Rare Complication Of Systemic Lupus Erythematosus Research Articles

Lupus Myocarditis with Pericardial Effusion: a Rare Presentation of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) can manifest with diverse cardiac presentations, with pericarditis being the most common. However, myocarditis, albeit rare, poses significant risks. Here, we present the case of a 20-year-old woman initially diagnosed with tuberculosis but later found to have lupus myocarditis, a rare complication of SLE. She presented with features of left ventricular failure and pericardial effusion, demonstrating the importance of considering alternative diagnoses in such cases. Prompt management with supportive care and oral steroids led to eventual improvement.

P028 Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus

Abstract Background/Aims Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease that develops following the reactivation of the John Cunningham virus (JCV) that in rare circumstances can occur in autoimmune disorders such as systemic lupus erythematosus (SLE). We present a 75-year-old female with SLE who had been treated with immunosuppressive therapy (mycophenylate and steroids) for one year. Methods A 75 year-old female presented with sub-acute deterioration of physical and cognitive function over the course of a few months including memory loss, ataxia, dysgraphia, word finding difficulty, confusion, low mood and reduced oral intake. Neurological examination revealed receptive and expressive aphasia, gaze fixation, unilateral weakness, brisk reflexes with positive plantar reflex and rigidity. A diagnosis of PML was made due to the detection of JC virus in the cerebrospinal fluid (234 copies/ml) and brain MRI findings consistent with the disease. Haematology results showed a profound lymphopenia (0.1x109 ). Mycophenolate was held and she was commenced on increasing doses of Mirtazapine up to 45mg. Clinical review at four months found marked improvement in her cognitive and physical abilities. Repeat CSF analysis found JC virus to be undetectable. Results PML has a 20-fold greater incidence in SLE when compared with the general population. Lymphopenia is a common finding in patients with SLE and has been found to correlate with both the risk and severity of opportunistic infections. The cause of this can be multifactorial and immunosuppressive drugs are often implicated however SLE in isolation can cause severe CD4+ lymphopenia leading to an increased risk of PML reactivation. This case demonstrates the importance of monitoring lymphocyte counts in SLE. Current guidance suggests adjusting regimes of immunosuppressive therapy if lymphocyte counts fall below 1x109 however further guidance on its management may be warranted. The heterogenous manifestation of SLE can make differentiating between the neurological syndromes associated with rheumatic disease and PML challenging. Given the management of PML is vastly different to that of SLE in regards to introduction or withdrawal of immunosuppressive therapies, there should be a high index of clinical suspicion in the early stages of investigation. In vitro studies have explored the possibility of mirtazapine (a 5-HT2A antagonist) providing a low-risk treatment option for those with SLE and this warrants further research into its efficacy in treating or preventing PML in this cohort. Our patient had improved symptomology after cessation of mycophenolate and the commencement of Mirtazapine. Conclusion PML is a rare complication of SLE but should be considered in patients admitted with neuropsychiatric presentations, the use of Mirtazapine may provide a low-risk treatment strategy. Disclosure D. Babla: None. Y. Suleman: None. A. Whittington: None. S. Hamdulay: None.

