Case Reports in Rheumatology The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . 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 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 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 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. All rights reserved. Systemic Lupus Erythematosus Presenting with Massive Ascites: A Case of Pseudo-Pseudo Meigs Syndrome Sun, 05 Jun 2016 05:57:23 +0000 The case presented is consistent with the phenomenon known as Pseudo-Pseudo Meigs Syndrome (PPMS). In it, we describe a young woman with newly diagnosed Systemic Lupus Erythematosus presenting with ascites, pleural effusions, and an elevated CA-125 level. Although rare, and of uncertain etiology, PPMS is becoming increasingly recognized in the literature. It should be considered as a differential diagnosis in such patients, along with the search for malignancy. S. McVorran, J. Song, V. Pochineni, and A. Abrudescu-Opran Copyright © 2016 S. McVorran et al. All rights reserved. HIV Infection and Osteoarticular Tuberculosis: Strange Bedfellows Thu, 26 May 2016 09:35:22 +0000 We report the case of a 47-year-old female patient with rheumatoid arthritis and HIV infection presenting with a 3-week history of a painful swollen knee, increased serum inflammatory markers, and a low CD4 lymphocyte count. The diagnosis of TB arthritis was made by synovial fluid culture, GeneXpert/PCR, and confirmed by histopathology of a synovial biopsy. A mini literature review suggests that although HIV infection is associated with extrapulmonary TB, osteoarticular TB is a relatively unusual presentation in an HIV positive patient. The diagnostic utility of the GeneXpert test is explored. We also describe the patient’s good response to an intra-articular corticosteroid injection in combination with standard anti-TB therapy. B. Hodkinson, N. Osman, and S. Botha-Scheepers Copyright © 2016 B. Hodkinson et al. All rights reserved. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis Wed, 18 May 2016 11:52:51 +0000 In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature. Mohammad E. Naffaa, Alexander P. Rozin, Netanel Horowitz, Ofer Ben-Itzhak, Yolanda Braun-Moscovici, and Alexandra Balbir-Gurman Copyright © 2016 Mohammad E. Naffaa et al. All rights reserved. Development of Eosinophilic Fasciitis during Infliximab Therapy for Psoriatic Arthritis Wed, 11 May 2016 14:11:58 +0000 Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue surrounding muscles, nerves, and blood vessels. While its pathogenesis is not entirely understood, this disorder is thought to be autoimmune or allergic in nature. We present here a case of a 59-year-old male who developed peripheral eosinophilia and subsequent eosinophilic fasciitis during treatment with infliximab. To our knowledge, eosinophilic fasciitis has not been previously described in patients during treatment with an inhibitor of tumor necrosis factor α. Richard Hariman, Payal Patel, Jennifer Strouse, Michael P. Collins, and Ann Rosenthal Copyright © 2016 Richard Hariman et al. All rights reserved. Pneumatosis Intestinalis Associated with Juvenile Dermatomyositis Sun, 08 May 2016 12:27:13 +0000 We herein report a case of pneumatosis intestinalis (PI), a condition characterized by the presence of gas within the wall of the digestive tract, associated with juvenile dermatomyositis (JDM). A 16-year-old girl, diagnosed with JDM at the age of 10, presented with abdominal pain and distention. She developed PI based on radiological findings that also included a dilated large intestine, extraluminal gas, and secondary diaphragmatic elevation. She was observed with medical therapy including bowel rest and hyperbaric oxygen therapy. However, she ultimately developed a strangulated obstruction 5 years after presentation with PI and large intestine resection and colostomy were performed emergently. Takako Miyamae, Naoko Ishiguro, Maria Yonezawa, Katsutoshi Tokushige, and Hisashi Yamanaka Copyright © 2016 Takako Miyamae et al. All rights reserved. Concomitant Guillain Barre Syndrome and Transverse Myelitis as Initial Neuropsychiatric Manifestation in a Case of Lupus: A Diagnostic Quandary Sun, 08 May 2016 09:16:56 +0000 Neuropsychiatric manifestations of systemic lupus erythematosus are varied. Presently nineteen in