Antiphospholipid (Hughes) syndrome 40th anniversary.

This article is a celebration of the 40th anniversary of APS, a disease that appears to affect one in 2000 people. The quality of life of patients affected has improved significantly as a result of early diagnosis and effective treatment.

Erythema nodosum in Behçet's disease in remission: Think COVID-19?

The coronavirus disease has several manifestations related to the activation of the immune system. Because of such activation, autoimmune diseases, including vasculitis, have been reported to occur. Behçet's disease, a variable vessel vasculitis, has been discussed in the context of coronavirus disease. Rarely, the induction of Behçet's disease flare or exacerbation has been reported necessitating aggressive treatment. The presentation of Behçet's disease flares secondary to coronavirus disease is variable, including mucocutaneous lesions and eye or joint involvement. We highlight the case of a 35-year-old woman with pre-existing Behçet's disease in remission on colchicine presenting with new onset erythema nodosum-like lesions on her right shin being diagnosed with coronavirus disease infection a few days after. Despite treatment with systemic corticosteroid, the lesions did not resolve, necessitating the initiation of anti-interleukin-6 therapy.

Night sweats (nocturnal hyperhidrosis) causing wrinkled (pruney) hands in a patient with SARS-CoV-2 infection.

We report the case of a 28-year-old female who developed severe night sweated on the ninth day of SARS-CoV-2 infection with wrinkling of the fingers and palms.

Rheumatoid Cerebral Vasculitis in a Patient in Remission.

Cerebral vasculitis is a very rare extra-articular complication of rheumatoid arthritis (RA) that is often challenging to diagnose. Elevated titers of rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP), and antinuclear antibodies (ANA) have been linked with severe complications. The absence of highly elevated titers of RF, anti-CCP, and ANA can complicate the diagnosis of RA-associated cerebral vasculitis. We report the case of a 59-year-old woman with long-standing arthritis maintained on rituximab and leflunomide who developed sudden headaches and altered level of consciousness. Laboratory work-up revealed normal lymphocyte count and mildly elevated total serum protein and anti-CCP with negative RF and ANA and no evidence for viral or bacterial infections. Cerebrospinal fluid analysis (CSF) showed slightly elevated anti-CCP with normal levels of CXCL-13 and interleukin 6 (IL-6). Brain magnetic resonance imaging (MRI) showed ill-defined lesion of high T2 signal. Using MR angiogram, MR perfusion, and MR spectroscopy, the diagnosis of rheumatoid cerebral vasculitis was confirmed. The patient was treated with intravenous methyl-prednisolone with fast complete improvement. We conclude that adequate immunosuppression in RA might not be able to prevent rare extra-articular manifestations such as rheumatoid cerebral vasculitis.

Musculoskeletal Manifestations of Sarcoidosis.

Since its initial description in the late 19th century, sarcoidosis has been extensively studied. Although the general mechanism of immune activation is known, many details especially in the context of disease associations are still missing. One of such associations is the musculoskeletal complications that are widely variable in terms of presentation and response to treatment. Sarcoidosis can involve the joints leading to acute and, less commonly chronic, arthritis. While acute arthritis is mostly self-resolving in nature, chronic arthritis may lead to deformity and destruction of the joint. Sarcoidosis can also involve the muscles, leading to different pathologies primarily categorized according to the clinical presentation, despite the efforts to find a new classification based on imaging, histological, and clinical findings. The bones can be directly and indirectly affected. Different types of bone lesions have been described, although around half of these patients remain asymptomatic. Osteoporosis, increased risk of fractures, hypercalcemia, and hypercalciuria are examples of the indirect effect of sarcoidosis on the bones, possibly contributed to elevated levels of calcitriol. Nevertheless, sarcoidosis can be associated with small-vessel, medium-vessel, and large vessel vasculitis, although it is frequently difficult to differentiate between the co-existence of a pure vasculitis and sarcoidosis and sarcoid vasculitis.

Optimising the management of osteoporosis.

With increasing longevity of the population globally, the prevalence of osteoporosis will rise, associated with significant morbidity, disability and increased mortality. Adequate intake of calcium, vitamin D, increasing physical activity, a strategy of avoiding falls, cessation of smoking and avoiding excessive alcohol intake are pivotal in maintaining healthy bones in all age groups. Oral bisphosphonates remain the most cost-effective first line of treatment. Better methods of identifying patients with high fracture risk is needed as there is adequate effective treatment for osteoporosis.

An old disease re-emerging: acute rheumatic fever.

We present the case of a 41-year-old Australian woman with a 3-day history of fevers and migratory polyarthritis. Three weeks prior she had been treated by her GP with phenoxymethylpenicillin for acute tonsillitis. Examination confirmed synovitis. Systemic assessment including cardiovascular, neurological and skin examination was unremarkable. Abnormal investigations included a leucocytosis with neutrophilia, C-reactive peptide of 116 mg/L, erythrocyte sedimentation rate of 103 mm/hour and acute transaminitis. The antistreptolysin O titre was 304 IU, which increased to 1,250 IU after 2 weeks. Troponin T, electrocardiogram and echocardiography were all normal. The patient was treated for acute rheumatic fever with corticosteroids and a 10-day course of cephalexin. After 8 weeks, she made a full recovery and had normalised inflammatory markers and liver biochemistry. She was then commenced on monthly prophylactic intramuscular benzathine penicillin. This case study aims to raise awareness of the presentation, diagnosis and management of acute rheumatic fever.