Case Reports in Rheumatology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . 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 http://www.hindawi.com/journals/crirh/2016/8605274/ 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 http://www.hindawi.com/journals/crirh/2016/1718309/ 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 http://www.hindawi.com/journals/crirh/2016/9510316/ 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 http://www.hindawi.com/journals/crirh/2016/8352893/ 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 http://www.hindawi.com/journals/crirh/2016/2576496/ 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 http://www.hindawi.com/journals/crirh/2016/3717392/ 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 http://www.hindawi.com/journals/crirh/2016/5495928/ 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 http://www.hindawi.com/journals/crirh/2016/3957605/ 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 http://www.hindawi.com/journals/crirh/2016/4146030/ 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 http://www.hindawi.com/journals/crirh/2016/7810916/ 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 http://www.hindawi.com/journals/crirh/2016/4829459/ 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 http://www.hindawi.com/journals/crirh/2016/9565427/ 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 http://www.hindawi.com/journals/crirh/2016/6571621/ 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 http://www.hindawi.com/journals/crirh/2016/2053804/ 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 http://www.hindawi.com/journals/crirh/2016/9068061/ 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 http://www.hindawi.com/journals/crirh/2016/4039801/ 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. Polymyalgia Rheumatica Revealing a Lymphoma: A Two-Case Report Thu, 11 Aug 2016 11:53:20 +0000 http://www.hindawi.com/journals/crirh/2016/2986297/ Introduction. Polymyalgia rheumatica (PMR) is one of the most common inflammatory rheumatism types in elderly population. The link between cancer and PMR is a matter of debate. Methods. We report two cases of PMR leading to the diagnosis of lymphoma and the growing interest of PET-TDM in this indication. Results. A 84-year-old man known for idiopathic neutropenia presented an inflammatory arthromyalgia of the limb girdle since one month. Blood exams highlighted the presence of a monoclonal B cell clone. Bone marrow concluded to a B cell lymphoma of the marginal zone. He was successfully treated with 0.3 mg/kg/d of prednisone, and response was sustained after 6 months. A 73-year-old man known for prostatic neoplasia in remission for 5 years presented arthromyalgia of the limb girdle since one month. PET-CT revealed bursitis of the hips and the shoulders, no prostatic cancer recurrence, and a metabolically active iliac lymphadenopathy whose pathologic exam concluded to a low grade follicular lymphoma. He was successfully treated with 0.3 mg/kg/d of prednisone. Conclusion. These observations may imply that lymphoma is sometimes already present when PMR is diagnosed and PET-CT is a useful tool in the initial assessment of PMR to avoid missing neoplasia. Frank Verhoeven, Xavier Guillot, Mickaël Chouk, Clément Prati, and Daniel Wendling Copyright © 2016 Frank Verhoeven et al. All rights reserved. Olecranon Bursitis Caused by Candida parapsilosis in a Patient with Rheumatoid Arthritis Tue, 09 Aug 2016 10:16:25 +0000 http://www.hindawi.com/journals/crirh/2016/2019250/ Septic bursitis is usually caused by bacterial organisms. However, infectious bursitis caused by fungi is very rare. Herein, we present a 68-year-old woman with long-standing rheumatoid arthritis who developed pain, erythema, and swelling of the right olecranon bursa. Aspiration of the olecranon bursa showed a white blood cell count of (41% neutrophils, 30% lymphocytes, and 29% monocytes). Fluid culture was positive for Candida parapsilosis. She was treated with caspofungin 50 mg intravenously daily for 13 days followed by fluconazole 200 mg orally daily for one week. She responded well to this treatment but had recurrent swelling of the bursa. Bursectomy was recommended but she declined