Another One Bites the Joint: A Case of Reactive Arthritis in an Otherwise Healthy Hispanic Male

Reactive Arthritis is a spondyloarthritis following gastrointestinal or genitourinary infections, traditionally described as a triad of conjunctivitis, urethritis, and arthritis. It is important to identify patients with reactive arthritis as certain disease features portend a poorer prognosis. We present a case of reactive arthritis in a 36-year-old gentleman after a self-limited episode of gastroenteritis. A 36-year-old man presented to the Rheumatology clinic for a 2-week history of multiple arthralgias. The patient reported that one week before the onset of arthritis he experienced a self-limited episode of gastroenteritis. Succeeding, the patient noticed pain and swelling to right wrist, left elbow, and pain and swelling to left wrist. Patient was found to have an elevated ESR and he was prescribed Medrol dose pack and indomethacin which did not resolve symptoms. Patient denied rashes, oral ulcers, conjunctivitis, or constitutional symptoms. Examination was remarkable for swelling of right elbow, bilateral wrists, left knee, and ankles. There was no conjunctivitis, oral ulcers, or keratoderma blenhorragicum. Labs showed negative ANA and rheumatoid factor, sedimentation rate of 107, CRP 8.9, positive HLA-B27. Patient was diagnosed with reactive Arthritis. He was started on Prednisone and Sulfasalazine. Reactive arthritis is a spondyloarthritis that typically affects middle-aged men and is self-limited with remission being achieved by 6-18 months, while recurrence occurs in 25-50% of cases, especially in HLA-B27 positive patients. These patients could potentially develop long term disease resulting in enthesitis and destructive arthritis

Acquired Factor VIII Deficiency Presenting as Gross Hematuria in a Hispanic, Pregnant Patient with Previously Undiagnosed Connective Tissue Disease

Acquired factor VIII deficiency is a bleeding disorder caused by the presence of autoantibodies against clotting factor VIII. We report a case of a 24-year-old pregnant woman who presented with gross hematuria secondary to acquired factor VIII deficiency in the presence of a previously undiagnosed connective tissue disease. *is article includes a literature review of pregnancy-related cases of acquired factor VIII deficiency. We also reviewed various therapeutic approaches for the management of the acquired factor inhibitor which include achieving hemostasis and elimination of the inhibitor via immunosuppressive agents. *is case report describes the rare presentation of acquired factor VIII deficiency related to pregnancy and highlights the importance of considering a factor VIII inhibitor in the differential diagnosis of patients who present with bleeding and prolonged PTT during the peripartum and postpartum periods

Disease Severity and Response to Induction Therapy in Hispanic Patients With Antineutrophilic Cytoplasmic Autoantibody-Associated Vasculitis-Related Diffuse Alveolar Hemorrhage

Objectives We examined the response to induction therapy of Hispanic patients with antibody-associated vasculitis (AAV)-related diffuse alveolar hemorrhage (DAH). This study aimed to determine the severity of disease at presentation and the response to induction therapy in our patient population. Methods We retrospectively reviewed the clinical data of Hispanic patients hospitalized with antineutrophil cytoplasmic antibody (ANCA) vasculitis between October 1, 2010, and December 31, 2021. We identified 98 Hispanic patients hospitalized with AAV and 19 admitted with AAV-related DAH. The Birmingham Vasculitis Activity Score (BVAS) was obtained from all patients on presentation. Results Based on the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, 12 patients met the diagnostic criteria for microscopic polyangiitis (MPA) and seven met the criteria for diagnosing granulomatosis with polyangiitis (GPA). All patients received methylprednisolone therapy. Induction therapy consisted of cyclophosphamide pulse therapy (n=3), cyclophosphamide plus plasmapheresis (PLEX) (n=1), rituximab induction therapy (n=8), and rituximab induction plus plasmapheresis (n=6), and one patient received one dose of cyclophosphamide followed by rituximab plus plasmapheresis. The average BVAS was 25.53 at presentation. Survival at six months included 67% (n=2) treated with cyclophosphamide alone, 75% (n=6) treated with rituximab alone, and 50% (n=3) treated with rituximab plus PLEX. The patient who received an initial loading dose of cyclophosphamide followed by rituximab plus PLEX did survive for six months; however, the patient treated with cyclophosphamide plus PLEX did not have early survival. Conclusions Hispanic patients with ANCA-associated vasculitis present with a more severe disease burden at presentation based on BVAS. Approximately 37% of our patient population had early death (death at \u3c6 months) despite adhering to the standard of care for induction therapy. Due to the more significant disease burden at presentation, it is vital to include ethnic minorities in large clinical trials to help improve outcomes in these patient populations

