That Which Keeps on Giving - A Case of Class IV Diffuse Proliferative Lupus Nephritis in a Hispanic Woman with underlying Systemic Lupus Erythematosus

Background: Glomerulonephritis is the primary cause of morbidity and mortality of systemic lupus erythematosus (SLE). Lupus nephritis is characterized by immune complex deposition in the mesangium leading to complement activation and hypocomplementemia. Studies show that up to 60% of adults with lupus develop renal involvement and it has been well established that Hispanic patients show poorer outcomes than Caucasians despite advances in treatment. Preserved kidney function with new-onset proteinuria should raise clinical suspicion for acute lupus nephritis. Further evaluation with a kidney biopsy is paramount in establishing a diagnosis, helping to define treatment strategy, and determining response to treatment. Case Presentation: A 41-yo-Hispanic-Woman with a PMH SLE without previous renal involvement, secondary Sjogren\u27s, hypertension, heart failure and cirrhosis presented to the ER with a worsening SOB, difficulty swallowing, and anasarca over two weeks. On evaluation, the patient was hypertensive, tachypneic, had positive JVD, wheezing in lung bases, +1 pitting edema in the lower extremities and skin hyperpigmentation on the face, neck, and upper extremities. Laboratory studies revealed leukocytosis of 11.1 th/uL, Hgb 10 gm/dL, platelets 192 th/uL, Cr 0.9 mg/dL, BUN 17 mg/dL, bicarbonate 19 mmo/L, sedimentation rate 94 mm/hr, and CRP 4.5 mg/L. A urinalysis was performed, which showed 3+ proteinuria with hematuria with a subsequent protein to creatinine ratio demonstrating 2,000 mg/gm. The workup for nephritic range proteinuria revealed an ANA 10U, anti-dsDNA 2.7 IU/mL, negative ANCA, anti-cardiolipin Ab IgMMPL, anti-cardiolipin Ab IgG 14 GPL, and non-reactive HIV, Hepatitis C, and Hepatitis B panels. Despite negative glomerulonephritis workup including anti-dsDNA antibody, a kidney biopsy was pursued and revealed class IV diffuse proliferative lupus nephritis with a component of thrombotic microangiopathy. Conclusion: Kidney biopsies are imperative when establishing a cause of new-onset proteinuria in a patient with a history of SLE. The goal of treatment is induction with immunosuppressive agents to reduce kidney inflammation promptly and prevent flares, decreasing the long-term risk of renal failure. Despite early recognition strategies and advances in treatment, Hispanic patients are likely to be diagnosed with more severe disease at presentation, specifically with class IV or V lupus nephritis. Subsequently, these patients are more likely to develop chronic renal failure compared to Caucasian patients. This case highlights the importance of screening urinalysis for proteinuria for early detection of renal involvement in patients with SLE

A Whole CLOTTA Pain: A Case of IVC Thrombosis Presenting as Severe Abdominal Pain in a Patient with Anti-Phospholipid Syndrome

Background: Anti-phospholipid syndrome (APS) is an immune-mediated condition characterized by the presence of antiphospholipid antibodies in the setting of venous and arterial thrombosis and or pregnancy loss [1]. APS can be a primary syndrome or can be associated with connective tissue diseases such as systemic lupus erythematosus. The goal of treatment in patients with APS is to decrease the risk of thromboembolic events and the standard of care is warfarin therapy. We describe a case of a 42-year-old gentleman with a history of APS on anticoagulation with apixaban who presented to the hospital with severe abdominal pain secondary to infrarenal IVC thrombosis. This case highlights the importance of appropriate anticoagulation in patients with APS. Case Presentation: A 42-year-old man with a past medical history of APS presented to the hospital with a 7-day history of bilateral, dull, non-radiating, generalized abdominal pain. He denied any associated diarrhea, constipation, changes in urination, fever, nausea, or vomiting. Physical examination was notable for tachycardia with HR 110s, BP 150/100 mmHg, and a distended, tender abdomen with visible abdominal varicosities. Labs revealed a WBC 20,000 th/uL, creatinine of 2.0 mg/dL, AST 15 IU/L, ALT 20 IU/L, alkaline phosphatase 101gm/dL, INR of 1.12, and a PTT of 62.2 secs. CT abdomen and pelvis without contrast showed severe narrowing and occlusion of the infrarenal inferior vena cava over a length of 6-7 cm with dilated tortuous varices. Liver US demonstrated normal-appearing flow in hepatic veins and the intrahepatic IVC. The patient was evaluated by surgery however recommendations were made to treat the patient medically with a heparin drip with a bridge to warfarin. Discussion: Anticoagulation therapy in patients with APS is important as these patients can develop severe, life-threatening thrombotic events. Warfarin therapy is superior to direct oral anticoagulant (doac) therapy in preventing thrombotic events in these patients. Given the episode of thrombosis on doac therapy, our patient will be on lifelong warfarin therapy with an INR goal of 3.5. He has not had a recurrence of thrombotic events

Interrupting the Axis: A Case of IgG-4 Related Hypophysitis in a Young Hispanic Male