number, they are classified as whether affecting the central or the peripheral compartments of the nervous system. Its diagnosis however remains difficult, more so when two or more of the syndromes are found concomitantly in the same patient and when they occur in absence of the more classical rash, serositis, and haematological manifestations. We present a case of lupus where myelopathy as well as demyelination existed simultaneously as the initial neurologic manifestation. Anshuman Srivastava, Bijit Kumar Kundu, and Diwakar Kumar Singh Copyright © 2016 Anshuman Srivastava et al. All rights reserved. Fever, Myositis, and Paralysis: Is This Inflammatory Myopathy or Neuroinvasive Disease? Mon, 28 Mar 2016 11:28:35 +0000 West Nile virus (WNV) is a mosquito-borne RNA Flavivirus which emerged in North America in 1999. Most patients present with a febrile illness but a few develop WNV neuroinvasive disease. Myopathy is an uncommon manifestation. We describe a case of a 42-year-old male from Los Angeles who presented with 8 days of fever and muscle pain. Initial physical exam was normal except for 4/5 muscle strength testing in his extremity proximal muscles. Laboratory revealed a creatine kinase of 45,000 and a urinalysis with large blood but no red blood cells, suggesting rhabdomyolysis. The patient’s condition declined despite aggressive supportive care and hydration, and on hospital day #6 he developed severe altered mental status and progressed to complete right arm paralysis and 2/5 muscle strength in bilateral legs. EMG/NCS showed sensorimotor axonal polyneuropathy and the cerebrospinal fluid was positive for IgM and IgG WNV antibodies. The patient was diagnosed with WNV neuroinvasive disease, poliomyelitis (and encephalitis) type with myopathy/muscle involvement. He was treated supportively and his muscle and neurologic disease gradually improved. At 12-month follow-up his muscle enzymes had normalized and his weakness had improved to 5/5 strength in bilateral legs and 3/5 strength in the right arm. Aneeta R. Kiran, Richard A. Lau, Kim M. Wu, Andrew L. Wong, Philip J. Clements, and Emil R. Heinze Copyright © 2016 Aneeta R. Kiran et al. All rights reserved. Combined Thenar and Hypothenar Hammer Syndromes and Raynaud’s Phenomenon Successfully Treated with Iloprost Tue, 22 Mar 2016 15:58:57 +0000 Thenar and hypothenar hammer syndromes are uncommon conditions characterised by digital ischemia of the hand as a result of repetitive trauma at level of the thenar and/or hypothenar eminence and damage to the radial and/or ulnar arteries, respectively. The symptoms are related to the mechanism of the trauma and a Raynaud’s phenomenon can be predominant for a long time. The angiography is the “gold standard” imaging technique which allows to confirm the diagnosis. Therapeutic strategy depends on the type of the lesion and severity of symptoms and includes pharmacological (antithrombotic and thrombolytic drugs) and surgical treatments. The authors present a case of a 53-year-old man, carpenter by profession, with combined thenar and hypothenar hammer syndromes and Raynaud’s phenomenon, successfully treated with a short course of intravenous infusion of iloprost. Alessandro Ciapetti, Marina Carotti, Marco Di Carlo, and Fausto Salaffi Copyright © 2016 Alessandro Ciapetti et al. All rights reserved. Herpes Zoster Meningitis Complicating Combined Tocilizumab and Cyclosporine Therapy for Adult-Onset Still’s Disease Tue, 22 Mar 2016 11:40:02 +0000 A 56-year-old female with refractory adult-onset Still’s disease presented with ocular herpes zoster infection during TCZ treatment. After three days of acyclovir treatment (5 mg/kg), she developed a severe headache and high fever. Viral DNA isolation and cerebral spinal fluid abnormalities led to a herpes zoster meningitis diagnosis. Her meningitis was cured by high doses of intravenous acyclovir (10 mg/kg for 14 days). To our knowledge, this is the first report of meningeal herpes zoster infection in rheumatic diseases under TCZ treatment. Shinichiro Tsurukawa, Nozomi Iwanaga, Yasumori Izumi, Atsunori Shirakawa, Chieko Kawahara, Tetsuo Shukuwa, Miwako Inamoto, Atsushi Kawakami, and Kiyoshi Migita Copyright © 2016 Shinichiro Tsurukawa et al. All rights reserved. Successful Discontinuation of Infliximab in a Refractory Case of Vasculo-Behçet