SAPHO Syndrome: An Unusual Cause of Dysphagia

Case Presentation: This case describes a 51-year-old male who presented to the emergency department with a complaint of two weeks of progressively worsening dysphagia as well as the emergence of superficial fluid collections on the anterior chest and leg during the same period. Computed tomography showed retropharyngeal and paratracheal fluid collections with adjacent vertebral osteitis; however, biopsies were negative for any infectious or mycobacterial source, and instead showed chronic inflammatory changes. Discussion: Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare rheumatic disorder that presents with multifocal osteitis and sterile neutrophilia. SAPHO syndrome may be easily mistaken for a diffuse infectious process on initial evaluation and imaging; however, it is treated with anti-inflammatory medications, including non-steroidal anti-inflammatory drugs and corticosteroids. Although most patients achieve remission of symptoms with treatment, the location of the fluid collections and resultant bony destruction may be life-threatening if undiagnosed.

Systemic lupus erythematosus and related disorders

Systemic lupus erythematosus is an autoimmune rheumatic disorder that can present with symptoms in almost any organ or system of the body. It is 10 times commoner in women than men, and commoner in Afro-Caribbeans than in other ethnic groups. Its aetiology is multifactorial, incorporating genetic, hormonal, and environmental elements. No single abnormality of the immune system can be considered responsible, pathogenesis depending on the interplay of several different factors, including autoantibodies, T lymphocytes, cytokines, the complement system, and apoptosis. Common symptoms are constitutional (fatigue, anorexia), musculoskeletal (arthralgia/arthritis, myalgia), dermatological (alopecia, butterfly rash, vasculitic skin lesions, purpura), cardiopulmonary (breathlessness, pleurisy), and neurological (migraine, seizures, depression, psychosis). Treatment for mild disease is NSAID, analgesics and hydroxychloroquine, more severe disease requires corticosteroid and immunosuppressant drugs.

Rheumatic Disease Autoantibodies in Patients with Autoimmune Thyroid Diseases

Background: Patients with autoimmune thyroid diseases (ATD) such as Graves’ disease (GD) and Hashimoto thyroiditis (HT) may have non-organ specific autoantibodies such as antinuclear antibodies (ANA) and rheumatoid factor (RF). Aim: To study the prevalence of rheumatic autoantibodies in a group of ATD patients without known rheumatic diseases and to evaluate its association with the patients’ epidemiological and treatment profiles. To follow positive non-organ specific autoantibody-positive ATD individuals to investigate whether they will develop a rheumatic disorder. Methods: A sample of 154 ATD patients (70 HT and 84 GD; mean age 45.3 ± 14.2) had determination of ANA by immunofluorescence, using hep-2 cells as substrate, extractable nuclear antigen profile by ELISA kits and RF by latex agglutination. Epidemiological and treatment profiles were obtained through chart review. These patients were followed for the mean period of 5 years, between 2010 and 2015. Results: Positive ANA was found in 17.5% (27/154) of the patients: anti-Ro/SS-A in 4/154 (2.5%); anti-RNP in 4/154 (2.5%), and anti-La/SS-B in 3/154 (1.9%). None had anti-Sm antibodies. RF was detected in 12/154 (7.7%) of ATD patients and was more common in older individuals (p = 0.007). There was a positive association between the presence of RF and ANA (p = 0.03; OR 3.89; 95% CI 1.1–13.3). None of the patients with positive autoantibodies developed clinical rheumatic diseases during the period of observation. Conclusion: We found rheumatic autoantibodies in 17.5% of ATD patients without rheumatic diseases. None of them were associated with the appearance of clinical rheumatic disorder during the period of 5 years.

Guidelines for Psychological Assistance to Chronically Ill Children and Their Parents

This study explored psychological features in 33 children aged 8—17 years suffering from rheumatic disorder and in their mothers. 11 children were in the emergency department, 22 in the department of rheumatology. The following methods were used: observation, conversation, the Draw-a-Person and Three Wishes tests, the Dembo-Rubinstein self-esteem scales, and the Parent’s Essay technique. According to the level of their mental activity the children were divided into 4 groups: ‘inactive’, ‘passive’, ‘relatively active’ and ‘active’. Working with the ‘inactive’ children was impossible; the main task was to help their mothers cope with emotional distress. The ‘passive’ children received emotional support and were taught how to understand and express their needs; their parents were taught how to communicate with the child in various ways. Working with the ‘relatively active’ children and their parents had the aim of reestablish- ing productive interactions between them. Working with the ‘active’ children consisted of supporting their self-esteem, self-respect and interests; their parents were taught to better understand age-specific and indi- vidual psychological features of the child and to take into account limitations caused by the illness.

Refractory Rheumatic Disorder: Atypical Postpregnancy Osteoporosis

This is a case report on a young patient with severe osteoporosis that was initially revealed when she presented with polyarthralgia during her second pregnancy. Postpartum, the pain increased and her X-ray did not show any abnormalities. A bone scintigraphy was performed. It indicated an inflammatory rheumatic disorder. Six months after partum, an investigation of right coxalgia revealed a spontaneous basicervical fracture. Given the persistent polyarthralgia, the patient underwent a new scintigraphy, which revealed areas of what looked to be old rib and L1 fractures. A subsequent full body magnetic resonance imaging (MRI) scan revealed signal abnormalities that could indicate multiple lower limb bone fractures. Despite exhaustive biological, radiological, and histological testing, no secondary cause for the osteoporosis was found. The patient was started on teriparatide. We finally concluded that, despite the atypical presentation, the patient was suffering from postpregnancy osteoporosis. It is possible that the frequency of occurrence of this still poorly understood disease is underestimated.