Browsing by Author "Quresh, Quretul"
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Item ANCA positivity in the case of acute bacterial endocarditis(2024-03-21) Forsyth, Alyssa; Quresh, QuretulBackground: The case of ANCA positivity in an individual with bacterial endocarditis demonstrates the importance of thorough testing and labs prior to treatment. This case draws attention to a diagnostic conflict, as starting treatment on immunosuppressants, as would be the standard therapy for ANCA-associated vasculitis (AAV), could exacerbate the bacterial endocarditis and carry severe consequences. In this case of glomerulonephritis and associated endocarditis, the patient presented with several symptoms which mimicked AAV. Thus, it is important for providers to include cultures in routine assessments of ANCA-positive cases to allow for thorough evaluation and appropriate therapy. Case information: A 60-year-old morbidly obese male presented for right knee pain. His medical history includes hypertension, congestive heart failure (CHF), osteoarthritis, atrial fibrillation, and stage IV chronic kidney disease (CKD). The patient denied history of smoking, drugs, or alcohol use. He has no family history of autoimmune disease. Upon evaluation, the patient was found to have a deep vein thrombosis (DVT) with leg pain, as well as paroxysmal atrial fibrillation. He was started on Coumadin for anticoagulation therapy. During his hospital stay, his creatinine levels climbed from 2.0 mg/dL to 6.3 mg/dL and was given a preliminary diagnosis of acute renal failure on top of his preexisting CKD. His renal decline prompted a kidney biopsy, which revealed focal necrotizing and crescentic glomerulonephritis with C3 dominant deposition. Urinalysis showed proteinuria and was positive for active urinary sediment. Bloodwork was remarkable for MPO-ANCA positivity (MPO-ANCA 1:640). Transesophageal echocardiogram (TEE) and blood culture revealed an aortic valve vegetation positive for methicillin-sensitive staphylococcus aureus (MSSA), and the patient was started on broad-spectrum antibiotic therapy. Conclusions: The details of this case prompt reflection on the diagnostic steps taken by providers when presented with an ANCA-positive patient. The standard protocol taken in the treatment of ANCA vasculitis differs tremendously from and could even have devastating consequences in the case of infectious endocarditis. Therefore, careful evaluation for bacterial endocarditis in the case of ANCA positivity should be incorporated into the diagnostic process. Most of the current literature represents cases of c-ANCA positivity in subacute infectious endocarditis (IE), often with Streptococcus viridans responsible. This case is unique in that the patient was positive for MPO-ANCA and not PR3 and the causative organism was MSSA. While several cases of MSSA IE with ANCA positivity have been reported, this combination of MSSA endocarditis and isolated MPO-ANCA positivity has only been represented one other time in the literature to our knowledge.Item A case of inclusion body myositis masquerading as statin-induced myopathy(2023) Fatima, Fariya; Quresh, Zehratul; Ansari, Mahira; Moizuddin, Mohammed; Quresh, QuretulBackground: Inclusion body myositis (IBM) is a common acquired myopathy in individuals older than 50 years of age. This idiopathic inflammatory myopathy carries an insidious progression with frequent delays in diagnosis and a high incidence of misdiagnosis resulting in significant morbidity and disability for affected individuals. Clinical features include asymmetric weakness predominantly affecting the quadriceps and/or finger flexors, and a slow, progressive course leading to loss of ambulation and dysphagia. Creatinine kinase (CK) levels are usually less than 10 times normal and erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) fall within normal limits. Myositis-specific autoantibodies are typically absent in patients with IBM; however, highly specific positive cytoplasmic 5'-nucleotidase 1A (cN1A) autoantibodies can help distinguish IBM from other forms of myositis, such as polymyositis. There is an association of IBM with autoimmune diseases, such as Sjögren's syndrome, sarcoidosis, and some lymphoproliferative disorders. Unlike many other autoimmune diseases, the condition typically affects males more frequently with a male-to-female ratio of approximately 3:1. Mean age of symptom onset ranges from 61 to 68, with over 20% of patients developing symptoms in their forties. Case presentation: A 71-year-old male patient with a long-standing history of hypertension and dyslipidemia was evaluated for complaints of progressively