Case Reports in Rheumatology The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. Sarcoidosis: Is It a Possible Trigger of Inclusion Body Myositis? Mon, 24 Apr 2017 00:00:00 +0000 Sarcoidosis is a multisystem disorder of unknown etiology, characterized pathologically by the presence of nonnecrotizing granulomatous inflammation in affected organs. Although skeletal muscle is involved in 50–80 percent of individuals with sarcoidosis, symptomatic myopathy has been shown to be a rare manifestation of the disease. Inclusion body myositis (IBM) is a rare acquired idiopathic inflammatory myopathy with the insidious onset of asymmetric and distal muscle weakness that characteristically involves the quadriceps, tibialis anterior, and forearm flexors. Moreover, dysphagia can be the presenting complaint in one-third of patients. Herein, we are presenting a case of 67-year-old African American female who presented with one-month history of new onset progressive dyspnea on exertion. She was diagnosed with stage IV sarcoidosis based on chest CT scan findings and transbronchial lung biopsy revealing nonnecrotizing granulomatous inflammation. Over the next three months after her diagnosis, she presented to the hospital with progressive dysphagia associated with asymmetrical distal muscle weakness. A quadriceps muscle biopsy revealed features consistent with inclusion body myositis. We are reporting this case as it may support the hypothesis of sarcoidosis being a trigger that possibly promotes the development of inclusion body myositis, leading to a very poor prognosis. Ali Zakaria, Issam Turk, Kenneth Leung, Ana Capatina-Rata, and Waseem Farra Copyright © 2017 Ali Zakaria et al. All rights reserved. SLE and Non-Hodgkin’s Lymphoma: A Case Series and Review of the Literature Mon, 27 Mar 2017 09:47:50 +0000 Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder punctuated by varied multiorgan complications all along the course of its natural history. Lymphoma represents a relatively well-recognized malignant phenomenon associated with lupus. The cause and effect relationships of lymphoma in SLE have been subject to extensive scrutiny with several studies reporting on clinic-pathologic characteristics and risk factors predicting lymphoma development in SLE. However, the pathogenic role of immunosuppressives in SLE-related lymphoma still remains unclear, and indices to help guide diagnosis, prognostication, therapy, and posttreatment monitoring are yet to be established. In this review, we describe 3 SLE patients who developed non-Hodgkin’s lymphoma at different time points of their disease. Through a careful dissection of the aforementioned cases, we intend to apprise readers of the currently available literature surrounding risk factors, management, and prognosis in SLE-related lymphoma. We will also review and discuss the implications of immunosuppressives in SLE-related lymphoma and the role of mycophenolate mofetil in SLE-related primary CNS lymphoma development. Prajwal Boddu, Abdul S. Mohammed, Chandrahasa Annem, and Winston Sequeira Copyright © 2017 Prajwal Boddu et al. All rights reserved. The Child as a Surrogate for Diagnosis of Lupus in the Mother Mon, 13 Mar 2017 06:44:47 +0000 Introduction. Neonatal lupus erythematosus (NLE) is an acquired disease of the newborn caused by transplacental transfer of maternal anti-Ro/SSA, anti-La/SSB, and infrequently anti-U1 RNP antibodies. Methodology. This is a case report of a male infant delivered via Caesarean section at 36-week gestation following detection of fetal bradycardia during routine antenatal clinic visit. Results. The mother was seropositive for antinuclear antibody (ANA) and anti-Ro/SSA and had elevated erythrocyte sedimentation rate. The baby was positive for ANA, extractable nuclear antigen (ENA), and anti-Ro/SSA. Pediatric echocardiography was abnormal and electrocardiography confirmed complete heart block. Olufemi O. Adelowo, Kenneth A. Ohagwu, Ejiehi E. Aigbokhan, and Richard O. Akintayo Copyright © 2017 Olufemi O. Adelowo et al. All rights reserved. An Unusual Presentation of Limited Granulomatosis with Polyangiitis Involving Vagina and Urethra Mon, 13 Mar 2017 00:00:00 +0000 Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina. The patient showed a good response after Corticoids and Methotrexate. Sandra Soro Marín, Enrique Júdez Navarro, Manuela Sianes Fernández, Ginés Sánchez Nievas, and Juan