Disease Sun, 13 Mar 2016 07:06:22 +0000 Reports have shown that antitumor necrosis factor alpha (anti-TNF-α) agents including infliximab (IFX) can dramatically suppress the disease activity of refractory vasculo-Behçet disease (vasculo-BD). However, it is completely unknown whether we can discontinue anti-TNF-α agents under clinical remission. A 31-year-old patient with vasculo-BD was initially treated with a high dose of steroid and intravenous cyclophosphamide therapy. Six months later, however, the disease recurred. IFX was administered and immediately the disease activity was reduced. Fortunately, we could discontinue IFX after 18-month remission and no recurrence has been observed. Based on previous reports and our patient, all patients who could discontinue IFX sustained clinical remission for at least one year, continued taking immunosuppressive agents such as methotrexate and azathioprine, and had vascular involvements only in non-life-threatening major vessels such as leg or arm arteries/veins. This is a report suggesting the possibility of discontinuation of IFX in vasculo-BD. Akihiro Nakamura, Tomoya Miyamura, and Eiichi Suematsu Copyright © 2016 Akihiro Nakamura et al. All rights reserved. Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options Thu, 03 Mar 2016 11:41:39 +0000 Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 33-year-old African-American male who presented with one-month duration of FUO along with skin rash, sore throat, and arthralgia. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report. Rajesh Gopalarathinam, Eric Orlowsky, Ramesh Kesavalu, and Sreeteja Yelaminchili Copyright © 2016 Rajesh Gopalarathinam et al. All rights reserved. ANA Negative Systemic Lupus Erythematosus Leading to CTEPH, TTP-Like Thrombocytopenia, and Skin Ulcers Wed, 24 Feb 2016 12:23:31 +0000 SLE affects almost every organ system, with differing degrees of severity. During its clinical course periods of flares may alternate with periods of remission culminating in disease and therapy related damage. We describe a case of ANA negative SLE with severe thrombocytopenia, cutaneous vasculitis, antiphospholipid antibody syndrome, and pulmonary artery hypertension. As there is no definitive cure for SLE the treatment lies in caring for the individual organ systems involved and simultaneously taking care of the patient as a whole. Khalid Hamid Changal, Fayaz Sofi, Sheikh Shoaib Altaf, Adnan Raina, and Ab. Hameed Raina Copyright © 2016 Khalid Hamid Changal et al. All rights reserved. A Case of Rituximab Use as an Induction and Maintenance of Remission in ANCA-Associated Vasculitis Tue, 23 Feb 2016 08:41:19 +0000 Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials. Neveen Awad, Shahd Hafiz, Abdurahman Albeity, and Hani Almoallim Copyright © 2016 Neveen Awad et al. All rights reserved. Atypical Cutaneous Manifestations in Adult Onset Still’s Disease Mon, 15 Feb 2016 08:21:02 +0000 Adult Onset Still’s Disease (AOSD), an adult variant of systemic onset juvenile idiopathic arthritis, is a rare systemic inflammatory disorder of unknown aetiology. The rarity of this disease is associated with low index of suspicion and delayed diagnosis in patients suffering from it and in the presence of atypical features the diagnosis can be further challenging. This is a case report on a 24-year-old woman, who was a diagnostic dilemma for 2 years due to the nonspecific symptoms of recurrent fever, generalized maculopapular persistent pruritic and tender rash, and polyarthralgia. She was initially diagnosed as leukocytoclastic vasculitis on a skin biopsy and was managed by a dermatologist with various medications including NSAIDs, hydroxychloroquine, dapsone, colchicine, cyclosporine, and high doses of oral steroids with minimal response. Subsequently, she has had multiple admissions with similar symptoms with raised inflammatory markers and negative septic workup. On one occasion, her iron study revealed hyperferritinaemia which led to the suspicion of AOSD. Once the rheumatic fever and infectious, malignant, autoimmune, and lymphoproliferative disorders were excluded, she was diagnosed as probable AOSD and managed successfully with IL-1 (interleukin-1) receptor antagonist, Anakinra, with remarkable and lasting response both clinically and biochemically. Champa Nataraja and