MISPLACED BLOOD: A CASE OF DIFFUSE ALVEOLAR HEMORRHAGE

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a life threatening condition manifesting as a result of disruption of the alveolar capillary basement membrane allowing blood to enter the alveolar space. Patients with systemic lupus erythematosus (SLE) do have multi organ involvement depending on the degree of disease, and although DAH is rare, it carries a high mortality rate. CASE PRESENTATION: A 36 year old female with a past medical history of SLE, hypertension, diastolic heart failure, diabetes mellitus, lupus cerebritis, lupus nephritis, lupus pericarditis presented to the hospital due to altered mental status. Patient was initially in status epilepticus secondary to lupus cerebritis requiring intubation and sedation and was successfully weaned off sedation and extubated; however patient then developed acute hypoxemic respiratory failure that was initially thought to be secondary to fluid overload versus infectious etiology. Patient deteriorated despite broad spectrum antibiotics and diuresis. Chest radiography showed bilateral airspace disease, superimposed pulmonary edema, transthoracic echocardiogram showed an ejection fraction of 45 to 50%. During the next few days she had an acute drop in her hemoglobin (Hb) and she continued to deteriorate. Bronchoalveolar lavage performed with the aliquots becoming progressively more blood tinged with each subsequent lavage. These findings were observed in both lungs. No frank blood was noted. Patient was noted to have diffuse alveolar hemorrhage likely secondary to lupus and started on methylprednisolone and cyclophosphamide. Her respiratory status began to improve and she was extubated to non-invasive ventilation then to room air shortly after systemic steroids were initiated. DISCUSSION: Pulmonary hemorrhage is a rare complication of SLE. The etiology is largely unknown however capillaritis and bland hemorrhage has been seen on histopathology. Lack of response to diuretics and rapid response to systemic steroids supports an inflammatory etiology such as capillaritis. CONCLUSIONS: Clinicians should include DAH in the differential in SLE patients with unexplained drop in hemoglobin and radiographic changes showing bilateral alveolar infiltrate. Prompt treatment with high dose steroids plus immunosuppressant should be initiated. REFERENCE #1: Kazzaz NM; Coit P; Lewis EE; McCune WJ; Sawalha AH; Knight JS; (n.d.). Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival. Lupus science & medicine. https://pubmed.ncbi.nlm.nih.gov/26430514/. REFERENCE #2: M R Zamora 1, M L Warner, R Tuder, M I Schwarz (n.d.). Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine. https://pubmed.ncbi.nlm.nih.gov/9193454/. DISCLOSURES: No relevant relationships by Nida Ahmed, source=Web Response No relevant relationships by Philip Almalouf, source=Web Response No relevant relationships by Robert Petrossian, source=Web Response No relevant relationships by Farheen Surtie, source=Web Response

Pulmonary Hypertension in Systemic Lupus Erythematosus with Raynaud’s Phenomenon: Case Report

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs such as skin, joints, kidneys, heart, lungs, nervous system and blood. Pulmonary hypertension is a rare complication of SLE.1 Systemic lupus erythematosus associated with pulmonary hypertension was defined as an increase in the average pressure in the pulmonary artery at rest ? 25 mmHg with pulmonary capillary wedge pressure ?15 mm Hg and an increase in pulmonary vascular resistance.2 The prevalence of pulmonary hypertension in SLE approximately 0.5 to 17.5%. Predictors factors of the occurrence of pulmonary hypertension in LES is Raynaud's phenomenon, anti-U1RNP antibody, and antibody positive anticardiolipin.3
 A woman aged 37 years came with a chief complaint of pain in the fingers and toes with black-colored wounds felt since 6 months before admission. From the anamnesis, physical examination, support to meet 5 of the classification criteria for systemic lupus erythematosus based on the 1997 ACR criteria which includes manifestations of arthritis, mucocutaneous, serositis, lupus antiokoagulan, and ANA IF positive. In these patients also found the typical signs and symptoms of Raynaud's phenomenon which leads to the symptoms of pain in the fingers of both hands when exposed to cold and pale to red when heated and has been confirmed from the results of arteriography. From the results of echocardiography reveal any pulmonary hypertension. This patient was treated with steroids, immunosuppressants and antiplatelet. The case was removed because of pulmonary hypertension is a complication LES rare and necessary sharpness in diagnosis. Patients with pulmonary hypertension of unknown or untreated can become a progressive right heart failure and lead to death.3

Pulmonary arterial hypertension associated with systemic lupus erythematosus

Pulmonary arterial hypertension (PAH) associated with connective tissue diseases is the most common cause of pulmonary hypertension. Systemic lupus Erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. The prevalence of PAH in SLE is less than 4% when the PAH diagnosis is based on the gold standard, namely right heart catheterization. The aim of this study was to describe the clinical and biological presentation of PAH in SLE. The study population consisted of 238 Tunisian patients with SLE who fulfilled the revised criteria of the American Rheumatism Association and were admitted to the internal medicine and rheumatology department in Fattouma Bourguiba University Hospital between 2010 and 2020. All patients underwent Doppler echocardiography study. PAH was hemodynamically defined by increased systolic pulmonary artery pressure (SPAP) at rest ≥ 25 mmHg. Of the 238 patients, 12 women presented PAH (5%). Mean age were 38, 2 years. SPAP ranged from 25 to 36 mmHg. Two patients had associated systemic scleroses. Comparison of patients with and without PAH found out that patients with PAH had significantly higher frequency of Reynaud's phenomenon (41,7% VS 13,4%; P = 0.023) and serositis (50% VS 21%; P = 0.046). Immunological variables associated with PAH include anti-SSA ( P = 0.003), anti-SSB ( P = 0.023) and anticardiolipine antibodies IgM/IgG ( P = 0.029). No significant differences between the two groups regarding cardiac involvement, thrombosis, anti-DNA and anti-SM antibodies were found. On follow up, the PAH was associated with an increased risk of mortality (OR = 7, 23; 95% CI = 1.294–40.447). PAH is a rare complication of SLE. Other studies are needed to confirm whether the presence of PAH is a risk factor for mortality in SLE patients.

