Comparison of rituximab efficacy in treatment-naive and refractory inflammatory myopathies: experiences from a tertiary care centre.

OBJECTIVES: To find out if Rituximab (RTX) is effective in "treatment naive" idiopathic inflammatory myopathies (IIM), and whether there could be differential treatment responses between the "treatment naive" and treatment "refractory" IIM. METHODS: Data obtained from a prospectively maintained database comprising patients with IIM treated with rituximab. Patient details were obtained at baseline, 3-months, 6-months intervals, and subsequent follow up visits. Treatment response was categorised as improved, worsening, or stable based on manual muscle testing (MMT8) scores, patient global and physician global improvement (PtGA and PGA) for skin and joint symptoms improvement and spirometry at 6 months. The time to clinical improvement and remission were noted and survival analysis curves were constructed. RESULTS: 60 patients with IIM (including 18 with anti-SRP myopathy) were included, out of which 33 who received RTX were treatment naïve. The remaining 27 were started on rituximab for refractory myopathy. Mean age was 39 years (SD12.58) in "treatment-naive" group and 43 years (SD 12.12) in "refractory" group. At 6 months of follow up, 48/55 (87%) patients showed response, 31/31 (100%) in "treatment-naive" and 17/24 (70%) in "refractory" cases, p 0.006*. In refractory group, 7 (29%) had stable disease. The mean changes in MMT8 were significantly more in the "treatment-naive" treatment group (13.41(SD 7.31) compared with "refractory" IIM 8.33 (SD 7.92) (p= 0.017*). Majority of patients were able to reduce dose below 5 mg/day before 6 months. No major adverse events were reported over the median follow-up of 24 (IQR 36) months. CONCLUSIONS: Rituximab is effective and safe across the spectrum of IIM. Early use in disease is associated with better outcomes.

Reevaluating Diagnosis of Sarcoidosis: Biopsy with Necrosis in Mycobacterial Endemic Areas.

BACKGROUND: Sarcoidosis is a multisystem inflammatory disease with a variable presentation. The most characteristic feature of sarcoidosis is nonnecrotizing granulomas. However, when sarcoidosis presents with rare organ involvement, and biopsy shows necrosis, the diagnosis becomes challenging. CASE PRESENTATION: Here, we present three cases of sarcoidosis with unusual organ involvement and biopsy findings of necrosis, leading to a delay in diagnosis and treatment. Case 1 was presented with lymphoreticular involvement within the intraparotid lymph node and genitourinary area. Biopsy from the epididymis showed necrosis, initially leading to treatment for tuberculosis (TB). Case 2 describes lymphoreticular involvement and cardiac symptoms. His cervical and bone marrow biopsies showed necrosis. Case 3's presentation was disseminated lymphadenopathy with hepatosplenomegaly, initially suspected as malignancy or TB. CONCLUSION: While biopsy plays a significant role in diagnosing sarcoidosis, the presence of necrosis alone should not lead to its exclusion.

The Effect of Hypertension and Diabetes Mellitus on White Matter Changes in MRI Brain: A Comparative Study between Patients with Alzheimer's Disease and an Age-matched Control Group.

BACKGROUND: White matter hyperintensities (WMH) on MRI brain in the periventricular and deep white matter regions are commonly seen in older persons with normal cognition and in patients with AD. AIMS: To compare presence and severity of WMHs in patients with AD with that in a cognitively normal control group, and to evaluate effect of presence of Hypertension and Diabetes on WMHs in both groups. MATERIAL AND METHODS: Thirty four patients with AD were serially recruited from Neurology and Psychiatry OPDs. An age and gender matched cohort of 24 persons with MMSE over 27/30 from the community acted as controls. Vascular risk factors, MMSE and MRI brain were assessed in all. Fezeka's and Pasquier grading of WMH and atrophy were done. Periventricular WMHs (PVWMH) and Deep WMH (DWMH) were assessed separately. RESULTS AND CONCLUSIONS: Overall, Periventricular WMHs of grade 2 and over were seen in 19/34 patients, and in 7/24 controls (P value 0.044). Significantly higher grades of PVWMHs were seen in hypertensives as compared to nonhypertensives in the case group, and in women compared to men. In the control group, hypertension had no effect on severity of PVWMHs. Among both Diabetics and non-diabetics, no difference in PVWMHs was found between the case and control groups. DWMHs were, conversely, seen only in the control group. Overall, over a quarter of cognitively normal older persons had WM hyperintensities of grade 2 and over on MRI brain; 55% of AD patients had PVWMH of Gd 2 or over, and no DWMHs.

Clinical profile of anti-NXP-2 antibody-positive inflammatory myositis and outcome in an Indian population.

INTRODUCTION: Myositis-specific antibodies (MSA) play an important role in the clinical presentation and prognosis of patients with idiopathic inflammatory myositis (IIM). Anti-NXP-2 is one of the newly described MSA. OBJECTIVE: We aimed to describe various clinical presentations associated with anti-NXP2 antibodies and assess response to treatment. METHODS: In this retrospective study, the electronic medical records of all patients who tested positive for anti-NXP2 during June 2019 to April 2022 were screened. Details of demography, clinical presentation, and treatment data were recorded. The anti-NXP2 was tested using the Euro line test kit. Any patient who had an intensity of ≥1+ was considered testing positive. The diagnosis of IIM was reviewed after applying the 2017 European League of Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria of myositis. RESULTS: Among the 660 suspected patients, 470 (71.2%) patients were positive for IIM, and 28 (5.95%) patients were positive for anti-NXP2. From anti-NXP2-antibody positive, 21/470 (4.46%) patients fulfilled criteria for IIM. Among 12 adult (57.14%) patients with IIM, 7 (58.33%) presented as polymyositis (PM) and 5 (41.6%) as dermatomyositis (DM) with median age at presentation of 45 (IQR: 25-58) years. Calcinosis and subcutaneous oedema were observed in 4 (19%) and 2 (9.52%), respectively; myalgia in 6 (28.6%); and distal muscle weakness in 5 (23.8%) patients. Malignancy at the time of diagnosis was observed in two adults with IIM (16.7%), one with DM (intraductal breast cancer), and another with PM (anaplastic large cell lymphoma). Remaining, 9 had juvenile dermatomyositis (JDM) with a median age of 4 (IQR: 3-8) years. Seven (77.8%) patients with JDM had skin rash specific for DM (heliotrope rash and Gottron's papule). None of the patients had cardiac and lung involvement, while GI symptoms, especially dysphagia, were present in 5 (23.8%) patients. During a median follow-up of 19 months (IQR: 12-26 months), 19/19 patients reported improvement and were in remission with treatment. CONCLUSION: The current study shows that adult DM patients with anti-NXP-2 autoantibodies have a unique clinical phenotype. Its presentation differs between adult and JDM, even in different parts of the world. Muscle weakness is mild and responds to treatment. Dysphagia needs more time and aggressive IS for improvement as compared to other muscle involvement. Key Points • Anti-NXP-2 antibody presentation varied from adult to child, as in different parts of the world. • In Indian adult patients, non-specific skin manifestations were more common, whereas in JDM, specific skin features were common. • There was less likely involvement of the lung and heart. But more risk of GI involvement requiring aggressive management. • Adult with anti-NXP-2 antibody should be screened for malignancy at the time of presentation.