value in screening for the presence of pheochromocytoma.

A family study of Behcet's syndrome.

The first-degree family members of patients with Behcet's syndrome were investigated to determine (1) whether there was a familial aggregation of Behcet's syndrome or of its component features, (2) whether there was an increased incidence of sacro-iliitis in these families, and (3) whether a link exists between Behcet's syndrome and spondarthritides in first-degree relatives. Of the 226 relatives and patients seen, 21 patients with definite Behcet's syndrome and their 71 relatives were analysed in some detail. Orogenital ulceration was found in 2 mothers of probands; 1 patient had psoriasis and Behcet's but neither disease featured in the pedigree. Another family showed numerous manifestations of psoriasis and psoriatic arthritis, 1 member having severe sacro-iliitis but the proband had Behcet's syndrome only. Family trees including HLA pedigrees, where known, are presented. Spouses suffered few of the complaints found in patients, and to a less extent in relatives, but showed a high incidence of backache, perhaps of psychogenic origen.