Clinical characteristics of systemic lupus erythematosus with chylothorax and/or chylous ascites: An analysis of 15 cases in China.

This analysis of clinical data from systemic lupus erythematosus (SLE) patients with chylothorax and/or chylous ascites was conducted to guide further clinical work.From June 2008 to June 2019, 15 SLE patients (14 females and 1 male) with chylothorax and/or chylous ascites were hospitalized at the Beijing Shijitan Hospital. Sixty SLE patients without chylothorax and chylous ascites were randomly selected as controls. Patients’, clinical data was investigated.The mean age of onset of chylothorax and/or chylous ascites in patients with SLE was 35.7 ± 3.7 years (range, 15–69 years). The mean disease duration of chylothorax and/or chylous ascites in patients with SLE was 13.7 ± 3.4 months (range, 1–48 months). Patients with chylothorax and/or chylous ascites were always diagnosed at later stages of SLE compared with the controls. Among cases, glomerulonephritis and hematologic system involvement were the most common complications. Anti-Sjogren's syndrome antigen A antibody was positive in 7 cases (46.7%). Among cases, direct lymphangiography was performed in 13 patients, indicating thoracic duct outlet obstruction or a poor backflow at the terminal of the thoracic duct. Subsequently, 13 patients were treated with corticosteroids, combined with immunosuppressants in 11 patients and thoracic duct surgery in 6 patients. Eleven patients were followed up for 0.5 to 7.0 years. One patient died of infection. Eight patients (53.3%) achieved remission.Chylothorax and/or chylous ascites are rare complications of SLE. An early diagnosis and timely initiation of glucocorticoids, immunosuppressants, and surgery are critical to relieve symptoms and to improve prognosis.

Shrinking lung syndrome - a diagnostic dilemma: a case report

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory connective tissue disorder with multi-organ involvement. A rare complication of SLE is shrinking lung syndrome (SLS), characterized by progressive exertional dyspnoea, reduced lung volumes, and diaphragmatic elevation. Case Presentation: We report a case of SLS secondary to SLE, who required an extensive work up to exclude other more common causes of lung involvement in such a condition. This case report highlights the disease burden of SLS in a once active, independent young lady requiring nocturnal non-invasive ventilation (NIV) to relieve shortness of breath in a recumbent position. This patient had the triad of hypoxia at rest, type 2 respiratory failure, and diaphragmatic palsy. Several different immunomodulators were used initially with little success and it was not until she received six cycles of intravenous cyclophosphamide, that she no longer required NIV support, displayed improvements in pulmonary function tests and diaphragmatic motility and attained a normal lifestyle. Conclusion: The use of immunomodulators appeared to be particularly effective in restoring normal functional capacity in shrinking lung syndrome. However, the pathophysiology of this condition requires further studies.

Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study

BackgroundNo population-based study has investigated the cumulative incidence of pulmonary arterial hypertension (PAH) in patients with newly diagnosed systemic lupus erythematosus (SLE) or the survival impact of PAH in this population.MethodWe used a nationwide database in Taiwan and enrolled incident SLE patients between January 1, 2000, and December 31, 2013. The cumulative incidence of PAH in the SLE patients and the survival of these patients were estimated by the Kaplan-Meier method. Potential predictors of the development of PAH were determined using a Cox proportional hazards regression model.ResultsOf 15,783 SLE patients, 336 (2.13%) developed PAH. The average interval from SLE diagnosis to PAH diagnosis was 3.66 years (standard deviation [SD] 3.36, range 0.1 to 13.0 years). Seventy percent of the patients developed PAH within 5 years after SLE onset. The 3- and 5-year cumulative incidence of PAH were 1.2% and 1.8%, respectively. Systemic hypertension was an independent predictor of PAH occurrence among the SLE patients (adjusted hazard ratio 2.27, 95% confidence interval 1.59–2.97). The 1-, 3-, and 5-year survival rates of SLE patients following the diagnosis of PAH were 87.7%, 76.8%, and 70.1%, respectively.ConclusionsPAH is a rare complication of SLE and the majority of PAH cases occur within the first 5 years following SLE diagnosis. Systemic hypertension may be a risk factor for PAH development in the SLE population. The overall 5-year survival rate after PAH diagnosis was 70.1%.

Pulmonary Arterial Hypertension In A Female Patient With Systemic Lupus Erythematosus

Abstract Introduction. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown origin, characterized by multisystemic involvement and a potentially severe evolution. Pulmonary arterial hypertension (PAH) is a rare complication of SLE, with low 5-year survival. Case presentation. We are presenting the case of a female patient, aged 56 years old, diagnosed in 1992 with SLE with cutaneous manifestations (butterfly-shaped erythematous rash), joint manifestations (polyarthritis), serositis manifestations (massive pleuropericarditis), and immunological manifestations (positive anti-dsDNA antibodies, decreased C3), ignored therapeutically for a long time. In 2010 she complained of dyspnea on medium exertion and leg edemas, with marked increase of PAPs by echocardiography. She was diagnosed with severe PAH (confirmed by right heart catheterization) and in the “Marius Nasta” National Institute of Pneumology she started a treatment with an endothelin receptor antagonist (Bosentan) in combination with a prostacyclin receptor agonist (Selexipag). Since 2013 the patient is on oral anticoagulant treatment for permanent atrial fibrillation. In 2015 she was referred back to out clinic as she complained of recurrent episodes of massive ascites with evacuatory paracenteses in amounts of about 6-9L per paracentesis. After excluding other causes, ascites was considered to be secondary to the SLE, and a treatment was initiated with Hydroxychloroquine (HCQ) and pulse therapy with Methylprednisolone, on which the remission of the ascites was achieved during the following months. Currently, the SLE is well controlled without recurrence of ascites on treatment with HCQ and gradual decrease until stopping of cortisone doses, and the PAH is stable. Conclusion. PAH is a rare complication of the SLE, with a complex pathophysiological immune mechanism, for which - together with the specific vasodilator treatment - the increase of immune suppression is recommended.

Common Complaints Masking a Rare Disease: Abdominal Pain and Diarrhea as the Presenting Signs of Lupus Enteritis

Systemic Lupus Erythematosus (SLE) is an autoimmune condition characterized by joint pains, rash, and other systemic manifestations. Herein we describe a patient who presented with GI complaints and was later discovered to have lupus enteritis (LE), a rare complication of SLE. A 28-year-old Asian woman with family history of Crohn's disease presented for abdominal pain, watery diarrhea, and bilious emesis. Initial labs were unremarkable with a negative pregnancy test. CT scan revealed mild ascites, mild diffuse enteritis, and sigmoid colitis. Stool cultures, O & P and C. diff toxin were all negative. She was treated with ciprofloxacin and metronidazole with improvement of symptoms and then discharged. She returned for recurrence of symptoms and repeat CT showed mild ascites, rapidly progressing enteritis with severe involvement of the ileum and descending colon. Again, labs were grossly normal including CMP, CBC, ESR, CRP, C. diff., and stool cultures. Colonoscopy was consistent with acute enterocolitis without mucosal changes or evidence of Ulcerative colitits or Crohn's disease. Terminal ileum biopsies were unremarkable. At this point, a rheumatological workup was initiated and she was started on steroids for presumed autoimmune etiology. Her symptoms subsided and ANA returned at 1:320 speckled pattern, complement C3 and C4 were low. She was diagnosed with LE and discharged on oral prednisone. LE is a rare manifestation of SLE that is not fully understood. Given its overlapping symptoms with common GI illnesses, the diagnosis is challenging and easily missed. Common symptoms of LE include abdominal pain (97%), vomiting, diarrhea, and fever. Laboratory features include low complement levels (88%), anemia, leukopenia, lymphopenia, and thrombocytopenia. Median CRP level is 2.0 mg/dL. Imaging findings include bowel wall edema with target sign, ascites, hydronephrosis, mesenteric abnormalities and bowel dilation. If left untreated, LE can progress to intestinal necrosis and perforation. In this case, the family history of Crohn's disease initially led us to an inflammatory cause. Only after common etiologies were ruled out, was an autoimmune etiology explored. LE can be considered in patients with prior diagnosis of an autoimmune disorder who present with recurrent and intractable abdominal pain, diarrhea and emesis. LE has an excellent response to steroids. If promptly treated, symptoms and disease processes are reversible.1560 Figure 1. Abdominal CT images of bowel-wall thickening and enhancement known as the “target sign” (blue/red arrows). The abnormalities are non-specific and can also be seen in patients with Inflammatory Bowel Disease and other gastrointestinal conditions.

