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PURPOSE: We report a rare and challenging case of bilateral necrotizing scleritis in primary Sjögren's syndrome (pSS). METHODS: Retrospective case report. RESULTS: A 72-year-old diabetic, hypertensive female patient presented with sudden onset of painful red left eye and was noted to have a corneal ulcer with severe thinning. She was managed with topical fortified antibiotics and tissue glue and bandage contact lens. During subsequent follow-ups, she developed necrotizing scleral melts in both eyes. On investigations, antinuclear antibodies were positive in a dilution of 1:160 with 2+ speckled pattern, with antinuclear antibody line immunoassay showing anti SS-A/ Ro52 positive. In view of rapidly developing scleral thinning and impending perforation, she was started on intravenous methylprednisolone 1 g/day for 3 days, along with steroid-sparing immunomodulatory therapy (mycophenolate mofetil 500 mg twice a day). She showed a rapid response to therapy and is currently stable on tapering oral steroids and mycophenolate mofetil. CONCLUSION: This case underscores the unique presentation of pSS, characterized by bilateral necrotizing scleritis. The favorable outcome was attained through prompt immunosuppressive intervention and a collaborative, multidisciplinary approach. Further, this case report addresses a gap in the existing literature concerning pSS-related scleritis. It also emphasizes the crucial role of a rheumatologist in the comprehensive management of this condition.
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Introduction: Immunoglobulin (Ig)G4-related disease belongs to a rare compilation of conditions in rheumatology and may involve a multitude of organs. Amidst the central nervous system (CNS) presentation, involvement of spinal cord is rarer still. Case Report: Case 1: A 50-year-old male came with chief complaints of tingling sensation over both soles since 2 months associated with lower back ache and gait disturbance in the form of spastic gait. X-rays of spine were suggestive of a growth at the level of D10-D12 compressing the spinal cord with no focal sclerotic/lytic lesions, and MRI dorsolumbar spine showed dural tail sign. The patient underwent excision of dural mass, and histopathology revealed majority of plasma cells positive for IgG4. Case 2: A 65-year-old female came with complaints of cough, shortness of breath, and fever on and off since 2 months. No history of hemoptysis, purulent sputum, weight loss. On examination: There were bilateral rhonchi in the left upper zone. MRI spine showed focal erosion with soft-tissue thickening at the right paravertebral region extending from D5-9. The patient underwent surgery (fusion D6-8 and ostectomy D7 posterior rib resection D7 right side) with the right pleural biopsy and transpendicular intracorporal biopsy D7. Histopathology was compatible with findings of IgG4 disease. Conclusion: IgG4 tumors presenting in CNS itself is rare and rarer in spinal cord. Histopathological examination is central to diagnose and prognosticate the disease further as IgG4-related disease manifestations may recur without proper treatment.
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Biosimilars or similar biotherapeutic products are the biological products approved by regulatory agencies based on the demonstration of similarity in quality, safety and efficacy with reference biologics (or original biologics). Though biosimilars could be considered as interchangeable therapeutic alternatives over original biologics, there are concerns regarding their similarity in effectiveness and safety with reference product along with the level of evidence of similarity required for approval. The biosimilars, particularly, monoclonal antibodies that are developed based on the complex manufacturing processes, require stringent comparative evaluations. The Indian Regulatory Authorities in July 2012 developed the first guidelines for approval of similar biologics, which comprised requirements for the manufacturing process, quality evaluation, preclinical and clinical studies, as well as post-marketing studies. The 2016 guidelines, an update to previous guidelines, were released with the intent to provide a well-defined pathway at par with international regulations for the approval of similar biologics in India. This article highlights the key attributes of the 2016 Regulatory Guidelines and also describes the aspects such as interchangeability, nomenclature and labelling of similar biologics in India. Rigorous consideration is imperative for highly complex similar biologics of monoclonal antibodies on a case-to-case basis.
