A Case of Acute Necrotizing Myopathy in a Patient with Systemic Lupus Erythematosus.

BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disorder affecting multiple organ systems with a wide spectrum of clinical presentation and associated with positive serologies. Musculoskeletal involvement in patients with SLE is relatively uncommon, occurring in approximately 4% to 16% of cases. Some patients can develop necrotizing myopathy without myositis. MRI in patients with SLE-associated necrotizing myopathy usually shows interstitial edema, while muscle biopsy often shows type 2 muscle fiber atrophy. We herein report an unusual case of acute necrotizing myopathy in a patient recently diagnosed with SLE. This case report focuses on the pertinent features related to the diagnosis of this patient while highlighting the management of acute necrotizing myopathy. CASE REPORT A 30-year-old African American woman presented to the Emergency Department with skin rashes, myalgia, polyarthralgia, and muscle weakness resulting in the inability to walk, 2 weeks after being diagnosed with SLE. Laboratory analysis showed elevated creatine kinase and myoglobin. She was found to have both MRI and biopsy findings suggestive of necrotizing myopathy. She was treated with mycophenolate mofetil and steroids, with an improvement of muscle strength and decrease in creatine kinase over a 2-week period. CONCLUSIONS Immune-mediated necrotizing myopathies are a rare group of debilitating myopathies that can be associated with SLE. The diagnosis of necrotizing myopathy in patients with SLE requires a high index of suspicion and careful work-up to establish a diagnosis. Muscle biopsy often shows type 2 muscle fiber atrophy. Immunosuppressive therapy is the mainstay of treatment, and early initiation of immunotherapies is associated with an improvement in patient outcomes.

The dilemma: scleroderma renal crisis vs lupus nephritis in a patient with mixed connective tissue disorder.

INTRODUCTION: Mixed connective tissue disorder (MCTD) is a rare connective tissue disorder characterized by features of systemic lupus erythematosus, dermatomyositis, systemic sclerosis, and rheumatoid arthritis. MCTD is associated with an elevated antibody titer to U1 small nuclear ribonucleoprotein. CASE DESCRIPTION: A 49-year-old man presented to the emergency department for evaluation of worsening shortness of breath with associated for bilateral hand pain and swelling associated with morning stiffness which was initially thought to be related to systemic lupus erythematous (SLE). He was also found to have a positive autoantibody, and he was later diagnosed with MCTD complicated by scleroderma renal crisis. CONCLUSION: MCTD is a rare connective tissue disorder with overlapping features of SLE, dermatomyositis, systemic sclerosis, and rheumatoid arthritis. The diagnosis of MCTD requires a high index of suspicion and careful workup. Immunosuppressive therapy is the mainstay of treatment that improves patient outcomes.

Missing in action.

Ectopically located parathyroid adenoma is one of the major causes of persistent and recurrent hyperparathyroidism and hypercalcemia. Approximately 0.3-8% of parathyroid adenoma is found in an ectopic location. Ectopic parathyroid adenomas are uncommon causes of persistent hypercalcemia and can be present at uncommon locations, including the hypoglossal nerve, the posterior triangle of the neck, axilla, and pericardium 3. A high index of suspicion is warranted when we see persistently elevated levels of parathyroid hormones (PTHs) and calcium levels post parathyroidectomy. Here, we present a patient who persistently had elevated calcium and PTH levels after parathyroidectomy.

Acute Fatty Liver of Pregnancy.

BACKGROUND Acute fatty liver of pregnancy (AFLP) is a rare obstetric emergency resulting from microvesicular infiltration of the liver by fat, leading to liver failure. It usually presents at 36 weeks of gestation, and risk factors include twin pregnancy and low BMI. The presentation of AFLP is nonspecific, requiring a high index of suspicion. The Swansea Criteria is used to aid diagnosis. CASE REPORT Case 1: A 23-year-old woman, G1P0 at 39 weeks of gestation, presented to the hospital with a 1-week history of fever, nausea, vomiting, and diarrhea. Examination revealed a gravid uterus with generalized abdominal tenderness. Laboratory investigations revealed elevated liver enzymes, with elevated total bilirubin and an INR of 1.26. CBC showed leukocytosis. Abdominal ultrasound was normal. Workup for other etiologies, including acetaminophen and salicylate overdose and infections, was negative. The Swansea score for AFLP was 8, confirming the AFLP diagnosis. An emergency Cesarean-section was performed, causing liver enzymes to improve over 3 days. Case 2: A 41-year-old woman, G1P1 with a twin gestation at 36 weeks, presented with a 3-day history of abdominal pain. She was jaundiced, with right upper quadrant tenderness. Laboratory investigations showed elevated liver enzymes and total bilirubin, with an INR of 1.26. Workup for viral hepatitis and autoimmune etiology was negative. Salicylate levels were within normal limits. She met criteria for AFLP and underwent emergency Cesarean-section. Liver enzymes improved over 4 days. CONCLUSIONS AFLP is a potentially life-threatening medical condition. From our experience, prompt diagnosis and early delivery leads to improved maternal and fetal outcomes.