UnMASking the Diagnosis: Idiopathic Non-Cirrhotic Portal Hypertension Diagnosed in the Setting of Macrophage Activation Syndrome Complicated by Severe Pancreatitis: 2017 Presidential Poster Award

Systemic Lupus Erythematosus (SLE) is a common rheumatologic condition with known GI involvement, most notably autoimmune hepatitis and lupus colitis. Acute pancreatitis (AP) is a rare complication of SLE and is typically associated with disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multiorgan dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare ultimately complicated by MAS in an adult patient and successfully treated with Anakinra. A 29 year-old female with SLE and non-adherence to medical therapy was admitted with severe necrotizing AP evidenced by epigastric pain, nausea, oral intolerance, lipase >3000 U/L, and CT scan demonstrating necrosis and inflammation in the pancreatic head. After admission, she developed tachycardia, hypothermia, lactate elevation of 17.2, and hypercarbic respiratory failure with pulmonary edema requiring intubation and mechanical ventilation with aggressive supportive care. Laboratory evaluation demonstrated evidence of DIC with pancytopenia, elevated ANA/ds DNA, and a ferritin of 1332 ng/mL. She was started on methylprednisolone for treatment of a SLE flare with clinical improvement leading to extubation. Following extubation, her ferritin level doubled in association with clinical deterioration raising suspicion for MAS. She was then started on Anakinra resulting in brisk clinical response and down trending ferritin. AP occurs in 1-8% of SLE patients and is proposed to be the result of vascular damage. MAS complicates approximately 5.5% of childhood SLE compared to 0.7% of adult SLE but is more commonly the result of AP in combination with SLE compared to SLE alone and is associated with higher mortality rates. Our case uniquely illustrates a rare etiology of AP and the relationship between systemic diseases and the GI system in a critically ill patient. Treatment of MAS is aimed at treating the underlying SLE; however, our patient required the addition of a chemotherapeutic agent. Anakinra is a novel agent which has been used to treat refractory childhood MAS and was successful in treating this adult patient. To the best of our knowledge, this is the only reported case of refractory MAS due to SLE induced AP necessitating treatment with Anakinra.

Systemic Lupus Erythematosus Complicated by Shrinking Lung Syndrome Treated With Belimumab: A Case Report