Dermatomyositis- Related Intestinal Dysmotility

Dermatomyositis (DM) is an inflammatory myopathy (IIM) characterized by proximal muscle weakness and pathognomonic skin lesions. A 69-year-old woman with a recent diagnosis of DM 1 month prior, treated with corticosteroids and immunomodulators, presented to our inpatient rehabilitation with worsening dysphagia and constipation. At the time of our evaluation, physical examination was notable for erythematous papules over the metacarpophalangeal joints, proximal interphalangeal joints, elbows, and knees as well as a violaceous rash on the face. Muscle strength was diminished bilaterally with proximal distribution being affected greater than distal. Laboratory studies were notable for the creatine kinase (CK) level of 31 IU/l, antinuclear antibodies (ANA) by immunofluorescence of 1:80, and aldolase 4 u/l. The 11-antibody myositis panel was negative showed partially treated acquired IIM with perifascicular atrophy. During hospitalisation, she was found to have pulmonary embolism. She received enoxaparin 1 mg/kg subcutaneous BID. Soon after, she developed rectal bleeding. Colonoscopy showed a stercoral ulcer caused by chronic constipation. While dysphagia is common, being present in 25–50% of patients with DM, lower gastrointestinal problems involving the small and large intestine are rare and typically present as a late manifestation of the disease. Decreased peristalsis in the large colon can lead to constipation, impaction, and subsequent mucosal ulceration, and pressure necrosis induced by faecaloma formation. Although rare, our case highlights the importance of recognising gastrointestinal complications that DM can cause and the effects that those complications have on morbidity and mortality

Rituximab for steroid-resistant organising pneumonia in a woman with rheumatoid arthritis

Organising pneumonia (OP) is a form of interstitial pneumonia characterised by inflammation and scarring leading to obstruction within the small airways and alveoli. Practice guidelines recommend treatment of moderate to severe OP with glucocorticoids; however, there have been cases of steroid-resistant OP successfully treated with rituximab. We describe a case of a woman in her 20s with rheumatoid arthritis who presented with pleuritic chest pain, haemoptysis and dyspnoea on exertion and was diagnosed with OP after multiple radiographic images and biopsies. The patient failed numerous treatment regimens, including corticosteroids, antibiotics and mycophenolate, but was successfully treated with rituximab. This case highlights the importance of identifying new therapeutic agents that will minimise the use of glucocorticoids in the treatment of OP

Acute Eosinophilic Pneumonia in a Patient with Long Standing Behcet’s Syndrome

Background: Behcet’s syndrome (BS) is a multisystemic disease that frequently manifests as oral and genital ulcers. Pulmonary involvement occurs in approximately 18% of patients and can have complex clinical manifestations, including vascular disease, hemorrhage, infarctions, and organizing pneumonia. Broad-spectrum radiological findings like loss of lung volume, lung opacities, and indistinct nodular or reticular lesions have been described. There is little data on the association between Behcet’s disease and eosinophilic pneumonitis; the latest is usually characterized by eosinophilic infiltration of the lung parenchyma caused by a hypersensitivity reaction to an inhaled antigen. Patients usually present with nonproductive cough, dyspnea, fever, and constitutional symptoms, mimicking many pulmonary conditions. Diagnosis is based upon clinical criteria and bronchoalveolar lavage with \u3e25 eosinophils present. Management includes supportive care, antibiotics, and chronic immunosuppression, usually with high-dose steroids and mycophenolate. Case Presentation: A 40-year-old woman with a history of long-standing Behcet’s syndrome, eczema, and allergic rhinitis, chronically immunosuppressed with mycophenolate mofetil, presented with acute onset shortness of breath, weakness, and productive cough and fever. Initial workup evidenced eosinophilia and elevated inflammatory markers. PCR for COVID-19, mycoplasma, legionella, HIV, and respiratory cultures were negative. Chest X-ray showed bilateral airspace opacities associated with small pleural effusions that were also evident on CT angiogram; reactive hilar and mediastinal lymphadenopathy was also noted. The patient was started on oxygen supplementation, IV antibiotics with Levofloxacin, and mycophenolate mofetil was discontinued. Bronchoscopy with bronchoalveolar lavage was performed and was remarkable for increased eosinophils suggestive of acute eosinophilic pneumonitis. The patient was started on IV methylprednisolone with further symptomatic improvement. Conclusion: Due to its prothrombotic nature, pulmonary involvement in Behcet’s syndrome is mainly secondary to vascular disease. The association between BS and eosinophilic pneumonia is infrequent, making the diagnosis and treatment challenging due to the non-specific symptomatology and broad-spectrum differential diagnosis. Currently, there are no diagnostic criteria other than clinical and bronchoalveolar lavage with the presence of eosinophils. Immunosuppression therapy has shown to be beneficial, although there is still a lack of evidence regarding the length and dosing of glucocorticoid therapy