this option. This case, together with other reports, suggests that the awareness of uncommon pathogens, their presentation, and predisposing risk factors are important to establish an early diagnosis and prevent long-term complications. Carla F. Gamarra-Hilburn, Grissel Rios, and Luis M. Vilá Copyright © 2016 Carla F. Gamarra-Hilburn et al. All rights reserved. Cutaneous Necrotizing Vasculitis and Leukopenia in a Cocaine User: Is Levamisole the Culprit? Sun, 07 Aug 2016 12:55:32 +0000 http://www.hindawi.com/journals/crirh/2016/2685267/ Levamisole is an antihelminthic drug banned by the US Food and Drug Administration (FDA) in 2000 because of its dangerous side effects. Over the past few years, it has been identified as an adulterant in cocaine and reported to cause cutaneous vasculitis in cocaine users. The health burden of levamisole is serious since it is estimated that over 5 million Americans use cocaine and that 70% of the cocaine used in the USA contains levamisole. In this paper we report the case of a 23-year-old female cocaine user that presented with purpuric rash and skin necrosis, found to have positive c-ANCA and anti-proteinase 3 antibodies. Her skin biopsy showed fibroconnective tissue with signs of necrosis, acute and chronic inflammation, and thrombus formation. She was diagnosed with levamisole-induced vasculitis and successfully treated with withdrawal of cocaine use and local wound care. Lara El Khoury, Nabil Zeineddine, Richard Felix, and Mark Goldstein Copyright © 2016 Lara El Khoury et al. All rights reserved. A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma Sun, 31 Jul 2016 10:09:57 +0000 http://www.hindawi.com/journals/crirh/2016/8536341/ Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly. Cody M. Lee, Diana Girnita, Arundhati Sharma, Surabhi Khanna, and Jean M. Elwing Copyright © 2016 Cody M. Lee et al. All rights reserved. Henoch-Schönlein Purpura with Adalimumab Therapy for Ulcerative Colitis: A Case Report and Review of the Literature Wed, 27 Jul 2016 10:53:46 +0000 http://www.hindawi.com/journals/crirh/2016/2812980/ Tumor necrosis factor-α (TNFα) inhibitor therapy has signified an important milestone in the fight against many rheumatological disorders and inflammatory bowel disease (IBD). Cutaneous adverse events caused by this class of medications are well known but relatively uncommon. Most reactions are mild and rarely warrant treatment withdrawal. Henoch-Schönlein purpura (HSP) is a disease with cutaneous vasculitis, arthritis, and gastrointestinal and renal involvement that is usually seen in children, though the worst complications are typically seen in adults. We present a case of HSP complicating adalimumab treatment in a patient with ulcerative colitis who had achieved endoscopic remission. We review similar cases reported in the literature and discuss the consequences of these autoimmune diseases. Joseph J. LaConti, Jean A. Donet, Jeong Hee Cho-Vega, Daniel A. Sussman, Dana Ascherman, and Amar R. Deshpande Copyright © 2016 Joseph J. LaConti et al. All rights reserved. Pauci-Immune Crescentic Glomerulonephritis in Connective Tissue Disease Mon, 18 Jul 2016 17:28:15 +0000 http://www.hindawi.com/journals/crirh/2016/9070487/ Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren’s syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus. In this small population of patients there is no correlation with ANCAs. Most of the patients were treated with aggressive immunosuppression and did well if they were treated early in the course of their disease. One of our patients required renal transplant, but she presented late in the course of her disease, as evidenced by chronicity on her renal biopsy. Whether these patients are overlap of vasculitis and other connective tissue diseases or to be considered as a separate entity is yet to be described. Clinicians must be aware of these presentations because initial presentation can be severe. Supraja Yeturi, Mary Cronin, Adam Robin, Campbell Lorna, and Ann K. Rosenthal Copyright © 2016 Supraja Yeturi et al. All rights reserved. Toxic Epidermal Necrolysis-Like Lesions and Systemic Lupus Erythematosus Possibly Triggered by Sulfasalazine Tue, 12 Jul 2016 11:08:59 +0000 http://www.hindawi.com/journals/crirh/2016/4501937/ This case report describes a patient with arthritis of the large joints, bilateral sacroiliitis, and positive anti-SSA and anti-dsDNA antibody, who received sulfasalazine and shortly thereafter became critically ill. He developed toxic epidermal necrolysis, hemolytic anemia, lymphopenia, markedly elevated ferritin, and muscle wasting. A diagnosis of systemic lupus erythematosus was made, and mycophenolate mofetil and systemic glucocorticoids brought this severe disease under control. Toxic epidermal necrolysis-like lesions and hemophagocytic syndrome have been reported as manifestations of systemic lupus erythematosus. This patient possibly had spondyloarthritis or an undifferentiated connective tissue disease at presentation, and we suggest, based on the timing of events, that sulfasalazine may have acted as a trigger of the severe disease manifestations. Simon Krabbe, Cigdem Gül, Bjarne Andersen, and Niels Tvede Copyright © 2016 Simon Krabbe et al. All rights reserved. Giant Cell Arteritis: An Atypical Presentation Diagnosed with the Use of MRI Imaging Mon, 04 Jul 2016 10:54:26 +0000 http://www.hindawi.com/journals/crirh/2016/8239549/ Giant cell arteritis (GCA) is the most common primary systemic vasculitis in western countries in individuals over the age of 50. It is typically characterised by the granulomatous involvement of large and medium sized blood vessels branching of the aorta with particular tendencies for involving the extracranial branches of the carotid artery. Generally the diagnosis is straightforward when characteristic symptoms such as headache, jaw claudication, or other ischemic complications are present. Atypical presentations of GCA without “overt” cranial ischemic manifestations have become increasingly recognised but we report for the first time a case of GCA presenting as mild upper abdominal pain and generalized weakness in the context of hyponatremia as the presenting manifestation of vasculitis that was subsequently diagnosed by MRI scanning. This case adds to the literature and emphasises the importance of MRI in the evaluation of GCA patients without “classic” cranial ischemic symptoms. Siddesh Shambhu and Lisbet Suarez Copyright © 2016 Siddesh Shambhu and Lisbet Suarez. All rights reserved. Assessment and Surgical Treatment of Calcinosis of the Shoulder Associated with CREST Syndrome Wed, 29 Jun 2016 09:57:19 +0000 http://www.hindawi.com/journals/crirh/2016/9759182/ We report an unusual case of a 65-year-old lady with CREST syndrome with multiple upper and lower limb calcinosis, who presented with severe shoulder pain and stiffness, with widespread intra- and extra-articular calcinosis, which was refractory to conservative measures. We were able to identify the main cause of her symptoms through serial diagnostic injections as calcific biceps tendinosis. We will discuss her assessment and surgical management and the pathophysiology and various treatment modalities for managing the soft tissue calcinosis in rheumatological diseases. R. Manohara and S. J. Breusch Copyright © 2016 R. Manohara and S. J. Breusch. All rights reserved. Triple Valvulopathy and Jaccoud’s Arthropathy: A Case Report and Literature Review Tue, 28 Jun 2016 15:07:21 +0000 http://www.hindawi.com/journals/crirh/2016/4147068/ Cardiac involvement is fairly common in patients with systemic lupus erythematosus (SLE). It may involve all layers of the heart and coronary arteries as well as the heart valves. We report an extremely rare presentation of valvulitis and valvular dysfunction associated with systemic lupus erythematosus. This is the first case of lupus valvulitis which required three mechanical prosthetic valve replacements with disease recurrence leading to a fatal outcome. This is, in our point of view, the consequence of aggressive natural history of the disease and perhaps late diagnosis and treatment of underlying SLE which was unsuccessful. Ali Naderi Mahabadi, Bassam Alhaddad, and Stanley Ballou Copyright © 2016 Ali Naderi Mahabadi et al. All rights reserved. Recurrent Interstitial Pneumonitis in a Patient with Entero-Behçet’s Disease Initially Treated with Mesalazine Sun, 26 Jun 2016 12:13:15 +0000 http://www.hindawi.com/journals/crirh/2016/5636489/ A 65-year-old