SESSION TITLE: Inflammatory Diseases Affecting the Lung SESSION TYPE: Student/Resident Case Report Slide PRESENTED ON: Monday, October 24, 2016 at 11:00 AM - 12:00 PM INTRODUCTION: Cardiopulmonary symptoms occur in up to 60% of patients with Systemic Lupus Erythematosus (SLE). Shrinking Lung Syndrome (SLS) is a rare complication of SLE where patients present with restrictive lung disease without imaging findings. We report a case of new onset SLS while receiving hydroxychloroquine, mycophenolate and prednisone treated successfully with belimumab. CASE PRESENTATION: A 44-year old woman with a history of SLE, Lupus Nephritis Class IV-V, presented to the Rheumatology Clinic with dyspnea on exertion. She had recently completed 6 cycles of cyclophosphamide induction therapy for lupus nephritis and was receiving hydroxychloroquine, mycophenolate, and prednisone. Physical examination was unremarkable. SLE Disease Activity Index score was unchanged from the previous visit. Imaging of the chest was perfomed to rule out life threatening conditions. Computerized Tomography Angiography failed to reveal parenchymal pulmonary disease or pulmonary artery filling defects. However, pulmonary function testing (PFT) showed a restrictive pattern with impaired diffusion capacity. To evaluate for diaphragmatic paralysis, seated and supine PFTs were performed which did not show Forced Vital Capacity (FVC) changes in supine position. ECG, echocardiogram and nuclear imaging failed to reveal cardiac causes of dyspnea. Afterwards, the differential diagnosis was narrowed to: SLS, myasthenia gravis, and steroid-induced myopathy. CPK level was slightly increased and anti-acetylcholine receptor antibody was negative. Since the patient had no weakness, myopathy was thought to be unlikely. Belimumab was chosen as a steroid sparing agent. The patient reported resolution of dyspnea after initiating therapy. Her exercise tolerance returned to baseline and repeat PFT showed a persistent restrictive pattern with increased TLC. DISCUSSION: Several hypotheses have tried to explain the findings of SLS but the primary mechanism remains unclear. Our patient developed SLS while undergoing therapy with hydroxychloroquine, mycophenolate and prednisone. Belimumab was used as a steroid sparing therapy. Belimumab, a B Cell Activating Factor, is a new drug in the armamentarium against SLE. The optimal treatment for SLS is not yet determined. Treatment options range from prednisone as first line agent to rituximab as used in some case reports. After treatment with belimumab our patient achieved symptom resolution, increased exercise tolerance, improved VC and TLC. While the mechanism of SLS is poorly understood, B cells likely play a significant role as suggested by positive outcomes with rituximab and belimumab. CONCLUSIONS: Belimumab represents a new weapon against SLE and showed improvement of symptoms and lung volumes after treatment in a patient with SLS. Reference #1: Langenskiöld, E., et al. (2012). Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide. Respiration; International Review of Thoracic Diseases, 84(2), 144-9. http://doi.org/10.1159/000334947 DISCLOSURE: The following authors have nothing to disclose: Felix Reyes, Deana Lazaro No Product/Research Disclosure Information

Symptomatic knee osteonecrosis in patients with systemic lupus erythematosus: a case-control study.

To explore the associated risk factors of symptomatic knee osteonecrosis (KON) in patients with systemic lupus erythematosus (SLE), we conducted a retrospective case-control study to compare the clinical and laboratory features between SLE patients with and without symptomatic KON matched by age and gender. Univariate and multivariate regression analyses were used to evaluate possible associated risk factors. Twenty (one male, nineteen females) out of 3941 lupus patients were identified as symptomatic KON, which was confirmed by magnetic resonance imaging. The mean age at KON onset was 34.4 (range 12-67)years, and the median course of lupus at KON onset was 72.5 (range 8-123)months. Univariate and multivariate analyses identified that the prevalence of cutaneous vasculitis (OR 5.23; 95% CI 1.11-24.70), hyperfibrinogenemia (OR 4.75; 95% CI 1.08-20.85), and elevated IgG levels (OR 6.05; 95% CI 1.58-23.16) were statistically higher in KON group, and hydroxychloroquine (HCQ) usage was statistically lower in KON group (OR 0.27; 95% CI 0.07-0.97). Glucocorticoid usage, in terms of maximal dose, duration of treatment, and the percentage of receiving methylprednisolone pulse therapy, did not show statistical difference between the two groups (p>0.05). Symptomatic KON is a relatively rare complication of SLE. Cutaneous vasculitis, hyperfibrinogenemia, and elevated IgG levels are possible risk factors, whereas HCQ may provide a protective effect. Our results suggest that lupus activity as well as hypercoagulation status may play a role in the pathogenesis of KON in lupus.