Gabriel Lorenzo Romero Copyright © 2017 Sandra Soro Marín et al. All rights reserved. A 64-Year-Old Woman with Chest Pain, Limb Weakness, and Endometrial Cancer Wed, 08 Mar 2017 00:00:00 +0000 Necrotizing autoimmune myopathy (NAM) is a rare subgroup of idiopathic inflammatory myopathies (IIM). This pathology usually affects proximal limb muscles and in some cases the myocardium. Patients usually display proximal limb weakness. Muscular biopsy is required to confirm the diagnosis. We report the case of a 64-year-old woman with an atypical first presentation of NAM, manifested by chest pain in the context of metastatic endometrial cancer. The diagnosis of NAM was however made when she returned a second time with proximal limb weakness. A treatment with prednisone was then initiated, to which rituximab was rapidly associated, beside a specific chemotherapy. Simon Ponthus, Omar Kherad, Nicole Petriccioli, Johannes Alexander Lobrinus, and Pierre-André A. Guerne Copyright © 2017 Simon Ponthus et al. All rights reserved. A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature Tue, 28 Feb 2017 09:12:09 +0000 This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases. Richard A. Lau, Ramandeep Bains, Duminda Suraweera, Jane Ma, Emil R. Heinze, Andrew L. Wong, and Philip J. Clements Copyright © 2017 Richard A. Lau et al. All rights reserved. Progressive Pseudorheumatoid Dysplasia or JIA? Tue, 21 Feb 2017 00:00:00 +0000 Progressive pseudorheumatoid dysplasia (PPD) or spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA) is a rare arthropathy of childhood involving the axial skeleton as well as small peripheral joints. A 10-year-old boy was referred by a general practitioner with pain and deformity in the fingers of hands and limping gait. There was no joint synovitis although the finger joints were bulky on examination with mild flexion deformity. Patient had exaggerated kyphosis and lumbar lordosis with pigeon chest and restricted hip joint movements. Anteroposterior X-rays of the hip joints revealed widened and flattened epiphyses of the femoral heads with narrow and irregular joint spaces. Hand X-rays revealed periarticular osteopenia, significant narrowing of the joint spaces of proximal interphalangeal, and distal interphalangeal joints, together with osseous enlargement of the basis of metacarpal bones and phalanges. Spinal X-rays revealed generalized platyspondyly and anterior beaking of vertebral bodies. There was a clear mega os trigonum in his feet images. All blood investigations were normal with no evidence of inflammation and thyroid hormone levels were normal. The diagnosis of PPD was favored by imaging studies and normal inflammatory markers and the patient was treated with physiotherapy, family counseling, and anti-inflammatory medications. Geetha Wickrematilake Copyright © 2017 Geetha Wickrematilake. All rights reserved. Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis Sun, 29 Jan 2017 00:00:00 +0000 Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases. We also hypothesize that the thrombogenic potential of eosinophils may play a central role in thrombogenesis in EGPA and aggressive immunosuppressive therapy remains the cornerstone of treatment, and the addition of anticoagulation therapy in the setting of thrombus formation and also very high risk of bleeding needs to be considered cautiously. Dan Zhu, Yiming Luo, Xiangyuan Liu, and Lingyun Zu Copyright © 2017 Dan Zhu et al. All rights reserved. Hydralazine Induced Lupus Syndrome Presenting with Recurrent Pericardial Effusion and a Negative Antinuclear Antibody Tue, 17 Jan 2017 07:58:56 +0000 Drug induced lupus erythematosus (DIL or DILE) is an autoimmune disorder caused by chronic use of certain drugs. We report a unique case of hydralazine induced lupus syndrome (HILS) with a negative antinuclear antibody in a female patient who was on hydralazine for a period of 1.5–2 years and developed recurrent pericardial effusion as a result of it. Initially her condition was managed with a pericardial window. The recurrence of a massive pericardial effusion necessitated a right hemipericardiectomy. After hydralazine was stopped, she never had any further episodes of pericardial effusion or tamponade. Praneet Iyer, Ahmed Dirweesh, and Ritika Zijoo Copyright © 2017 Praneet Iyer et al. All rights reserved. Primary Idiopathic Osteoarthropathy: Could It Be Related to Alcoholism? Mon, 02 Jan 2017 14:16:18 +0000 Background. Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by abnormal proliferation of the skin and bony structures at the distal extremities resulting in digital clubbing, periosteal bony reaction, and joint effusion. It can be primary (idiopathic) without any clear identifiable etiology as well as secondary to variety of systemic diseases most notably lung pathology. Case Presentation. We describe a rare case of primary idiopathic osteoarthropathy in a male patient who presented with severe pain and tenderness in his legs. His history was significant for long standing alcoholism. Physical examination showed severe fingers and toes clubbing. He reported similar changes in his mother. Clinical and radiological findings were remarkable for distal leg tenderness and diffuse periosteal bony reactions, respectively. Computerized tomography scan failed to show any pathology apart from fatty liver infiltration. In the absence of obesity or diabetes, this was consistent with alcoholic steatosis. He was started on nonsteroidal anti-inflammatory drug which dramatically improved his symptoms. Conclusion. Primary hypertrophic osteoarthropathy should be considered in a previously healthy person presenting with bony pain and finger clubbing especially after ruling out the common secondary causes. Moreover, alteration of prostaglandin metabolism secondary to alcoholic consumption might be a contributing factor. Yanal Alnimer, Suresh Subedi, Thair Dawood, and Ghassan Bachuwa Copyright © 2017 Yanal Alnimer et al. All rights reserved. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis Thu, 29 Dec 2016 14:43:35 +0000 The clinical presentation of Systemic Lupus Erythematosus (SLE) is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA), polyarteritis nodosa (PAN), and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA), among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition. Pamela Traisak, Shristi Basnyat, Hala Eid, Patrick Cronin, Halyna Kuzyshyn, and David Feinstein Copyright © 2016 Pamela Traisak et al. All rights reserved. Postchikungunya Chronic Inflammatory Rheumatism Wed, 28 Dec 2016 11:57:10 +0000 A 65-year-old male resident of Guatemala presented with a 5-month history of distal symmetric arthritis and generalized fatigue. This was associated with night sweats, chills, and weight loss. Symptoms were refractory to oral prednisone and hydroxychloroquine. Keith A. Sacco and Razvan M. Chirila Copyright © 2016 Keith A. Sacco and Razvan M. Chirila. All rights reserved. Can Cell Bound Complement Activation Products Predict Inherited Complement Deficiency in Systemic Lupus Erythematosus? Thu, 15 Dec 2016 09:14:50 +0000 Activation of the classical pathway complement system has long been implicated in stimulating immune complex mediated tissue destruction in systemic lupus erythematosus (SLE). C3 and C4 complement levels are utilized as part of SLE diagnosis and monitoring criteria. Recently, cell bound complement activation products (CBCAPs) have shown increased sensitivity in diagnosing and monitoring lupus activity, compared to traditional markers. CBCAPs are increasingly utilized in rheumatology practice as additional serological markers in evaluating SLE patients. We report a case of a patient diagnosed with SLE that had chronically low C3 and C4, along with negative CBCAPs. We surmise that the patient has an inherited complement deficiency as the etiology of her SLE and that CBCAPs could be used to predict such deficiency. Naveen Raj and Barry Waters Copyright © 2016 Naveen Raj and Barry Waters. All rights reserved. Chronic and Asymptomatic Diffuse Alveolar Haemorrhage with Microscopic Polyangiitis: A Case Report and Review of the Literature Mon, 05 Dec 2016 12:05:47 +0000 Diffuse alveolar haemorrhage (DAH) is one of the major causes of death in microscopic polyangiitis (MPA) patients, because of acute respiratory failure with various respiratory symptoms. We, herein, present a case of chronic and asymptomatic DAH in a patient with MPA who was diagnosed by fibreoptic bronchoscopy. The patient showed localized reticular shadows, without any respiratory symptoms, and absence of inflammatory reactions, such as fever and CRP elevation, which is atypical for DAH. Three months after appearance of the lung abnormalities, DAH with MPA was diagnosed by fibreoptic bronchoscopy. She was initially treated with only corticosteroids and has