The time of equipoise on the use of biological DMARDs in for inflammatory arthritis during pregnancy is finally over: a reappraisal of evidence to optimise pregnancy management.

Active inflammatory arthritis in pregnancy is associated with an increased risk of adverse pregnancy outcomes. Treatment of active inflammation and maintenance of low disease activity with medication reduces these risks. Therapeutic decisions on disease-modifying antirheumatic drugs (DMARDs) in pregnancy are complicated by safety concerns, which have led to inappropriate withdrawal of treatment and consequential harm to mother and fetus. Studies of inflammatory arthritis in pregnancy have consistently shown minimal safety concerns with the use of biological DMARDs and an increased risk of disease flare with discontinuation of biological DMARDs. It is our opinion that during pregnancy, the benefits of disease control with biological DMARDs, when required in addition to conventional synthetic DMARDs, outweigh the risks. In this Series paper, we review the reasons for reconsideration of equipoise and propose an agenda for future research to optimise the use of biological DMARDs in inflammatory arthritis during pregnancy.

A curious case of 'COVID toes' in pregnancy.

The novel coronavirus of 2019 (COVID-19) can affect multiple organ systems with a wide spectrum of illness severity. Its effect on the respiratory tract is well-documented and has resulted in considerable excess mortality worldwide. However, observed cutaneous manifestations of COVID-19 are rising, ranging from short-lived viral exanthems to vesicular eruptions and urticaria. An unusual subgroup of these manifestations - pseudo-chilblains, also referred to as pernio-like lesions or 'COVID toes' - describes the acral areas of erythema and oedema that can affect young individuals following COVID-19. We present a case associated with pustule and vesicle formation occurring in the context of pregnancy.

Prescribing for pregnancy: inflammatory rheumatic disease.

Inflammatory rheumatic disease during pregnancy requires careful management. Key factors for successful pregnancy outcome are disease remission at the time of conception and optimal disease control during pregnancy. This article forms part of a series on prescribing for pregnancy and discusses the impact of inflammatory arthritis on pregnancy and the influence pregnancy may have on inflammatory arthritis. It highlights the importance of prepregnancy care and collaborative working between obstetric and rheumatology specialties as well as focusing on prescribing before, during and after pregnancy.

Case series of COVID-19 infection in pregnancy complicated by ketoacidosis and symptomatic breathlessness.

Background: The differential diagnosis of acute shortness of breath in a pregnant woman with COVID-19 is broad. Pregnancy is a ketosis-prone state, which can result in metabolic acidosis and tachypnoea. Methods: We describe four pregnant women with COVID-19 and breathlessness where ketoacidosis was found to contribute to symptomatic tachypnoea. Results: One patient did not have associated COVID-19 pneumonitis, but presented with severe tachypnoea and metabolic acidosis; three women had pneumonitis and metabolic acidosis. Corrective treatment for the metabolic abnormalities resulted in resolution of the ketoacidosis in all cases. No women had coexistent diabetes. Conclusion: This is the first series of COVID-19 in pregnancy complicated by ketoacidosis and symptomatic tachypnoea. Ketoacidosis associated with COVID-19 is an important cause of tachypnoea requiring specific treatment, which should not be overlooked. Potential mechanisms for this are discussed with a framework for interpretation of blood gas results during pregnancy.

Case report: Hereditary angioedema in pregnancy.

Hereditary angioedema (HAE) is a rare genetic condition associated with episodic swelling due to dysfunction of bradykinin regulation pathways. This is most frequently caused by low level and/or function of the C1-esterase inhibitor protein (C1INH) which is known as hereditary angioedema with C1 inhibitor deficiency (C1INH-HAE). Pregnancy and labour can precipitate an attack, but the majority of women have an uncomplicated, spontaneous vaginal delivery. Intravenous C1INH is the first-line therapy in pregnancy and breastfeeding. It should be given if any obstetric intervention is planned. Routine prophylactic administration for uncomplicated vaginal birth is not mandatory but may be appropriate if symptoms recur frequently during the third trimester. Pregnant women with C1INH-HAE should deliver in a hospital with C1INH replacement, fiberoptic intubation and front-of-neck access equipment readily available. A documented treatment plan should be developed within a multi-disciplinary team to pre-empt complications. We describe a case of C1INH-HAE diagnosed in pregnancy.

A retrospective series of conditions and mortality associated with extreme hyperferritinaemia in adults.

BACKGROUND: Serum ferritin is commonly used in the diagnosis of iron deficiency anaemia. However, extreme hyperferritinaemia suggests a significant illness, including the differential diagnosis of haemophagocytic lymphohistiocytosis (HLH), which is rare and associated with a high mortality, particularly if untreated. This series aims to identify the causes and associated mortality of severe hyperferritinaemia in patients seen at a teaching hospital in London, UK. METHOD: Demographic and medical data were collected for all patients over 18 years of age with extreme hyperferritinaemia (defined as serum ferritin levels of ≥4000 mcg/L). Conditions associated with hyperferritinaemia and in-hospital mortality were identified from medical records, laboratory data and discharge and death notification. RESULTS: One hundred and fifty-five cases of extreme hyperferritinaemia in adults were identified. Associated conditions included iron overload (35%), malignancy (24%), infectious disease (21%) and hepatocellular disease (12%). Autoimmune disease and HLH resulted in significantly higher median peak ferritin levels compared with all cases (10 616 mcg/L, P < .01 and 19 138 mcg/L, P < .05, respectively). Patients with confirmed HLH had the highest median peak ferritin. Uncommon infections were identified in this series, and included such as dengue, syphilis, HIV and murine typhus. HLH had been confirmed in seven patients (5%). In five patients (3%) no clear cause for raised ferritin was identified. Overall mortality in the whole cohort was 14% (n = 22), but there was a very high mortality of 80% in the group where no cause was found for the hyperferritinaemia, and these patients were significantly more likely to die during the index admission (P < .01). CONCLUSION: Extreme hyperferritinaemia is associated with a broad differential diagnosis of significant medical conditions, including iron overload, infections, cancer and liver disease. Rare infectious causes were also identified, and this series reports a greater proportion of cases of HLH than has previously reported. Unexplained hyperferritinaemia was associated with significant mortality.

Sjögren's syndrome.

This review discusses important aspects of the diagnosis and management of Sjögren's syndrome, covering clinical features, diagnosis and management, and summarizes recent developments in diagnosis, prognostication and treatment.