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

A Rare Cause of Lymphadenopathy in a Young Hispanic Female: Unmasking Recurrent Nodal Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can present with painless bilateral symmetrical cervical lymphadenopathy, mimicking lymphomas. RDD is characterized by excessive tissue infiltration by dendritic cells, macrophages, or monocyte-derived cells, with a histopathologic diagnosis based on the presence of CD68+, CD163+, and S100+ histiocytes, which differentiate it from other histiocytic neoplasms. In this case report, we present a young Hispanic female with recurrent subcutaneous growths and lymphadenopathy, initially thought to be lymphoma, who was diagnosed with RDD after a significant diagnostic workup. Treatment initially consisted of surgical excision; however, due to recurrence, the patient was successfully treated with corticosteroids and a steroid-sparing agent, 6-mercaptopurine, with significant improvement in symptoms. RDD should be considered a differential diagnosis for patients with cervical lymphadenopathy, and an interdisciplinary approach is essential to managing this rare disorder effectively. The report highlights the need for an interdisciplinary approach to managing this rare disorder effectively and underscores the importance of multimodal treatment in disease suppression. As a rare disease with slow advancement of defined guidelines for diagnostic and treatment strategies, this case report adds to the existing literature on RDD

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

The Importance of a Broad Differential Diagnosis: Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

Introduction: Mixed cryoglobulinemia syndrome (MCS) is a systemic inflammatory syndrome affecting small-medium sized vessels due to the presence of type II or III cryoglobulins in the serum. MSC can manifest as systemic vasculitis with symptoms varying from weakness, arthralgia, palpable purpura, peripheral neuropathy, and renal involvement. The most common cause of MCS includes lymphoproliferative disorders, autoimmune diseases and viral infections, with hepatitis C virus (HCV) being the most common etiology. Case Presentation: A 60-year-old lady was referred to our office for evaluation of rheumatoid arthritis. She reported history of bilateral thumb pain, bilateral knee pain associated with episodes of swelling, warmth, and erythema, and left shoulder pain resulting in restricted range of motion. Further, she reported new onset of slightly painful and itchy skin lesions on her arms bilaterally for one year associated with onset of chemotherapy for treatment of colorectal cancer. She denied fever, chills, fatigue, weight loss, hearing loss, dry, red or painful eyes, nasal or oral sores, epistaxis, Raynaud phenomenon, shortness of breath, chest pain, hemoptysis, dysphagia, hematemesis, hematochezia, and hematuria. Physical examination was significant for red non-blanchable lesions on bilateral legs and feet without ulceration, bilateral tenderness of carpometacarpal joints, and left shoulder tenderness with restriction of range of motion. Patient had a skin biopsy done which had revealed leukocytoclastic vasculitis. Differential diagnoses included rheumatoid vasculitis, IgA vasculitis, Cryoglobulinemia, and ANCA related vasculitis. The patient was empirically started on Prednisone 40 mg daily to be titered down. Work up revealed elevated liver enzymes, positive cryoglobulins, cryocrit more than one percent, positive ANA with nuclear speckled pattern with a titer of 1:80, and reactive HCV antibody. Based on history, physical examination and lab findings, a diagnosis of HCV induced cryoglobulinemic vasculitis was made. Conclusion: In the context of biopsy proven leukocytoclastic vasculitis, a broad differential diagnosis including systemic causes of vasculitis should be undertaken. MCS associated with HCV is a severe form of the disease with a 5-year mortality rate of 25 percent. Therefore, it is of utmost importance to make an accurate diagnosis and initiate the appropriate treatment to improve the quality of life, reduce complications and the mortality

Granulomatous Mastitis: A rare or an underdiagnosed disease?

Introduction:Granulomatous mastitis (GM) is a benign chronic inflammatory breast disease that is poorly understood, with no universal agreement on underlying etiologies and treatment protocol. The proposed etiologies include trauma, metabolic processes, hormonal changes, autoimmune disorders, and infections. This entity commonly presents with unilateral painful firm and erythematous breast mass, and less frequently with areolar retraction, fistula formation and ulceration. The disease is more common in Hispanic and Asian population. Due to its inflammatory nature, the mainstay of treatment includes a tapered course of high-dose corticosteroid, and methotrexate has proven to be effective in some cases. Case Presentation: A 47-year-old Hispanic woman presents to the office with a chief complaint of left tender red breast mass. She reports a similar episode in 2016, after a miscarriage, for which she was diagnosed with mastitis and underwent incision and drainage with subsequent resolution. She also reports one episode of breast mass associated with high fevers that resolved spontaneously. She denied fever, chills, weight loss, or breast discharge. The physical exam was remarkable for a 5 cm indurated tender left breast mass with surrounding erythema. Based on presentation, history, and physical examination the differential diagnosis included infectious mastitis, breast cancer, and granulomatous mastitis. Labs were remarkable for ESR of 51, RF 53. Mammogram demonstrated focal asymmetries of the left breast and ultrasound revealed one focus suspicious of abscess formation and another representing a resolving abscess with granulation tissue formation. A core needle biopsy of the mass was consistent with granulomatous inflammation. Patient was diagnosed with granulomatous mastitis and started on a course of antibiotic therapy with doxycycline and anti-inflammatory with celecoxib. On follow up, she reported significant improvement in her symptoms. Conclusion: The early diagnosis of GM may be challenging due to its clinical presentation and imaging findings mimicking other etiologies such as infectious or neoplastic causes. Therefore, it is important for physicians to keep a high index of clinical suspicion when evaluating a patient with such clinical presentations to make an early accurate diagnosis. The ultimate goal is to avoid unnecessary investigations and procedures which can cause emotional, physical, and financial burdens to the patient

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

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