thereafter been maintained with corticosteroids and azathioprine without relapse to date. We reviewed the literature for similar cases and opined that physicians should perform fibreoptic bronchoscopy in MPA patients with chronic lung abnormalities and anaemia to identify DAH, even if the patients show no respiratory symptoms and in the absence of inflammatory reactions. Hiroki Tashiro, Koichiro Takahashi, Hironori Sadamatsu, Masaru Uchida, Shinya Kimura, and Naoko Sueoka-Aragane Copyright © 2016 Hiroki Tashiro et al. All rights reserved. Combination Immunosuppressive Therapy Including Rituximab for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Adult-Onset Still’s Disease Mon, 28 Nov 2016 11:26:13 +0000 Hemophagocytic lymphopcytosis (HLH) is a life-threatening condition. It can occur either as primary form with genetic defects or secondary to other conditions, such as hematological or autoimmune diseases. Certain triggering factors can predispose individuals to the development of HLH. We report the case of a 25-year-old male patient who was diagnosed with HLH in the context of adult-onset Still’s disease (AOSD) during a primary infection with Epstein-Barr virus (EBV). During therapy with anakinra and dexamethasone, he was still symptomatic with high-spiking fevers, arthralgia, and sore throat. His laboratory values showed high levels of ferritin and C-reactive protein. His condition improved after the addition of rituximab and cyclosporine to his immunosuppressive regimen with prednisolone and anakinra. This combination therapy led to a sustained clinical and serological remission of his condition. While rituximab has been used successfully for HLH in the context of EBV-associated lymphoma, its use in autoimmune diseases is uncommon. We hypothesize that the development of HLH was triggered by a primary EBV infection and that rituximab led to elimination of EBV-infected B-cells, while cyclosporine ameliorated the cytokine excess. We therefore propose that this combination immunosuppressive therapy might be successfully used in HLH occurring in the context of autoimmune diseases. Eva Johanna Schäfer, Wolfram Jung, and Peter Korsten Copyright © 2016 Eva Johanna Schäfer et al. All rights reserved. Swift and Complete Healing of Digital Ulcers after Macitentan Treatment Tue, 22 Nov 2016 13:46:13 +0000 Digital ulcers are a burdensome and painful condition with sparse options of treatment. We report the case of a 78-year-old female patient with limited cutaneous systemic sclerosis that sequentially developed digital ulcers. After the appearance of digital ulcers in the soles of her feet she was successfully treated with bosentan. The report of two new digital ulcers in her hands 9 months later alongside with elevated transaminase levels led to a switch to macitentan treatment. A swift and complete healing of both digital ulcers was observed after 3 months, with the restoration of normal biochemical values. Emilio Giner Serret Copyright © 2016 Emilio Giner Serret. All rights reserved. A Case of Dermatomyositis and Anti-EJ Autoantibody with Chronic Intestinal Pseudoobstruction Successfully Treated with Octreotide Thu, 03 Nov 2016 07:47:58 +0000 Chronic intestinal pseudoobstruction (CIPO) is a serious complication in patients with connective tissue disease (CTD) and is sometimes life-threatening or fatal despite intensive medical treatment. Here, we report a patient with dermatomyositis (DM) and anti-EJ autoantibody who developed CIPO that was improved by octreotide. Because her abdominal pain and bloatedness were so severe and persistent, we introduced octreotide to relieve symptoms. In this case, continuous intravenous administration as well as long-acting subcutaneous injection of octreotide was effective for treating CIPO. Chiho Yamada, Shinji Sato, Noriko Sasaki, Takayoshi Kurabayashi, Sho Sasaki, Yasushi Koyama, Naofumi Chinen, Takayuki Wakabayashi, and Yasuo Suzuki Copyright © 2016 Chiho Yamada et al. All rights reserved. Rituximab Not Effective for Hearing Loss in Cogan’s Syndrome Mon, 24 Oct 2016 12:49:34 +0000 Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan’s syndrome. Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan’s syndrome was made. The patient’s hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient’s hearing loss did not improve. Conclusion. Hearing loss in Cogan’s syndrome is difficult to treat. Though rituximab was ineffective in our case, earlier administration in the disease course could be effective for future patients. Daniel R. Bunker and Leslie Dubin Kerr Copyright © 2016 Daniel R. Bunker and Leslie Dubin Kerr. All rights reserved. Hemorrhagic Pericardial Effusion with Tamponade: A Rare Adverse Effect of Infliximab—Case Report and Literature Review Sun, 16 Oct 2016 07:57:50 +0000 Introduction. Antitumor necrosis factor (TNF) alpha agents are commonly used biologic therapies for a wide variety of rheumatic and inflammatory diseases. Here, we present a case of hemorrhagic pericarditis as a consequence of infliximab and review the literature on pericardial complications stemming from this drug class. Methods. For the literature review, search terms using versions of antitumor necrosis factor alpha AND pericardial effusion OR pericarditis OR pleuropericarditis OR cardiac tamponade were used. Results. Pericarditis is a rare but serious complication of anti-TNF based therapy, and hemorrhagic fluid is even more rare, with only one additional case reported. It is likely that this complication was secondary to a robust immune response to very high titer anti-infliximab antibodies. Providers should be aware that this complication can occur and that abnormal elevations in procalcitonin may accompany this unusual finding. Henry D. Lather and J. Michelle Kahlenberg Copyright © 2016 Henry D. Lather and J. Michelle Kahlenberg. All rights reserved. Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease Successfully Treated with Anakinra Wed, 12 Oct 2016 07:17:08 +0000 Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still’s disease (Still’s disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still’s disease, whether it is therapeutic for MAS associated with Still’s disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation. Striking hyperferritinemia was noted without evidence of infection, malignancy, or hemophagocytosis on bone marrow biopsy. NK cells were undetectable in the peripheral blood, whereas soluble IL-2 receptor was elevated. His multiorgan disease resolved in association with methylprednisolone pulse therapy, Anakinra, and a tapering course of prednisone. This case reinforces the notion that Still’s disease is inherently poised to manifest MAS as one of the clinical phenotypes by shedding light on the role of IL-1 underlying both Still’s disease and related MAS. Aswini Kumar and Hiroshi Kato Copyright © 2016 Aswini Kumar and Hiroshi Kato. All rights reserved. Coexistent Pseudogout and Mycobacterium avium-intracellulare Septic Arthritis in a Patient with HIV and ESRD Mon, 10 Oct 2016 09:36:35 +0000 Pseudogout is a crystal-induced arthropathy characterized by the deposition of calcium pyrophosphate dihydrate (CPPD) crystals in synovial fluid, menisci, or articular cartilage. Although not very common, this entity can be seen in patients with chronic kidney disease (CKD). Septic arthritis due to Mycobacterium avium-intracellulare (MAI) is a rare entity that can affect immunocompromised patients such as those with acquired immunodeficiency syndrome (AIDS) or those who are on immunosuppressive drugs. Here, we describe a 51-year-old female who presented with fever, right knee pain, swelling, warmth, and decreased range of motion for several days. The initial assessment was consistent with pseudogout, with negative bacterial and fungal cultures. However, due to high white blood cell (WBC) count in the synovial fluid analysis, she was empirically started on intravenous (IV) vancomycin and piperacillin-tazobactam and discharged on IV vancomycin and cefepime, while acid-fast bacilli (AFB) culture was still in process. Seventeen days later, AFB culture grew Mycobacterium avium-intracellulare (MAI), and she was readmitted for relevant management. This case illustrates that septic arthritis due to MAI should be considered in the differential diagnosis of septic arthritis in immunocompromised patients. Wais Afzal, Omer M. Wali, Kelly L. Cervellione, Bhupinder B. Singh, and Farshad Bagheri Copyright © 2016 Wais Afzal et al. All rights reserved. Adult Onset Henoch-Schonlein Purpura and Intussusception: A Rare Presentation Mon, 26 Sep 2016 16:39:14 +0000 We present an unusual case of a young 26-year-old male who was diagnosed with Henoch-Schonlein Purpura (HSP). Initial presentation was primarily mild gastrointestinal symptoms, which progressed to a life threatening intussusception