Hedley Griffiths Copyright © 2016 Champa Nataraja and Hedley Griffiths. All rights reserved. An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome Sun, 14 Feb 2016 11:14:20 +0000 Hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH) is an immune mediated phenomenon that can occur in the setting of an autoimmune disease, chronic immunosuppression, malignancy, or infection. It has been more commonly described in the pediatric population and less commonly in adults. We describe a case of a 52-year-old male who presented with a rash. He simultaneously met the Systemic Lupus International Collaborating Clinics (SLICC) criteria for the diagnosis of systemic lupus erythematosus (SLE) and the diagnostic criteria of HS as described in the hemophagocytic lymphohistiocytosis (HLH) 2004 trial. The bone marrow on autopsy showed the presence of abundant hemosiderophages with focal hemophagocytosis. SLE-associated HS might be underdiagnosed due to the overlap in clinical findings. This case represents the importance of prompt diagnosis and treatment of such a potentially fatal clinical syndrome. Saika Sharmeen and Nazia Hussain Copyright © 2016 Saika Sharmeen and Nazia Hussain. All rights reserved. Lymphomatoid Granulomatosis with Paraneoplastic Polymyositis: A Rare Malignancy with Rare Complication Thu, 04 Feb 2016 09:22:03 +0000 Lymphomatoid granulomatosis is a rare Epstein-Barr virus driven lymphoproliferative disease. It most commonly presents with symptoms of lung involvement such as cough, chest tightness, and dyspnea or constitutional symptoms of weight loss, malaise, and fever. The diagnosis is obtained by biopsy and histopathology. Here we report the case of a 31-year-old male who presented with weight loss, rash, and weakness and was diagnosed with lymphomatoid granulomatosis with paraneoplastic polymyositis. We explore the relationship of Epstein-Barr virus with inflammatory myopathy and discuss paraneoplastic inflammatory myopathy. Shanley O’Brien and Paul Schmidt Copyright © 2016 Shanley O’Brien and Paul Schmidt. All rights reserved. Eosinophilic Esophagitis in Two Patients with Systemic Sclerosis Wed, 20 Jan 2016 14:03:36 +0000 The gastrointestinal tract (GIT) is the most common extracutaneous organ system damaged in systemic sclerosis (SSc) and is the presenting feature in 10% of patients. The esophagus as the portion of the GIT is the most commonly affected and there is an association of gastroesophageal reflux (GER) with SSc interstitial lung disease (ILD). Thus, an aggressive treatment for GER is recommended in all SSc patients with ILD; however, it is recognized that a long-term benefit to this treatment is needed to understand its impact. In this case report we discuss the presence of eosinophilic esophagitis (EoE) in two SSc patients and discuss the role for early EGD in SSc patients with moderate-severe GER symptoms for tissue study. Assessment of esophageal biopsy specimens for the presence of eosinophils and possibly ANA can help elucidate disease pathogenesis and direct therapy, as the presence of EoE in SSc has important management considerations, particularly with regards to dietary modification strategies. Tracy M. Frech, Kathleen Boynton, Erinn Downs-Kelly, Bryan Jones, John D. Kriesel, and Kathryn Peterson Copyright © 2016 Tracy M. Frech et al. All rights reserved. Rheumatoid Arthritis, Kartagener’s Syndrome, and Hyperprolactinemia: Who Started It? Wed, 20 Jan 2016 08:58:29 +0000 We report a case of an 18-year-old girl who presented to our hospital with history of recurrent respiratory infections, amenorrhea, and symmetric polyarthritis. She was diagnosed with rheumatoid arthritis (RA), Kartagener’s syndrome (KS), and hyperprolactinemia. There have been very few case reports in the literature of RA occurring in the setting of KS, theoretically proposed to be due to chronic stimulation of the immune system by recurrent infections. Furthermore, hyperprolactinemia has been hypothesized to mirror RA disease activity and case reports of treatment with dopamine agonists have led to the speculation of whether or not they represent a new line of experimental treatment in the future. Our patient was found to have both KS and hyperprolactinemia together in the setting of RA, and based on our literature search, this is the first reported case of such a