worsening proximal muscle weakness, fatigue, and dyspnea for more than 2 years in duration. He had been prescribed 40 mg of atorvastatin and was presumed to have statin-induced myopathy after taking the drug for 1 year. Physical examination revealed an otherwise healthy male with proximal muscle weakness with stable vital signs. CK was within normal limits and CRP was 6.6. He was noted to have a negative ANA screen extensive myositis and HMGCR antibody, but was found positive for cytoplasmic 5'-nucleotidase 1A (cN1A) autoantibodies, thus confirming suspicion of IBM. MRI of the thigh demonstrated generalized muscular atrophy with no active inflammation. Given the prolonged nature of the disease in the patient, no active inflammation was noted. He is responding well to tapering doses of steroids and other immunomodulatory options being discussed with him. Conclusion: This case illustrates the importance of appropriate recognition of IBM, and the challenges associated with diagnosis and management. This acquired myopathy may be mistaken for other conditions such as statin-induced myopathy and should be considered in the evaluation of progressive muscle weakness in individuals over 50 years of age. Appropriate intervention is essential to prevent progressive disability with rapid loss of strength and function in affected individuals.Item A Rare Case of Anti-NXP2 Antibody Positivity in Juvenile Dermatomyositis – A Case Study(2024-03-21) Bosques, Rebeca; Quresh, QuretulBackground: Dermatomyositis (DM) is one of the many subcategories of Idiopathic Inflammatory Myositis (IIM), a treatable group of myopathies. Antibodies serve as a diagnostic tool with prognostic indicators. DM-specific antibodies include anti-NXP2, anti-MDA5, anti-TIF1gamma, Anti-Mi2, Anti- SAE, and anti-SRP. These DM-specific antibodies are mutually exclusive suggesting the idea that each plays a specific role in shaping different phenotypes. 1-17% of adult DM/ polymyositis (PM) and 23-25% of juvenile dermatomyositis (JDM) present with a positive anti-NXP2 antibody. Clinically, positive anti-NXP2 antibody DM patients present with a rash, debilitating muscle weakness, calcinosis, dysphagia, and a higher risk of malignancy. As compared to patients with negative anti-NXP2 antibody DM, positive anti-NXP2 antibody DM patients were younger at the age of onset, with shorter duration between symptom onset to diagnosis, and no significant sex diseases. The prognosis for positive anti-NXP2 antibody DM depends on the complications developed. The worrisome complications which lead to a poor prognosis are calcinosis and malignancies. Case Information: 22 y/o female was referred to rheumatology for evaluation of Systemic Lupus Erythematosus (SLE). For 3 years prior, the patient experienced muscle and generalized weakness, and myalgia in the legs leading to difficulty climbing stairs. At the time of referral, the patient showed CPK 34, ANA positive anti-dsDNA +12, BUN 15, creatinine 0.70, GFR 123, albumin 4.6, AST 15, ALT 6, ESR 35, WBC 6.5, hemoglobin 10.8, platelets 320. On physical exam, upper and lower extremities showed 4/5 muscle strength. An autoimmune panel to test myositis specific antibodies was ordered. Which showed positive anti-NXP2 21, positive ANA screen IFA, actin antibody (IgG) 34, thyroid peroxidase antibodies 267 IU/mL, ANA titer 1:80. 20 days after, the patient had difficulty getting out of bed with profound generalized weakness. The patient had developed a rash in the upper chest and abdomen. The diagnosis of juvenile dermatomyositis (JDM) was made. Patient admitted to hospital treated with 1 g Methylprednisolone for 3 days and tapered 1 mg/kg body weight. Care with neurology was coordinated to start IVIG treatment at 2 g/kg body weight. The patient was found to have positive acetylcholine receptor antibodies and diagnosed with Myasthenia gravis. The patient was given IVIG for 5 days. The patient was also found to have thyroid nodule swelling s/p biopsy concerning Hurthel cell cancer. On follow-up one month later, the patient stated a return of muscle strength with no rashes. The patient was advised to get an EMG and follow up with neurology, the dose of prednisone was decreased to 40 mg daily and advised to take vitamin D 50,000 units weekly. Conclusion: Anti-NXP2 antibody positive JDM has a poor prognosis compared to other subcategories. This stems from increased risk of malignancies and calcinosis leading to fatalities. Our patient was a rare case with anti-NXP2 antibody positive JDM, Myasthenia gravis, and Hurthle cell cancer. There is an increased need in data and research regarding NXP2 antibody and its heterogenous prognosis with DM patients.