A Retrospective Study on Antinuclear Antibody Patterns in Systemic Lupus Erythematosus Patients and Its Correlation With Serological Markers.

INTRODUCTION: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Detection of antinuclear antibodies (ANAs) aids in the diagnosis of SLE. The indirect immunofluorescence (IIF) assay is often used a routine screening test for the detection of ANA. The pathogenic role and significance of various patterns produced in IIF is yet to be explored. AIM: This study aimed to detect ANA patterns generated by IIF and correlate these patterns with specific antibodies detected by line immunoassay. We also investigated the significance of each ANA pattern and its association with specific serological SLE markers, such as complement molecules, anti-dsDNA, antiphospholipid antibody, and C-reactive protein (CRP), along with associations with direct Coombs test (DCT). MATERIALS AND METHODS: We conducted a retrospective study that included 204 patients newly diagnosed with SLE according to the European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) criteria. The detection and pattern determination of ANA was performed by IIF using HEp-20-10. Furthermore, line immunoassay was performed, and the antibody profile of each sample was obtained. Other immunodiagnostic markers were analyzed, including C3, C4, anti-dsDNA, antiphospholipid antibodies (anti-cardiolipin antibodies, anti-beta-2-glycoprotein I, and lupus anticoagulant), CRP, and DCT. RESULTS: Of the 204 samples, the most frequent ANA pattern observed was nucleus speckled (52.9%), followed by nucleus homogenous (27.5%), mixed (13.7%), and cytoplasm speckled (5.9%). The nucleus homogenous pattern showed the most pathogenic immune profile due to its close association with markers of disease activity, namely, high anti-dsDNA titer, low C3 level, and DCT positivity.  Conclusion: This study showed that the most common pattern associated with SLE is nucleus speckled, followed by the nucleus homogenous pattern. Based on associations with specific serological markers, the nucleus homogenous pattern may be linked to a high disease activity in SLE.

Clinical profile of osteonecrosis in systemic lupus erythematosus - Experience from a tertiary care centre in South India.

INTRODUCTION: Osteonecrosis or Avascular necrosis of bone (AVN) is a well recognized complication of systemic lupus erythematosus (SLE) leading to significant morbidity. METHODS: We did a cross sectional descriptive study in cohort of SLE patients, on regular follow-up at our Rheumatology OPD over a period of 5 years from 2012 to 2017. RESULTS: Of the total 415 SLE, 5.1% (n = 21) patients were diagnosed to have osteonecrosis. The mean age was 32.8 ± 7.6 years. Male: female were 1:4.2. Mean time interval between the onset of SLE and diagnosis of osteonecrosis was 4.1 ± 2.7 years. Pain (100%) was the most common presenting symptom followed by limping gait (42.8%). Most common site affected by osteonecrosis was femoral head (80.9%) (n = 17). 14.3% (n = 3) had multifocal involvement. The most common systemic involvement was musculoskeletal system (80.9%). In total 28.5% had secondary antiphospholipid syndrome. Mean SLEDAI-2K at the time of diagnosis of osteonecrosis was 5.3 ± 2.9. Hypertension 19%, hypothyroidism 9.5%, osteoporosis 24%, and chronic HCV infection 4.7% were the associated comorbidities. The most common stage by imaging at diagnosis was stage IV (38%), followed by 24% stage V, 19% stage III and 9.5% stage II and 9.5% stage VI. Medical management include bisphosphonates (100%), statins (90.4%) and anticoagulant therapy (28.5%), while 9.5% received core decompression surgery and 14.3% underwent total hip replacement. The mean daily dose of prednisolone at diagnosis of osteonecrosis was 8.5mg (range 5-20mg). CONCLUSION: This study described the prevalence and epidemiology of osteonecrosis in our cohort of SLE patients.

Aggressive eosinophilic granulomatosis with polyangiitis and transverse sinus thrombosis.

We report a case of a patient with eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) who succumbed to respiratory failure complicated by sepsis, pneumonitis and transverse sinus thrombosis. In the absence of antineutrophilic cytoplasmic antibodies positivity, a clinical diagnosis was made based on The American College of Rheumatology-1990 criteria. Glucocorticoids, cyclophosphamide, rituximab and intravenous immunoglobulin were administered at different stages. The patient recovered momentarily after each cycle of treatment, but his vasculitis progressed and he eventually died. Cardiac or renal involvement, which is more commonly associated with an adverse outcome, was not present.

Autologous platelet rich plasma in the management of non-healing vasculitic ulcers.

AIM: To determine the role and efficacy of platelet rich plasma (PRP) in the healing of recalcitrant vasculitic ulcers and to study the rapidity of ulcer healing with respect to platelet concentration in the prepared PRP. METHODS: This is a pilot study which included 20 patients with biopsy-proven vasculitic ulcers after excluding human immunodeficiency virus, uncontrolled diabetes mellitus, hypertension, obesity, active smoking and platelet abnormalities in peripheral smear. PRP was prepared from whole blood by centrifugation and activated using 10% calcium chloride. Activated PRP was applied over the ulcer by spraying using a syringe. Weekly dressing was done until the ulcer healed and healing was monitored using the Leg Ulcer Measurement Tool (LUMT). RESULTS: The total number of patients were 20 (male four; female 16). The mean age was 34.5 ± 15.36 years (2.0 SD). Mean duration of illness was 8.6 ± 2.9 months(2.0 SD). Ulcers were located in the lower third of the leg (n = 13), ankle (n = 4) and foot (n = 3) in the descending order of frequency. On average, epithelization of the wound occurred at 4.4 weeks. Faster healing was seen in mixed connective tissue disease (average 3.5 weeks) and delayed healing in systemic lupus erythematosus (average 4.7 weeks). Average platelet count obtained in the PRP was 5.52 lakhs/mm3 to 18.6 lakhs/mm3 with an increase in platelet count of 3.4- to 6-fold, respectively. Duration of healing corresponded with the platelet concentration in the prepared PRP. All the 20 patients showed complete healing within 6 weeks. CONCLUSION: PRP is a safe, effective, outpatient procedure for the treatment of chronic recalcitrant vasculitic ulcers which improves the quality of life of patients and reduces their financial burden.