Idiopathic recurrent pericarditis (IRP) impairs quality of life. Although its precise etiology is not certain, it is believed to be immunologically mediated. Its optimal treatments are unknown. Initial therapy is with non-steroidal anti-inflammatory drugs and colchicine. Steroids, which are often used, however may promote recurrences. European guidelines advocate azathioprine or cyclophosphamide in refractory cases despite limited evidence. We report two adults with IRP successfully treated with the interleukin-1 antagonist, anakinra. We combine this experience with the first systematic literature review of immunosuppression in IRP. A total of 8 papers were included in the review, which alongside our patients described 18 cases. The best treatments comprised anakinra and intravenous immunoglobulin, with respective remission rates of 100% and 67%. Cyclophosphamide and azathioprine were less efficacious. The unprovoked inflammatory episodes in our patients alongside their prompt response to anakinra indicate that in some instances IRP may represent an autoinflammatory condition. We suggest that in IRP refractory to initial treatment, anakinra should be considered as a potential therapy. Clinical trials are required to confirm its benefits in IRP.
Objectives Real-world secukinumab gastrointestinal related adverse events (GIRAE) data as treatment for ankylosing spondylitis (AS) and psoriatic arthritis (PsA) are lacking. We aimed to achieve this through baseline evaluation of pre-existing inflammatory bowel disease (IBD), rates and predictors of GIRAE. Methods Patient electronic and paper records commencing secukinumab from ten UK hospitals between 2016–2019 were reviewed. GIRAE after initiation were defined as: definite (objective evidence of IBD (biopsy proven), clear temporal association, resolution of symptoms on drug withdrawal, no alternative explanation felt more likely), probable (as per definite, but without biopsy confirmation) or possible (gastrointestinal symptoms not fulfilling definite or probable criteria). Results Data for all 306 patients started on secukinumab were analysed: 124 (40.5%) AS and 182 (59.5%) PsA. 24/306 (7.8%) experienced GIRAE after starting secukinumab. Amongst patients who developed GIRAE, 4 (1.3%) had definite, 7 (2.3%) probable and 13 (4.2%) possible IBD. All definite cases were patients with AS and stopped secukinumab; two had pre-existing IBD and two (0.7%) were de-novo cases of which one required surgical intervention. Seven patients (2.3%) had pre-existing diagnoses of IBD prior to initiation of which 5 patients experienced GIRAE. Conclusion Absolute rates of new IBD in patients starting secukinumab are low. The majority of patients developing new GIRAE did not develop objective evidence of IBD or stop therapy. For patients with pre-existing IBD and AS the risk of GIRAE is much higher, and prescribing alternatives should be considered.
Cocaine is a commonly used illicit drug that is associated with cocaine-induced midline destructive lesions (CIMDLs). It is increasingly adulterated with levamisole, which has been shown to cause levamisole-induced vasculitis (LIV), a systemic vasculitis with ENT repercussions. Approximately 976 000 people aged 16-59 used powder cocaine in 2018/19 in the UK. 1 Levamisole, initially marketed in 1971 as an anti-helminthic drug, was removed from the US market in 2000 due to adverse effects, such as vasculitis. 2,3 It adds weight to cocaine and may potentiate its euphoric effect. 4 Approximately 70% of seized cocaine contained levamisole, and the proportion of cocaine "cut" with levamisole is increasing globally. 4 Whilst the prevalence of CIMDL and LIV is small compared with overall cocaine use, clinical repercussions can be severe.
A young woman with systemic lupus erythematosus (SLE) developed recurrent enterovirus meningoencephalitis while taking prednisolone, azathioprine and rituximab. After reducing the immunosuppression, she developed a central nervous system (CNS) flare of SLE, with enterovirus still present in the cerebrospinal fluid (CSF). There are no evidence-based specific treatments for enterovirus encephalitis, but she responded well to intravenous immunoglobulin alongside pulsed methylprednisolone and rituximab. This case highlights the difficulties in managing people with co-existing infective and autoimmune conditions, especially if each affects the CNS. A viral infection and SLE flare can resemble one another clinically, although here the radiological differentiation of CNS lupus versus enterovirus encephalitis helped to guide the diagnosis.
Numerous post-Streptococcal syndromes (PSS) have been described in the literature. The role of antibiotic therapy in the management of PSS is best established with acute rheumatic fever. We present a patient with streptococcus-associated medium vessel vasculitis with multiple flares despite immunosuppressive therapy that achieved a sustained remission with long term oral penicillin V 250 mg twice daily.
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