Successful Use of Bronchoscopic Recombinant Activated Factor VII in the Treatment of Systemic Lupus Erythematosus-Related Diffuse Alveolar Hemorrhage

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a life-threatening complications characterized by damage to the microcirculatory system of alveoli leading to accumulations of intraalveolar red blood cells. DAH is a rare (2-5.4%) but dangerous (50-80% mortality) complication in systemic lupus erythematosus (SLE). We present a case of a patient with a prolonged history of SLE who presented with DAH refractory to standard therapy who was successfully treated with recombinant activated Factor VIIa (rFVIIa) administered locally through bronchoscopy. CASE PRESENTATION: The patient is a 28 year old African American woman with a past medical history of SLE complicated by biopsy proven lupus nephritis who presented to the emergency room with acute onset of right-sided chest pain and shortness of breath. She developed hemoptysis and hypoxic respiratory failure requiring intubation. Bronchoscopy performed was consistent with diffuse alveolar hemorrhage through serial aliquots on bronchoalveolar lavage (BAL). She initially treated with pulse dose intravenous methylprednisolone and received 5 treatments of therapeutic plasma exchange. She was extubated on hospital day 6, but had severe hemoptysis prompting same day reintubation, with bronchoscopy demonstrating persistent diffuse alveolar hemorrhage persistent on serial BAL aliquots on hospital day 6/7. On hospital day 7, family was consented for an attempt to control the hemorrhage with local instillation or rFVIIa (75μg/kg) divided into six aliquots and delivered at each secondary bronchi. After repeat dosing with local rFVIIa targeted at bronchi with persistent infiltrates on CT scan, repeat bronchoscopy revealed resolution of DAH and patient was successfully extubated. Coagulation studies during dosing of local rFVIIa remained unchanged. DISCUSSION: DAH is a rare complication of SLE and carries a high mortality rate (50-80%) if left untreated. It is often refractory to the conventional therapy of high dose steroids, cyclophosphamide and 3-6 cycles of plasma exchange as in our patient. Off-label use of rFVIIa by local instillation or aerosolization into lungs has been utilized in treatment of DAH with some reported success and a excellent safety profile as it does not cross the alveolar-blood barrier. CONCLUSIONS: We describe a case of successful treatment of SLE-associated DAH refractory to conventional therapy by means of locally administered rFVIIa. Reference #1: Esper RC, et al. Treatment of diffuse alveolar hemorrhage secondary to lupus erythematousus with recombinant activated factor VII administered with a jet nebulizer. Journal of Intensive Care 2014,2:47. Reference #2: Heslet L, et al. Successful pulmonary administration of activated recombinant factor VII in diffuse alveolar hemorrhage. Crit Care 2006;10(6):R177. Reference #3: Estella A, Jareno A, Fontaina LP. Intrapulmonary administration of recombinant activated factor VII in diffuse alveolar hemorrhage: a report of two case stories. Cases Journal 2008,1:150. DISCLOSURE: The following authors have nothing to disclose: Joseph Zeman, Brett Sadowski, John Sherner No Product/Research Disclosure Information

Concurrent Occurrence of Hemophagocytic Syndrome and Myelofibrosisin a Case of SLE

We experienced a case of Systemic Lupus Erythematosus (SLE) with myelofibrosis and hemophagocytosis. Recently, it has become clear that hemophagocytosis can occur with autoimmune diseases, including SLE. By contrast, myelofibrosis is a rare complication of SLE, and only ten cases have been reported. The mechanism by which myelofibrosis complicates SLE remains unknown. There are several theories: myelofibrosis may result from microvasculitis in the marrow or the unusual release of cytokines. Myelofibrosis accompanying SLE appears to be reversible with immunosuppressive therapy, although primary myelofibrosis is not. This suggests that the pathogenesis of myelofibrosis with SLE is quite different from that of primary myelofibrosis. This is the first report of SLE accompanied by myelofibrosis and hemophagocytosis. In our cases, serum TGF-β was elevated remarkably. Our case indicated that in the process of hemophagocytosis, activated macrophages produce monokines, such as TGF-β, which sequentially induce myelofibrosis. Hemophagocytosis may predispose SLE patients to myelofibrosis.

AB0709 Pulmonary arterial hypertension in systemic lupus erythematosus: a study of 80 tunisian patients

BackgroundPulmonary hypertension (PH) is a serious form of pulmonary complication that occurs in Systemic lupus erythematosus (SLE). It is less frequent in SLE compared to systemic sclerosis. The symptoms of...

Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12-year-old girl

While pleuropulmonary involvement in systemic lupus erythematosus (SLE) is a common occurrence, shrinking lung syndrome (SLS) is a rare complication of SLE, particularly in children. We report on a teenager girl with a primary SLE diagnosis, which was based upon clinical, imaging, lung-function and histological findings ascertained to be compatible with SLS. Following a pneumonia, the patient developed inflammatory residues in the lower lobes, an event that probably caused diaphragmatic immobility and subsequently led to SLS. Treatment response to steroids, cyclophosphamide and hydroxychloroquine in this case was excellent, and efficacy was more profound than previously has been reported in the literature with respect to pediatric patients. This case report argues that prognosis of SLS in SLE is likely to be favorable when the diagnosis is made early and the disease is treated appropriately.

Allogeneic transplantation of umbilical cord-derived mesenchymal stem cells for diffuse alveolar hemorrhage in systemic lupus erythematosus

Umbilical cord-derived mesenchymal stem cell transplantation (UC-MSCT) has been proved to be effective in the treatment of systemic lupus erythematosus (SLE), based on animal experiments and clinical trials. Diffuse alveolar hemorrhage (DAH) is a rare complication of SLE with a high mortality usually over 50%. This study aimed to assess the efficacy of UC-MSCT in the treatment of SLE-associated DAH. Four SLE patients complicated with DAH, who underwent UC-MSCT, were included. Clinical changes before and after transplantation were assessed by measurements of hemoglobin, platelet level, oxygen saturation, and serological factors. High-resolution CT (HRCT) scans of the chest were performed to evaluate pulmonary manifestation. All the four patients showed dramatic improvements of their clinical manifestations. Hemoglobin was elevated after UC-MSCT and was sustained at a normal level 6 months after UC-MSCT in the four patients. Platelet level was upregulated in two patients who had thrombocytopenia at baseline. Oxygen saturation appeared to be normal at 1 month after UC-MSCT, and this result was confirmed by the HRCT scan of the chest. Serum albumin elevated to 3.5 g/dl 6 months after transplantation. Our findings suggest that UC-MSCT results in amelioration of oxygen saturation as well as hematological and serologic changes, which revealed that UC-MSCT could be applied as a salvage strategy for DAH patients.

Aortic Dissection Complicated with Hemothorax in an Adolescent Patient with Systemic Lupus Erythematosus: Case Report and Review of Literature

To report a young patient with systemic lupus erythematosus (SLE) complicated by aortic dissection. The relevant literature on the association of SLE and aortic dissection is reviewed. We describe an adolescent patient with childhood-onset SLE diagnosed aortic dissection with presentation of hemothorax. The literature review was performed by a PubMed search using the keywords systemic lupus erythematosus (SLE), aortic dissection, hemothorax, and carotid intima-media thickness (CIMT). A 17-year-old male was admitted to the hospital for severe abdominal pain. His past medical history included childhood-onset SLE complicated with lupus nephritis. Acute aortic dissection complicated with hemothorax was diagnosed and he died despite medical therapy. The accelerated CIMT progression of our patient, 0.14 mm in 20 months, might suggest ongoing premature atherosclerotic changes in the aortic wall. On reviewing the English literature, 21 cases of aortic dissection in SLE have been analyzed and discussed. Patients younger than 40 years of age, hypertension, and long-term steroid use are common features of aortic dissection in SLE patients. The possible pathogenesis of aortic dissection in SLE includes atherosclerosis, degeneration, and vasculitis. The widely accepted CIMT measured by ultrasound could be a potential diagnostic tool to access the risk of cardiovascular events. Aortic dissection is a rare complication of SLE, but could take place in an adolescent patient with childhood-onset disease. It is important to include aortic dissection as a differential diagnosis in SLE patients with unexplained sharp abdominal, chest, or back pain.

Generalized laxity of connective tissue as a possible syndrome in systemic lupus erythematosus

We report a case of systemic lupus erythematosus (SLE) with Jaccoud's arthropathy, presenting atlantoaxial subluxation and multiple joint hypermobility. Radiological studies showed degenerative changes, but no destructive changes like rheumatoid arthritis (RA) were observed. Since these are rare complications of SLE, the coexistence of these complications suggests that they have a common pathogenesis. Generalized laxity of connective tissues may be a novel syndrome in SLE.