and subsequently resolved with prompt glucocorticoid use rather than typical surgical intervention. Of importance, the patient’s initial gastrointestinal symptoms without associated skin manifestations made the diagnosis difficult. In conclusion, it is important to recognize uncommon presentations of HSP as it may lead to life threatening complications and surgical intervention may be avoided with prompt treatment. Mridula Krishnan and Joseph Nahas Copyright © 2016 Mridula Krishnan and Joseph Nahas. All rights reserved. A Case of Abdominal Aortic Retroperitoneal and Mesenteric Amyloid Light Chain Amyloidoma Mon, 26 Sep 2016 06:18:30 +0000 We report the case of a Japanese woman with amyloid light chain (AL) amyloidoma in the abdominal aortic retroperitoneum and mesentery. Irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery were initially detected by computed tomography at another hospital. The lesions gradually compressed the duodenum, causing symptoms of bowel obstruction. The patient was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day. However, the lesions did not change in size and her symptoms continued. She was transferred to our hospital and underwent mesenteric biopsy for histopathology using abdominal laparotomy. The histopathological and immunohistological findings of the mesenteric specimen demonstrated lambda light chain deposition. Accordingly, the patient was finally diagnosed with AL amyloidoma with no evidence of systemic amyloidosis. After laparotomy, her general condition worsened because of complications of pneumonia and deep vein thrombosis. She died suddenly from acute myocardial infarction. We have concluded that abdominal aortic retroperitoneal and mesenteric AL amyloidoma may have very poor prognoses in accordance with previous reports. In addition, the size and location of AL amyloidoma directly influence the prognosis. We suggest that early histopathology is important for improving prognosis. Kazuhiro Yokota, Dai Kishida, Hidekazu Kayano, Masahide Yazaki, Yuki Shimada, Yuji Akiyama, and Toshihide Mimura Copyright © 2016 Kazuhiro Yokota et al. All rights reserved. A Curious Case of Proximal Muscle Weakness with Eosinophilic Polymyositis Wed, 14 Sep 2016 14:31:18 +0000 Eosinophilic polymyositis (EPM) is part of a rare disorder, eosinophilic myopathies (EM), which is a form of polymyositis characterized by the presence of eosinophils in muscle biopsy sections and occasionally blood eosinophilia. Herein, we are presenting an interesting case of eosinophilic polymyositis presenting with muscle pain with no other organ systems involved. Ciel Harris, Robert Ali, Julio Perez-Downes, Firas Baidoun, Marianne DeLima, Jaimin Shah, Win Aung, and Raafat F. Makary Copyright © 2016 Ciel Harris et al. All rights reserved. Skin Findings in a Patient with Sjogren’s Syndrome Thu, 08 Sep 2016 17:46:22 +0000 Hypergammaglobulinemic purpura (HGP) is a syndrome constellating recurrent purpura, hypergammaglobulinemia, positive rheumatoid factor (RF), anti-Ro/La antibodies, and elevated erythrocyte sedimentation rate (ESR). We present a case of a 29-year-old female who was diagnosed with Sjogren’s syndrome four years prior to presenting with rash on her lower extremities for a period of 6 months. Skin biopsy at the initial visit was consistent with leukocytoclastic vasculitis and was initiated on treatment for it. Her rash evolved into 2–5 mm scattered purpurae while she was on the treatment and a repeat biopsy showed extravasation of RBCs, a sparse mononuclear infiltrate with deposition of plasma cells, and no evidence of leukocytoclastic vasculitis, thus showing a transition from neutrophilic to mononuclear inflammatory vascular disease which is a rare occurrence. Hypergammaglobulinemic purpura sometimes turns out to be a challenging disease to manage and requires an integrated effort from the primary care doctors, rheumatologist, and dermatologist. Prajwal Boddu, Abdul S. Mohammed, and Sonali Khandelwal Copyright © 2016 Prajwal Boddu et al. All rights reserved. A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy Thu, 08 Sep 2016 09:28:46 +0000 A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy. Naofumi Chinen, Yasushi Koyama, Shinji Sato, and Yasuo Suzuki Copyright © 2016 Naofumi Chinen et al. All rights reserved. Heparin-Related Thrombocytopenia Triggered by Severe Status of Systemic Lupus Erythematosus and Bacterial Infection Tue, 