Two Cases of Autoimmune Syndrome Induced by Adjuvants (ASIA): A Multifaceted Condition Calling for a Multidisciplinary Approach

Silicone implants have been used for cosmetic enhancement and reconstructive purposes for over 60 years. Despite assiduous efforts to ensure safety, there is continuous evidence that they are not as biologically inert as previously postulated. We present two cases of autoimmune syndrome induced by adjuvants (ASIA) in Hispanic women. The first patient developed biopsy-proven immune-mediated necrotizing myopathy that was successfully treated with the combination of silicone explantation along with immunosuppressive therapy. Findings after implant removal demonstrated rupture and leakage of silicone from gluteal implants. The second patient developed autoimmune hemolytic anemia in the setting of a ruptured silicone breast implant. Similarly, the patient was treated with corticosteroids followed by breast implant removal with complete resolution of symptoms. The successful treatment of these patients was achieved by collaboration between rheumatology and plastic surgery, which emphasizes the need for a multidisciplinary approach in the diagnosis and management of patients with ASIA

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

Two Cases of Autoimmune Syndrome Induced by Adjuvants (ASIA): A Multifaceted Condition Calling for a Multidisciplinary Approach

Silicone implants have been used for cosmetic enhancement and reconstructive purposes for over 60 years. Despite assiduous efforts to ensure safety, there is continuous evidence that they are not as biologically inert as previously postulated. We present two cases of autoimmune syndrome induced by adjuvants (ASIA) in Hispanic women. The first patient developed biopsy-proven immune-mediated necrotizing myopathy that was successfully treated with the combination of silicone explantation along with immunosuppressive therapy. Findings after implant removal demonstrated rupture and leakage of silicone from gluteal implants. The second patient developed autoimmune hemolytic anemia in the setting of a ruptured silicone breast implant. Similarly, the patient was treated with corticosteroids followed by breast implant removal with complete resolution of symptoms. The successful treatment of these patients was achieved by collaboration between rheumatology and plastic surgery, which emphasizes the need for a multidisciplinary approach in the diagnosis and management of patients with ASIA

A Very di-Still-ed Diagnosis- Adult-Onset Still’s Disease Presenting in a Middle-Aged Hispanic Patient

Background: Adult-Onset Still’s Disease (AOSD) is a systemic inflammatory disorder characterized by daily high fevers, arthritis, evanescent rash, and leukocytosis (1). Patients can present without typical manifestations and pose a challenging differential. We present a case of a 52-year-old gentleman with a one-year history of recurring fever, lymphadenopathy, and weight loss diagnosed with AOSD. This case highlights the diagnostic challenge that AOSD poses and the strategies to help aid in the diagnosis. Case Presentation: A 52-year-old gentleman presented to the ED for a 2-week history of fever associated with chills and bone pain. He reported that he has been having intermittent fever, weight loss, night sweats, and rash for the past year with prior workup being unrevealing. He endorsed swollen glands and fatigue but denied productive cough, chest pain, gastrointestinal, urinary, or neurological symptoms. On physical examination, the patient was febrile at 101.9 deg F, tachycardic 121 BPM, and RR 21 br/min. He appeared cachexic, with dry oral mucosa, palpable lymphadenopathy, and bilateral knee tenderness. Laboratories were remarkable for WBC 22.3 th/mm3, hemoglobin 11.1 gm/dL, platelet 513 th/mm3, sedimentation rate 120 mm/h, CRP 23 mg/dL, lactic acid 0.89 mmol/L, ferritin level 28,595.9 ng/mL, and LDH 603 IU/L. Peripheral smear revealed reactive neutrophilic leukocytosis. Infectious etiology, including SARS Covid-19PCR, HIV, blood cultures, lumbar puncture with CSF analysis, and QuantiFERON gold were negative. Autoimmune workup was unrevealing. CT Chest/Abdomen demonstrated moderate pleural effusions and reactive bilateral hilar, mediastinal, and retroperitoneal lymphadenopathy, and hepatomegaly. CT-guided biopsy of the left inguinal lymph node showed benign follicles with mixed B and T cells. Flow cytometry showed increased granulocytes and eosinophils without immunophenotypic abnormalities to suggest hematologic malignancy. After excluding infectious and malignant causes, rheumatology was consulted. Based on symptomatology, laboratory, and radiographic findings, a diagnosis of AOSD was entertained. Yamaguchi\u27s criteria supported the diagnosis with four major and four minor criteria met (2). The patient was started on prednisone 1 mg/kg with excellent response. Conclusion: AOSD is a diagnosis of exclusion, and the appropriate clinical scenario should warrant further investigation. AOSD should be in the differential after careful workup and excluding infectious etiology, malignancy, and other connective tissue diseases