man with entero-Behçet’s disease (BD) being treated with mesalazine was presented to our hospital complaining of dyspnea. Computed tomography (CT) of the chest showed ground-glass opacities and he was initially diagnosed with mesalazine-induced interstitial pneumonitis (IP). Besides the discontinuation of mesalazine, a high dose of oral prednisolone was administered and the patient seemed to recover. However, four months later, dyspnea recurred and repeated CT revealed more extensive pulmonary infiltration despite steroid therapy. After the exclusion of infections, we suspected either a recurrence of mesalazine-induced IP or BD-related IP as a clinical manifestation of BD. The patient was treated with intravenous methylprednisolone and cyclophosphamide, followed by orally administered azathioprine, based on the assumption of underlying vasculitis. Thereafter, his condition improved. BD-related IP is an extremely rare condition with limited reports in the literature. Mesalazine-induced IP is also uncommon but the prognosis is generally good after discontinuation of mesalazine with or without steroid therapy. We discuss an extremely rare case, especially focusing on BD-related IP and mesalazine-induced IP as a potential cause of recurrent IP in a patient with entero-BD. Akihiro Nakamura, Tomoya Miyamura, Brian Wu, and Eiichi Suematsu Copyright © 2016 Akihiro Nakamura et al. All rights reserved. An Autopsy Case of Fulminant Amebic Colitis in a Patient with a History of Rheumatoid Arthritis Sun, 12 Jun 2016 07:49:06 +0000 http://www.hindawi.com/journals/crirh/2016/8470867/ Generally, amebic colitis is localized around the mucosal membrane and often accompanied by diarrhea and abdominal pain. We describe a patient with a history of rheumatoid arthritis who had received prolonged steroid therapy. The patient complained of breathing difficulties because of rheumatoid lung disease. Although the patient was given antibacterial agent, the symptoms did not improve until death. We did an autopsy and found that he had fulminant amebic colitis, although the patient was not previously examined. Histochemical analysis revealed severe inflammation and full-thickness necrosis of the colon by ameba, suggesting the involvement of ameba in the progression of the overall condition. Naoko Kawabe, Fuyuki Sato, Miho Nagasawa, Masako Nakanishi, and Yasuteru Muragaki Copyright © 2016 Naoko Kawabe et al. All rights reserved. Catastrophic Antiphospholipid Syndrome Tue, 07 Jun 2016 12:49:49 +0000 http://www.hindawi.com/journals/crirh/2016/4161439/ This paper reports one case of successfully treated patients suffering from a rare entity, the catastrophic antiphospholipid syndrome (CAPS). Management of this patient is discussed in detail. Rawhya R. El-Shereef, Zein El-Abedin, Rashad Abdel Aziz, Ibrahim Talat, Mohammed Saleh, Hanna Abdel-Samia, Amro Sameh, and Mahmoud Sharha Copyright © 2016 Rawhya R. El-Shereef et al. All rights reserved. Undiagnosed Sjögren’s Syndrome Presenting as Mesenteric Panniculitis Sun, 05 Jun 2016 11:12:06 +0000 http://www.hindawi.com/journals/crirh/2016/7207638/ Mesenteric panniculitis is a rare inflammatory and fibrotic process that affects the small intestine mesentery. It may occur following abdominal surgery or in association with a variety of conditions, including malignancy, infection, and certain autoimmune and inflammatory conditions. Herein, an unusual case of mesenteric panniculitis in a patient with primary Sjögren’s syndrome will be presented. The patient presented with abdominal pain, weight loss, sicca symptoms, fatigue, and arthralgia. An abdominal CT revealed mesenteric fat stranding and prominent lymph nodes of the small intestine mesentery. She was found on laboratory workup to have positive antinuclear and anti-SSa antibodies. Minor salivary gland lip biopsy revealed focal lymphocytic sialadenitis. The patient’s symptoms and CT findings improved with corticosteroids. This case suggests that Sjögren’s syndrome should be considered as an underlying disease process in the evaluation of patients with mesenteric panniculitis. Rebecca L. Burns and Sharukh J. Bhavnagri Copyright © 2016 Rebecca L. Burns and Sharukh J. Bhavnagri. 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