combination. This strikes a very intriguing question: are these three conditions interlinked by a yet to be defined association? And treatment of which condition leads to the resolution of the other? Hussein Halabi and Israa Mulla Copyright © 2016 Hussein Halabi and Israa Mulla. All rights reserved. Pulmonary Disease Secondary to Reflux Mimicking Interstitial Pneumonia in Systemic Sclerosis: Case Report and Literature Review Thu, 14 Jan 2016 16:05:45 +0000 Systemic sclerosis is a complex disease due to the variety of clinical presentations, often superimposed on other conditions, related or not to the connective tissue. We report a 43-year-old Brazilian woman with limited systemic sclerosis and pulmonary symptoms secondary to gastroesophageal reflux disease, with a clinical presentation similar to a diffuse interstitial lung disease. Because of the frequency of interstitial lung injury due to systemic sclerosis, this was an important differential diagnosis, which could be excluded after optimized treatment of reflux disease, with clinical and radiological improvement. Clinical management of patients with collagen diseases requires clinician skills to identify the natural history and understand its nuances. This is a common situation in clinical practice, but with a few discussions in international literature. Ricardo Azêdo de Luca Montes, Nathalia Mazolli Veiga, Pedro Gemal Lanzieri, and Luis Otávio Cardoso Mocarzel Copyright © 2016 Ricardo Azêdo de Luca Montes et al. All rights reserved. Polyarteritis Nodosa Presenting as Digital Gangrene and Breast Lesion following Exposure to Silicone Breast Implants Wed, 30 Dec 2015 13:54:26 +0000 Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis of small and medium sized arteries. We report a case of a 49-year old woman who presented with PAN following exposure to silicone breast implants. Although the relationship between silicone implants and connective tissue diseases has been investigated in the literature, no prior reports were found documenting PAN after silicone mammoplasty. While the pathogenesis of idiopathic PAN is not known yet, responsiveness to immunosuppressive therapy may suggest an immunologic mechanism. More robust research is needed to understand the connection between silicone breast implants and autoimmunity. Yamen Homsi, John Andrew Carlson, and Samer Homsi Copyright © 2015 Yamen Homsi et al. All rights reserved. Coexistence of Takayasu Arteritis and Crohn’s Disease in a Maltese Patient Tue, 29 Dec 2015 07:28:19 +0000 Takayasu arteritis (TA) and Crohn’s disease (CD) are uncommon chronic granulomatous disorders affecting the large arteries and the gastrointestinal tract, respectively. At least 40 occurrences of these two conditions in the same patient have been reported in the literature, raising the possibility of an association between them. We report the coexistence of TA and CD in a young Maltese patient and review the literature to discuss possible aetiological mechanisms that might explain this association. John Paul Caruana Galizia and Paul John Cassar Copyright © 2015 John Paul Caruana Galizia and Paul John Cassar. All rights reserved. Successful Treatment with Intravenous Cyclophosphamide for Refractory Adult-Onset Still’s Disease Tue, 22 Dec 2015 07:09:52 +0000 We report a 64-year-old female case of intractable adult-onset Still’s disease (AOSD). Initial high-dose steroid therapy combined with cyclosporin A was ineffective against macrophage-activation syndrome (MAS), which was accompanied by the systemic type of AOSD. Treatment for MAS with intravenous cyclophosphamide resulted in remission of AOSD and a reduction in the high doses of steroids. Efficacy of biologics against MAS in AOSD is unclear. Cyclophosphamide, a conventional cytotoxic agent, should be considered as one of the therapeutic options for refractory types of AOSD with MAS. Yoshika Tsuji, Nozomi Iwanaga, Anna Adachi, Kinuyo Tsunozaki, Yasumori Izumi, Yuji Moriwaki, Kazuhiro Kurohama, Masahiro Ito, Atsushi Kawakami, and Kiyoshi Migita Copyright © 2015 Yoshika Tsuji et al. All rights reserved. Cutaneous Ulcers as Initial Presentation of Localized Granulomatosis with Polyangiitis: A Case Report and Review of the Literature Tue, 17 Nov 2015 14:10:21 +0000 Background. Granulomatosis with polyangiitis (GPA) is an ANCA associated small