06 Sep 2016 17:35:56 +0000 A patient with severe lupus nephritis developed thrombocytopenia during treatment with high-dose steroids. In addition to viral- or disease-induced cytopenia, the pathology was believed to arise from diverse contributing factors, such as thrombotic microangiopathy and heparin-related thrombocytopenia (HIT). By combining plasma exchange therapy and intravenous cyclophosphamide, we successfully controlled the SLE activity and improved the thrombocytopenia. An antecedent bacterial infection or SLE activity is believed to have contributed to the concurrent HIT. Satoshi Suzuki, Shihoko Nakajima, Taiki Ando, Keisuke Oda, Manabu Sugita, Kunimi Maeda, Yutaka Nakiri, and Yoshinari Takasaki Copyright © 2016 Satoshi Suzuki et al. All rights reserved. Severe Primary Raynaud’s Disease Treated with Rituximab Mon, 29 Aug 2016 11:00:08 +0000 Raynaud’s phenomenon refers to reversible spasms of the peripheral arterioles that can be primary Raynaud’s phenomenon (PRP) or secondary Raynaud’s phenomenon (SRP) to underlying connective tissue disease, both of which are characterized by a triphasic color response triggered by cold exposure or stress. PRP is typically a benign disease, whereas SRP may progress into digital ulcers and/or gangrene. Here, we report a case of a 55-year-old female diagnosed with PRP 7 years ago. Treatment with first-line agents, including calcium channel blocker, aspirin, and phosphodiesterase inhibitor, did not control her symptoms, which progressed to digital ulceration and gangrene. There were no symptoms of underlying autoimmune disease or malignancy, and autoimmune, serology, and immunology test results were normal; a biopsy of her left little finger was negative for vasculitis. Development to critical digital ischemia necessitated treatment with intravenous iloprost and heparin infusion followed by angioplasty, which led to a partial improvement. Due to persistent symptoms, rituximab therapy was initiated and two cycles induced a complete resolution of symptoms. Mohammed Shabrawishi, Abdurahman Albeity, and Hani Almoallim Copyright © 2016 Mohammed Shabrawishi et al. All rights reserved. Repository Corticotropin Injection for Treatment of Idiopathic Inflammatory Myopathies Tue, 23 Aug 2016 14:29:41 +0000 Idiopathic inflammatory myopathies are a group of systemic autoimmune diseases that involve inflammation of skeletal muscle. The two most common forms are dermatomyositis and polymyositis, the former of which entails a skin component. There are few approved therapeutics available for treatment of this group of diseases and the first-line therapy is usually corticosteroid treatment. Considering that a large proportion of patients do not respond to or cannot tolerate corticosteroids, additional treatments are required. There are second-line therapies available, but many patients are also refractory to those options. H.P. Acthar® Gel (repository corticotropin injection [RCI]) is a melanocortin peptide that can induce steroid-dependent effects and steroid-independent effects. Herein, we present a series of cases that involved the use of RCI in the management of dermatomyositis and polymyositis. RCI treatments resulted in improvement in three of four patients, despite failure with previous therapies. The use of RCI did not exacerbate any comorbidity and no significant changes in blood pressure, weight, or glycemic control were observed. Overall, these results are encouraging and suggest that randomized, controlled clinical trials applying RCI to dermatomyositis and polymyositis are warranted. Aarat Patel, Georgia Seely, and Rohit Aggarwal Copyright © 2016 Aarat Patel et al. All rights reserved. Coexistence of Ankylosing Spondylitis and Neurofibromatosis Type 1 Sun, 14 Aug 2016 08:17:10 +0000 Ankylosing spondylitis (AS) is a systemic disease primarily characterized by the inflammation of sacroiliac joints and axial skeleton. Neurofibromatosis type 1 (NF1) is a multisystem genetic disease which is characterized by cutaneous findings, most importantly café-au-lait spots and axillary freckling, by skeletal dysplasia, and by the growth of both benign and malignant nervous system neoplasms, most notably benign neurofibromas. In this case report, we present a 43-year-old male with AS and NF1. Baris Gundogdu, Servet Yolbas, Ahmet Yildirim, Murat Gonen, and Suleyman Serdar Koca Copyright © 2016 Baris Gundogdu et al. All rights reserved.