Background: Hypophysitis refers to inflammation of the pituitary gland that can lead to the disruption of the hypothalamic-pituitary-adrenal axis. Primary hypophysitis has five variants differentiated by histologic findings: lymphocytic, granulomatous, xanthomatous, IgG4-related, and necrotizing. IgG4-related hypophysitis is very rare and is commonly a manifestation of a multi-organ systemic disorder. We present a case of a 43-year-old man presenting with severe headache and symptomatic hyponatremia in the setting of isolated IgG-4 related hypophysitis. Case Presentation: A 43-year-old male presented with a two-day history of severe posterior headache associated with several episodes of vomiting, photophobia, generalized weakness and fatigue. Patient had no history of headaches and denied any recent trauma, focal weakness, altered mentation, changes in vision or seizure activity. Vitals on admission were remarkable for BP 179/99 mmHg. Neurological examination was non-focal, cranial nerves were grossly intact. Labs showed unremarkable CBC, sodium 117 mmol/L, potassium 3.3 mmol/L, chloride 84 mmol/L, glucose 244 mg/d, TSH 0.6 uIU/mL, cortisol 4.6 ug/dL, urine osmolality 540, serum osmolality 259 and prolactin level 7.8. CT head without contrast showed a mass-like enlargement of the Sella measuring 1.6 cm x 1.4 cm suspicious for macroadenoma. Patient was started on hypertonic saline, fludrocortisone, hydrocortisone, and salt tablets in the setting of suspected macroadenoma. Once the patient was stabilized, he underwent transsphenoidal resection of pituitary lesion. Pathology of the lesion showed a small area rich in plasma cells with positive IgG4 immunostaining in most of the plasma cells, consistent with IgG4-related pituitary hypophysitis. Conclusion: IgG4 hypophysitis usually presents in a context of multiple organ IgG4 involvement, however isolated hypophysitis has been reported. The patients present with mass effect symptoms and/or anterior hypopituitarism. When histologic diagnosis is obtained via biopsy, IgG4 hypophysitis can be managed exclusively with corticosteroids

Patent foramen ovale, deep venous thrombosis and stroke; a paradoxical embolism in an 80-year-old male

Background: A patent foramen ovale (PFO) is a congenital cardiac malformation describing a shunt in between the atrial walls. The overall incidence of a PFO is around 27.3% with a progressive decrease to 25.4% in the 4th and 8th decades. Once it has been established that a patient with an ischemic stroke/transient ischemic attack (TIA) has a PFO and other sources of the stroke have been ruled out, it is imperative to consider deep vein thrombosis (DVT) as the source of a paradoxical embolus. Case Presentation: 80-year-old gentleman with a history of right internal carotid occlusion of 80-90% status post right internal carotid endarterectomy. Presented to the emergency department (ED) for sudden left-sided weakness, left facial droop, slurred speech, and dizziness. Upon initial evaluation he was asymptomatic; NIH stroke score 1. Magnetic resonance imaging of the brain showed important watershed infarcts. Cerebral angiogram found 50% concentric stenosis of the right middle cerebral artery (MCA) with minimal lineal filling defect in the stenotic segment. The filling defect cleared after injection of intra-arterial integrillin. Post procedure, patient was started on heparin drip. Cardiology was consulted for suspected paroxysmal atrial fibrillation and a transesophageal echocardiogram (TEE). Two days after the post cerebral angiogram, the patient began to complain of severe right leg pain. He was noted to be tachycardic and hypoxemic. The venous doppler of the leg revealed a DVT and subsequent CT chest angiography revealed bilateral pulmonary embolism (PE). TEE results showed a positive agitative saline test with defect in the intra-atrial wall. The patient was on heparin drip and transitioned after 7 days to oral anticoagulation. Loop recorder was installed with possible PFO correction by cardiology in the outpatient. Patients\u27 symptoms improved and he was discharged to inpatient rehab. Conclusion: Although rare a paradoxical embolus should be considered in patients presenting with a stroke/TIA, PFO, and an unidentified source of embolus. Although this patient did have 50% occlusion of the R MCA, we cannot fully exclude a PFO-related stroke. The discovery of a DVT, and bilateral PEs supports the high suspicion for PFO-related stroke in the form of paradoxical embolus

It Is Not Pneumocystis jiroveci (PCP), It Is Cyclophosphamide-Induced Pneumonitis

Cyclophosphamide (CYC) is an immunosuppressive medication used to treat life-threatening complications of various rheumatic diseases like vasculitis and systemic lupus erythematosus. A rare side effect of this medication is pneumonitis, which occurs in less than 1% of patients. We describe a case of an 83-year-old woman with a past medical history of microscopic polyangiitis, who presented with progressive dyspnea at rest, exacerbated on exertion, and associated with orthopnea that was attributed to CYC-induced pneumonitis. Three months before this presentation, the patient was diagnosed with antineutrophil cytoplasmic antibodies (ANCA)-positive pauci-immune crescentic and necrotizing glomerulonephritis and started on CYC. On admission, a computed tomography (CT) chest showed worsening bilateral ground-glass opacities in a mosaic distribution and inter and intralobular septal thickening, not present on the CT performed three months prior. The patient underwent an extensive workup, which included an echocardiogram, bronchoscopy with bronchoalveolar lavage, and viral respiratory panel to rule out infectious and cardiac pathologies. She was started on empiric treatment with antibiotics and diuretics, however, despite these interventions, she continued with respiratory distress. A multidisciplinary team convened, and the diagnosis of CYC-induced lung injury was entertained. The CYC was discontinued, and the patient was started on prednisone with significant improvement in symptoms. This case highlights the importance of recognizing CYC as a rare cause of interstitial pneumonitis. When considering CYC-induced lung toxicity, other etiologies, such as opportunistic infections, cardiac etiologies, and diffuse alveolar hemorrhage, should be ruled out