vessel vasculitis characterized by necrotizing granulomatous inflammation involving the upper and the lower respiratory tract and the kidneys. The disease has a broad clinical spectrum that ranges from limited/localized involvement of a single organ system to a generalized systemic vasculitis that affects several organs with evidence of end organ damage. Atypical forms of the disease have been recognized with or without respiratory tract involvement with a long protracted course before manifesting as generalized disease. Case Presentation. We describe a 57-year-old woman who presented with recurrent fever and cutaneous ulcers on her legs who was diagnosed to have granulomatosis with polyangiitis (GPA) after an extensive evaluation which excluded infectious, other vasculitides, connective tissue disease and malignant etiologies. Conclusion. In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician. Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease. Noreen Nasir, Syed Ahsan Ali, and Hafiz Mohammed Mehmood Riaz Copyright © 2015 Noreen Nasir et al. All rights reserved. Dramatic Improvement of Subcutaneous Calcinosis by Intermittent, High-Dose Etidronate plus Cimetidine in a Patient with Juvenile Dermatomyositis Thu, 12 Nov 2015 09:15:21 +0000 A 17-year-old boy with juvenile dermatomyositis presented with typical skin symptoms, mild myositis, and bilateral lower limb calcinosis. His skin and muscle symptoms responded to treatment with prednisolone and azathioprine. However, calcinosis did not improve, and the patient had a limited range of knee joint motion and resultant disturbance of daily activities. Cimetidine was combined with intermittent administration of high-dose etidronate, leading to marked improvement of both subcutaneous and muscular calcinosis with no skeletal adverse reactions during a long treatment period exceeding 5 years. As a result, the range of knee joint motion has increased and performance of daily activities has improved. Takayuki Wakabayashi, Noriko Sasaki, Naofumi Chinen, and Yasuo Suzuki Copyright © 2015 Takayuki Wakabayashi et al. All rights reserved. Efficacy of Adalimumab in a Girl with Refractory Intestinal Behcet’s Disease Mon, 02 Nov 2015 06:43:56 +0000 We describe our experience with a juvenile patient who had refractory intestinal Behcet’s disease that responded to adalimumab, a fully humanized antibody against soluble TNF- and its receptor. The patient, a 13-year-old girl, presented with oral aphthous ulcers, vulvar pain, and rashes on the lower extremities. She gradually developed a low-grade fever, abdominal pain, diarrhea, and hematochezia. Lower gastrointestinal endoscopy revealed ulcers in the terminal ileum, consistent with intestinal Behcet’s disease. Methylprednisolone pulse therapy was initiated, after which the symptoms transiently improved, but, during the corticosteroid taper, the abdominal pain recurred. The symptoms resolved soon after the administration of adalimumab. Of importance, the dose of corticosteroids was successfully reduced without exacerbation during 8 months of observation. This is the first reported case in which adalimumab was used for pediatric gastrointestinal Behcet’s disease. Adalimumab is a good choice for intestinal Behcet’s disease refractory to conventional treatment. Mariko Kaji, Takayuki Kishi, Takako Miyamae, Satoru Nagata, Hisashi Yamanaka, and Satoshi Fujikawa Copyright © 2015 Mariko Kaji et al. All rights reserved. A Case of Cerebral Vasculitis Associated with Ulcerative Colitis Sun, 18 Oct 2015 07:25:02 +0000 Ulcerative colitis (UC) is a chronic, debilitating condition characterized by inflammation of the colonic mucosa. It is regarded as a systemic inflammatory disorder that can affect a number of organ systems. Central nervous system disease associated with UC is a rare sequela of inflammatory bowel disease, occurring in less than 5% of cases. These manifestations include arterial and venous thrombosis, leukoencephalitis, seizures, and vasculitis. We present a case of a 61-year-old female with a two-year history of well-controlled ulcerative colitis, who developed altered mental status and weakness. On brain imaging, she was found to have cerebral lesions which were biopsied. Histopathology subsequently revealed coagulative necrosis and inflammation characteristic of vasculitis. