Catatonia in systemic lupus erythematosus: case based review.

Catatonia is a rare psychomotor syndrome characterized by stupor, posturing and echophenomena. It can be associated with schizophrenia, infections, drugs and autoimmune causes like anti N-methyl D-aspartate (NMDA) receptor encephalitis and systemic lupus erythematosus (SLE). Here we report two cases of systemic lupus erythematosus with catatonia, who improved with immunosuppressive treatment and review the cases described in the literature. The first case presented with fever, pancytopenia, toxic epidermal necrolysis (TEN)-like rash and later developed catatonia and macrophage activation syndrome (MAS). The second case presented with acute cutaneous lupus erythematosus (ACLE), fever, alopecia, polyarthralgias, nephritis, cytopenias along with catatonia. Successful management of this syndrome requires prompt recognition and treatment with immunosuppression as well as benzodiazepines with or without electroconvulsive therapy (ECT).

Teleconsultation experience with the idiopathic inflammatory myopathies: a prospective observational cohort study during the COVID-19 pandemic.

Teleconsultation has assumed a central role in the management of chronic and disabling rheumatic diseases, such as the idiopathic inflammatory myopathies (IIM), during COVID-19. However, the feasibility, challenges encountered, and outcomes remain largely unexplored. Here, we describe our teleconsultation experience in a prospectively followed cohort of adult and juvenile IIM. 250 IIM enrolled into the MyoCite cohort (2017-ongoing) were offered the option of audio/visual teleconsultation using WhatsApp during the nationwide lockdown. Clinical outcomes (major/minor relapse) and prescription changes were compared between IIM subsets. Socio-demographic and clinico-serological characteristics of those who sought teleconsultation were compared with those who did not. 151 teleconsultations were sought over a 93 day period by 71 (52.2%) of 136 IIM (median age 38 years, F:M 4.5:1). Nearly one-third (38%) consulted on an emergency basis, with voice consultations being the primary medium of communication. Over a quarter (26.8%) reported relapse (15.5% minor, 11.3% major), these being more common in JDM [71.4%, OR 8.9 (1.5-51)] as compared with adult IIM, but similar across various antibody-based IIM subtypes. Patients who relapsed required more consultations [2(2-3) vs 1(1-2), p 0.009]. The demographic and socioeconomic profile of the patients seeking consultation (n = 71) was not different from those who did not (n = 65). Voice-based teleconsultations may be useful to diagnose and manage relapses in IIM during the pandemic. Patient education for meticulous and timely reporting may be improve care, and larger multicentre studies may identify subsets of IIM that require greater care and early tele-triage for effective management of the condition.

A prospective study of novel disease activity indices for ankylosing spondylitis.

There is an ongoing quest for robust disease activity measures in Ankylosing spondylitis (AS). Thus, we prospectively validated two new disease activity indices, Simplified AS Disease Activity Score (SASDAS) and modified Juvenile Spondyloarthritis Disease Activity Score (JSpADA). Patients with AS were assessed for BASDAI, ASDAS and other outcome measures at baseline and 3 months. Comparisons were drawn between those with juvenile onset, early disease and peripheral involvement, with the rest. Fisher's r to Z transformation was used to compare correlations. Receiver-operating characteristic (ROC) curves were used to calculate cutoffs for inactive, low, high and very high disease activity. Of the 107 patients (mean age-29 years) of 6-years long disease, 38.3% had a juvenile onset. SASDAS and modified JSpADA exhibited excellent correlation with BASDAI and ASDAS (all p < 0.001) and were higher in active vs. inactive disease. Treatment responders had a greater fall in SASDAS and modified JSpADA as compared to non-responders. The novel scores were higher in those with peripheral disease. Only SASDAS could discriminate early from late disease. Based on the previously proposed cutoffs, optimal scores for inactive, moderate, high and very high disease activity were deduced. SASDAS-CRP showed better internal consistency than SASDAS-ESR and correlated better with ASDAS-CRP in late disease (Z = 3.04; p = 002) and those with adult onset disease (Z = 2.18; p = 0.03). SASDAS and Modified JSpADA perform as well as standard complex scores and have potential for simpler daily use. From our analyses, SASDAS with CRP performs better than SASDAS-ESR, pending further validation.

Microsporidial myositis in adult-onset immunodeficiency: case-based review.

Polymyositis is a diagnosis of exclusion. In patients with odd features, it can be of infective etiology. A high index of suspicion is required for diagnosis. A 55-year-old gentleman presented with gradual-onset proximal muscle weakness. Examination revealed mild distal weakness but no rash. Muscle enzymes were raised and tests for autoantibodies were negative. Biopsy revealed microsporidiosis. In view of this unusual infection, immunodeficiency was considered and he was found to have lymphopenia which antedated his illness. Later, he developed cranial nerve palsies due to multiple lesions in the pons. In addition, he had Cytomegalovirus viremia. Literature was reviewed to identify 20 cases of microsporidial myositis, its presentation, underlying immunodeficient state, and clinical course. Infective polymyositis should be considered in a patient with paucity of clinical and serological autoimmune features. Lymphopenia can point to underlying immunodeficiency. CMV infection could be the contributor to or bystander-effect of idiopathic lymphopenia.