At first look, the two authors of this opinion piece might appear to represent specialties that could be considered an odd couple. One is a rheumatologist, the another a psychiatrist. Don’t they represent two completely different, non-overlapping specialties? Hardly. The last few years have clearly demonstrated a great overlap between rheumatologic/inflammatory
disorders and mental health impairments. Despite this, the fact remains that these two
specialties rarely collaborate, which ends up being a disservice to the patients we treat. It is time to divide no more, and for both specialties to collaborate with each other to ensure better treatment outcomes for our patients.

It has been recently suggested that primary Sjögren’s syndrome (SS) patients present with a variety of personality structure disturbances and psychiatric symptoms. To evaluate this finding further, we assessed hostility structure and psychiatric symptoms in 33 Sjögren’s syndrome patients and compared the results with those of 33 healthy women, and 41 women with solid malignant tumors. The utilized psychometric instruments were the hostility and direction of hostility questionnaire (HDHQ) and the symptom checklist 90R (SCL-90R).
High levels of introverted hostility were reported by SS patients in relation to the other two groups. Cancer and SS patients reported higher scores on anxiety and depressive symptoms compared to the healthy women, but the differences were not significant. In addition, high scores on paranoid ideation, somatization and obsessive compulsive symptoms were found in SS patients compared to the cancer and healthy controls. The results strongly suggest that psychiatric disorders are common in primary SS patients, who may need appropriate therapy.

Children and adults who present with severe, newly acquired neurological or psychiatric deficits should be evaluated for an underlying inflammatory brain disease—the inflammation could be reversible, if diagnosed and treated rapidly. In our experience, primary angiitis of the central nervous system (PACNS) is the most common inflammatory brain disease and is increasingly recognized across patients of all ages. Distinct disease subtypes have been reported with characteristic disease courses, neuroimaging features and histopathological findings. In this Review, we provide a comprehensive comparison of childhood and adult PACNS, revealing distinct gender distributions, characteristic presenting clinical phenotypes and tailored differential diagnosis evaluations in the different subtypes of PACNS. Novel and traditional laboratory markers can help to define disease subtype and activity, whilst MRI and angiography can aid diagnosis in both children and adults. Characteristic patterns of parenchymal lesions and vessel involvement have been identified in PACNS and differ markedly between subtypes. Brain histopathology has also revealed distinct inflammatory pathways at different ages. Immunosuppressive treatment protocols have been shown to be effective and safe across the age spectrum; overall, in the past few years, the mortality of PACNS has decreased dramatically.

Although numerous articles have been written on the subject of chorea in children, the important and often serious neuropsychiatric aspects of this disease have not been adequately emphasized. When we describe the sudden, irregular, purposeless, incoordinate movements of chorea we should realize that the mental disturbances are apt to be of an emotional nature of similarly irregular type. These emotional disturbances may incapacitate the child and assume an equal or greater significance than the well known motor symptoms of the disease. The personality of the child may suffer after he has passed through a period of lack of motor control, and this may be shown by various defects of adjustment, including a relationship to juvenile delinquency. The interference with school progress during the receptive period of the child may assume great importance in the later adaptation of the child.

Sydenham’s chorea is a rare movement disorder associated with streptococcal infection. The co-occurrence of neuropsychiatric symptoms has raised the question of whether streptococcal infection could trigger these symptoms without chorea. This study evaluated the prevalence of behavioral diagnoses before, during, and after the onset of chorea in a cohort of children with a history of Sydenham’s chorea for whom demographic and clinical data were available. In all, 28 Sydenham’s chorea patients were evaluated, with a mean age of 10.3 years. Retrospective analysis was performed for subject demographics, streptococcal titers, and presence of arthritis and carditis. Structured diagnostic interviews were performed on 14 available patients and parents. Streptococcal titers and duration of treatment for chorea were compared between groups. The prevalence of attention deficit hyperactivity disorder before and after chorea was 30 and 37%, respectively. The proportion of children meeting combined, subthreshold, and suprathreshold criteria before, during, and after Sydenham’s chorea was 71, 79, and 79% for anxiety and 19, 69, and 44% for depression. Streptococcal antibody titers and duration of treatment did not correlate with attention deficit hyperactivity disorder, depression, or anxiety disorders. During and after the diagnosis of Sydenham’s chorea, clinicians should be vigilant for signs and symptoms of anxiety, depression, and attention deficit hyperactivity disorder.

The authors investigated obsessive-compulsive behavior, obsessive-compulsive disorder (OCD), and attention deficit and hyperactivity disorder (ADHD) in 50 healthy subjects, 50 patients with rheumatic fever without chorea, and 56 patients with Sydenham chorea. Obsessive-compulsive behavior, OCD, and ADHD were more frequent in the Sydenham chorea group (19%, 23.2%, 30.4%) than in the healthy subjects (11%, 4%, 8%) and in the rheumatic fever without chorea group (14%, 6%, 8%). ADHD was more common in persistent Sydenham chorea.

The 20-item Leyton Obsessional Inventory–Child Version was completed by children and adolescents who had had Sydenham’s chorea (N = 23) or rheumatic fever without chorea (N = 14). The Sydenham’s chorea subjects had significantly more obsessive thoughts and compulsive behaviors and significantly greater interference from these behaviors. Three Sydenham’s chorea patients but no rheumatic fever patients had substantial obsessional interference and met criteria for obsessive-compulsive disorder when interviewed by telephone. This suggests that obsessive-compulsive disorder, at least in some patients, may be due to basal ganglia dysfunction.

OBJECTIVE: The incidence and course of neuropsychiatric symptoms were determined in pediatric patients with rheumatic fever. METHOD: The Leyton Obsessional Inventory and National Institute of Mental Health Global Obsessive-Compulsive Scale were used to evaluate children and adolescents who had rheumatic fever with Sydenham’s chorea (N=30) or without chorea (N=20). They were assessed three times over 6 months from the onset of rheumatic fever. Psychiatric diagnoses were also determined. RESULTS: Obsessive-compulsive symptoms abruptly appeared and peaked during the 2 months after the onset of rheumatic fever in 21 patients with chorea (70.0%) and were absent in all patients without chorea. Obsessive-compulsive disorder (OCD) was diagnosed in five patients with chorea (16.7%). CONCLUSIONS: The association between Sydenham’s chorea and OCD supports suggestions that similar mechanisms involving the basal ganglia underlie both disorders. Obsessive-compulsive symptoms occurred at the beginning of rheumatic fever, so early psychopathological assessments are essential. (Am J Psychiatry 1998; 155:1122–1124)

Eleven children with Sydenham’s chorea (8 girls and 3 boys, mean age = 8.4 ± 2.2 [SD] years) underwent comprehensive physical, neuropsychologic, and psychiatric examination. The chorea was manifested as dysarthria, gait disturbances, and frequent adventitious movements of the face, neck, trunk, and extremities. Antineuronal antibodies were present in 10 of 11 children. All children exhibited concomitant psychologic dysfunction, specifically obsessive-compulsive symptomatology, increased emotional lability, motoric hyperactivity, irritability, distractibility, and age-regressed behavior. Obsessive-compulsive symptoms were observed in 9 (82%) children, 4 of whom met diagnostic criteria for obsessive-compulsive disorder. These behavioral symptoms began several days to weeks before the chorea was observed, and they waxed and waned in severity along with the motoric abnormalities. These results suggest that psychologic, particularly obsessive-compulsive, symptoms are accompanying manifestations of Sydenham’s chorea which may require medical attention.

1. During a long-term follow-up study of patients previously hospitalized with Sydenham’s chorea, a high incidence of psychological disturbance was encountered prior to the onset of chorea, and at the time of current evaluation, an average of 29 years later.

2. Seventy-five per cent of patients with Sydenham’s chorea were currently found to manifest evidence of psychiatric disturbance.

3. A control population similarly evaluated showed a 25% incidence of psychiatric disability—about the expected incidence for the general population.

4. This study emphasizes the need to consider psychological factors as part of the total evaluation of patients with Sydenham’s chorea.

The prevalence of rheumatic fever and rheumatic heart disease reflects the adequacy of preventive medical care in any given community. In more developed countries, these diseases have become rare, whereas in many developing countries, rheumatic heart disease remains the leading cause of heart disease among children and young adults.

Question Is streptococcal throat infection associated with obsessive-compulsive disorder (OCD) and tic disorders, as suggested by the pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) hypothesis?

Findings In this population-based cohort study, streptococcal throat infections increased the risk of any mental disorder, OCD, and tic disorders; however, compared with nonstreptococcal throat infections, only the risk of any mental disorder and OCD was significantly higher after a streptococcal infection.

Meaning The PANDAS hypothesis was supported to some extent, but because other infections also increased the risk of mental disorders, our results could also support aspects of the wider diagnostic concept of pediatric acute-onset neuropsychiatric syndrome.

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus (PANDAS) research is based on the hypothesis that infections trigger changes in behavior and movement in children. We enrolled 693 children (ages 3 to 12 years) into a systematic, longitudinal study. Data were collected monthly for 8 months (October–May) to determine point prevalence of Group A Streptococcal (GAS) infections, tics, behavior, and choreiform movements. Simultaneous throat cultures were obtained, and relational analyses were made between GAS and movement/observation ratings.

Streptococcus pyogenes infections have been associated with two autoimmune diseases of the CNS: Sydenham’s chorea (SC) and Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus infections (PANDAS). Despite the high frequency of pharyngeal streptococcus infections among children, only a small fraction develops SC or PANDAS. This suggests that several factors in combination are necessary to trigger autoimmune complications: specific S. pyogenes strains that induce a strong immune response toward the host nervous system; genetic susceptibility that predispose children toward an autoimmune response involving movement or tic symptoms; and multiple infections of the throat or tonsils that lead to a robust Th17 cellular and humoral immune response when untreated. In this review, we summarize the evidence for each factor and propose that all must be met for the requisite neurovascular pathology and behavioral deficits found in SC/PANDAS.