Exacerbation of Behcet's Disease and Pyoderma Gangrenosum Following COVID-19 Infection: A Case Report.

Behcet's disease (BD) and pyoderma gangrenosum (PG) are rare autoimmune inflammatory diseases that have been reported to relapse following COVID-19 infection. BD is a multisystemic syndrome that may involve multiple body organs. PG is a skin disease that can be a part of the skin involvement of BD. We report a 33-year-old woman with BD and PG who developed headaches, arthralgias, and rapidly progressive painful skin ulcers after COVID-19. She had not complained about BD or PG symptoms for two years prior to admission. Treatment at admission comprised infliximab 560 mg every eight weeks, azathioprine 50 mg daily, and low-dose aspirin. Due to the suspicion of neuro BD and the rapid appearance and progression of the ulcers, she was treated with intravenous (IV) methylprednisolone 1000 mg daily three times followed by prednisone at 1 mg/kg/day. Azathioprine was increased to 100 mg bid. Local ulcer care was provided. She was discharged home on the eighth hospital day. The arthralgias were completely gone, and the headaches and skin ulcers had improved. Six months after discharge, she was off prednisone and continued infliximab and azathioprine. She had no headaches or joint pains, and the ulcers had completely healed. One year after admission, BD and PG signs and symptoms had completely disappeared. This case highlights the importance of recognizing that autoimmune diseases may exacerbate COVID-19. Timely management is crucial to prevent complications and morbidity. To our knowledge, this is a rare case report describing BD and PG exacerbation following COVID-19.

An Unusual Case of Kidney Injury in a Young Woman with a Connective Tissue Disease.

A 32-year-old female was admitted to our institution with thrombocytopenia, fever, serositis, hepatosplenomegaly, diffuse lymphadenopathy, and renal insufficiency. A diagnosis of systemic lupus erythematosus was made. Due to recalcitrant thrombocytopenia, serositis, and renal insufficiency methylprednisolone was prescribed in high doses. In addition to proteinuria and hematuria, she was found to have uric acid crystals in her urinalysis. A serum uric acid was found elevated at 18 mg/dL. Rasburicase infusions were started. Within 5 days of commencing rasburicase and continuing high-dose methylprednisolone, her serum creatinine normalized and proteinuria resolved. The microhematuria disappeared within 2 weeks of beginning rasburicase. The rapid reversal of renal insufficiency and all urinary abnormalities after the start of rasburicase infusions suggests that the renal injury was most likely due to uric acid-mediated renal injury and not lupus nephritis. Our case illustrates the co-occurrence of 2 distinct clinical entities, one common for the patient's age, sex, and foremost clinical findings, while the other uncommon and unexpected, but both associated to kidney injury. Clinicians must be aware that careful evaluation of symptoms and laboratory tests is needed to make a thorough differential diagnosis and provide the right treatment at the most opportune moment.

Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome.

Macrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we present a 22-year-old Hispanic woman with SLE who was hospitalized because of a three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain. Initial laboratories showed severe pancytopenia with marked elevation of liver enzymes and ferritin levels. Bone marrow biopsy revealed macrophages with engulfed erythrocytes consistent with MAS. The patient was treated with high-dose corticosteroids, intravenous immunoglobulins, and cyclosporine 3 mg/kg/day. She had a remarkable clinical response to this therapy. She was continued on cyclosporine, and prednisone dose was gradually decreased to 7.5 mg daily without experiencing recurrent disease. She remained in full clinical remission for 12 months. Our case, together with other reports, suggests that combination therapy with corticosteroids, immunoglobulins, and cyclosporine appears to be effective for patients with SLE-associated MAS. Furthermore, cyclosporine seems to be a good drug for maintenance of remission.

Severe systemic inflammatory response syndrome immediately after spinal surgery in a patient with axial gout.

We report a 55-year-old man with gouty arthritis who developed a 3-month history of low back pain, gradual lower extremities weakness and urinary incontinence. Lumbar MRI showed an exophytic lesion at L3-L4. Immediately after spinal decompression surgery, he developed fever, disorientation, polyarthritis, acute kidney injury and leucocytosis. He was treated with multiple antimicrobial agents for presumed spinal abscess but did not improve. Multiple body site cultures were negative. Aspiration of the sacroiliac joint revealed the presence of monosodium uric acid crystals. A diagnosis of acute gout was done, and he was treated with high-dose intravenous methylprednisolone and colchicine. Within 48 hours, he had a remarkable clinical improvement. At discharge, neurological and laboratory abnormalities had resolved. Awareness of risk factors for axial gout and a high degree of suspicion are important to establish a prompt diagnosis and treatment to prevent severe complications as seen in this case.

Inflammation in Osteoarthritis.

Advances in the understanding of chondrocytes and the synovial membrane as targets of and participants in the inflammatory process in articular joints have provided insights into the role of inflammation in cartilage and subchondral bone injury in rheumatic diseases. Reports that describe the inflammatory cellular infiltration of synovial membranes in patients with osteoarthritis, studies that associate cartilage structural injury with synovial membrane inflammatory status, the development of new imaging modalities that quantitatively measure synovial membrane inflammation and basic science advances that explore inflammatory pathways in the synovial cavity all suggest that inflammation plays an important role in cartilage injury in osteoarthritis. As a result there is a shift in the notion that osteoarthritis is a disease caused merely by mechanical forces that increase joint loading. In response to this change in the current paradigm, innovative treatments involving the use of medications that modify the body's immune response (i.e., biologic and disease- modifying agents) are being studied. Implications for the population of Puerto Rico are discussed herein.

Rheumatology practice profiles in teaching and community settings in Puerto Rico: implications to subspecialty training.

BACKGROUND: University-based fellowship training programs may not always provide the required experience to manage common and less complex medical conditions usually seen at the community setting. Few studies have addressed this concern. OBJECTIVE: The purpose of this study was to compare the demographic features, diagnoses, and rheumatologic procedures in patients seen at the University of Puerto Rico Medical Sciences Campus Adult Rheumatology Training Program practice and 3 community-based rheumatology practices in Puerto Rico. METHODS: Claim forms submitted to health care insurance companies in 2007 from the university and community practices were evaluated. Demographic parameters, primary diagnoses (per International Classification of Diseases, Ninth Revision), medical visits, and rheumatologic procedures (per Current Procedural Terminology 4) were examined. Variables were analyzed by χ and Student t tests. RESULTS: A total of 11,373 claim forms were reviewed: 1952 from the university setting and 9421 from the community-based practices. Younger age and female sex were more common in visits from the university-based setting. Autoimmune diseases were reported more commonly at the university setting (65.3% vs 37.6%, P < 0.001). Conversely, osteoarthritis (24.4% vs 9.8%, P < 0.001), fibromyalgia syndrome (7.4% vs 1.9, P < 0.001), and regional rheumatic pain syndromes (7.2% vs 1.4%, P < 0.001) were seen more frequently at community practices. Hospital/emergency room visits (11.8% vs 0.3%, P < 0.001) were more frequent at the university setting, whereas joint (13.3% vs 2.9%, P < 0.001) and periarticular injections (6.2% vs 1.7%, P < 0.001) were performed more frequently at the community practices. CONCLUSIONS: Several differences were found in the profile of demographic parameters, diagnoses, medical visits, and rheumatologic procedures between patients seen at the University of Puerto Rico Medical Sciences Campus rheumatology practice and 3 community-based practices. Efforts may be required to diversify and increase the exposure of rheumatology fellows to patients with conditions commonly seen at the community setting.

Pulmonary hemorrhage in a patient initially presenting with discoid lupus.

As a cutaneous variant of lupus erythematosus, discoid lupus erythematosus (DLE) is thought to have a good prognosis; however, the involvement of internal organs with a transition to systemic disease may occur. The progression from DLE to systemic lupus erythematosus has been reported in up to 28% of patients. This progression to systemic disease has been associated with a benign course. Herein, we report the case of a 31-year-old woman with a 10-year history of discoid lupus, now presenting with dyspnea and pleuritic chest pain of 1 month's duration. A significant drop in hemoglobin and hematocrit levels was observed in association with leukopenia, lymphopenia, a positive ANA, and hypocomplementemia. Chest radiography and computed tomography revealed bilateral infiltrates. An open lung biopsy confirmed the presence of intra-alveolar hemorrhage. Based on the results of the tests and analyses detailed herein, a diagnosis of pulmonary hemorrhage secondary to systemic lupus erythematosus was made. To our knowledge, pulmonary hemorrhage as the initial manifestation of the systemic involvement of discoid lupus has not been reported before.

Hidden in plain sight: macrophage activation syndrome complicating Adult Onset Still's Disease.

Hemophagocytic Lymphystiocytosis is a rare and fatal complication of rheumatic diseases, particularly Adult Onset Still's Disease (AOSD). It may be precipitated with immunosuppressive drugs and with viral and bacterial infections. A diagnosis depends on a high index of suspicion associated to certain clinical manifestations (fever, rash, Splemomegaly, any cytology blood dyscrasia, hipertrigliceridemia, hiperfibrinogenemia, and others), as well as pathologic evidence of hemophagocitosis from bone marrow biopsy or tissue samples of affected organs. Therapy consists of high dose corticosteroids and immunosuppressive drugs. We present a 42 year old woman with AOSD in remission who developed HLH in spite of receiving therapy with high dose steroids and immunosuppressive drugs. She had 2 negative bone marrow aspirates. Evidence of Hemophagocytosis was detected in both bone marrow biopsies. Timely evaluation and recognition of the signs and symptoms of HLH is crucial for the prompt management and a decrease in the mortality associated with this disease.

Reactive thrombocytosis: an early manifestation of Takayasu arteritis.

A woman with Takayasu arteritis is reported who presented with constitutional symptoms and persistent thrombocytosis documented since 3 years before the diagnosis. Disease-specific symptoms such as arm claudication, transient loss of vision, and self-remitting eye ptosis present at the time were apparently missed, because she is a non-English-speaking Hispanic woman whose history was obtained through an interpreter. Extensive workup done at the time failed to reach a definite etiology. A computed tomography scan of the chest done because of midthoracic back pain and an elevated erythrocyte sedimentation rate showed circumferential wall thickening and mild surrounding edema throughout the thoracic and abdominal aorta and both carotid arteries consistent with Takayasu arteritis. Prednisone at a dosage of 1 mg/kg twice a day decreased the platelet count within 45 days of its initiation. Takayasu arteritis should be considered in the differential diagnosis of unexplained thrombocytosis, particularly in young women.

Association of sunlight exposure and photoprotection measures with clinical outcome in Systemic Lupus Erythematosus

This study was designed to explore the relationship of sunlight exposure and ultraviolet (UV) light protection measures with clinical outcome in systemic lupus erythematosus (SLE). A structured questionnaire was administered to sixty Puerto Rican SLE patients, to assess their attitudes and behavior regarding sunlight exposure and photoprotection measures. Medical records were reviewed to evaluate the clinical outcome measures that included: clinical manifestations, number of SLE-related hospitalizations, number of exacerbations and pharmacologic treatment. Almost all (98.3 percent) patients were well acquainted of sunlight effects on disease activity. Two thirds were exposed to direct sunlight for an average of less than one hour per day and 33.3 percent for one hour or more. Thirty patients (50 percent) reported use of sunscreen, with sun protective factor of 15 or greater, when exposed to sunlight. Less than 40 percent of patients regularly wore hat or long-sleeves clothes to protect from sunlight. Although there were some clinical differences between the groups with different sunlight exposure times, none reached statistical significance. Also, no significant differences were found between the groups in regards to sunlight protective clothes. However, patients that regularly used sunscreen had significantly lower renal involvement (13.3 vs 43.3 percent), thrombocytopenia (13.3 vs 40 percent), hospitalizations (26.7 vs. 76.7 percent), and requirement of cyclophosphamide treatment (6.7 vs. 30 percent) than patients that did not used it (P < 0.05). We conclude that use of sunscreen photoprotection was associated with a better clinical outcome in our SLE patients. These findings further support the importance and benefits of photoprotective measures in patients with SLE

Prognostic factors in patients with IVDA and bacteremia

The medical records of all the patients with bacteremia and recent use of illicit intravenous drugs admitted to Hospital Universitario Ramón Ruíz Aranau from January 1, 1988 to June 30, 1989 were reviewed. It consisted of 28 records, 21 of which were male patients and 7 females. The mortality rate among these patients was 46%. Staphhylococcus aureus was the most common pathogen recovered from blood cultures. All the S. aureus were methicillin sensitive. The presence of clinical sepsis, a low Karnofsky performance status at the time of admission and multiorgan abnormalities were the most important prognostic factors that determined outcome in these patients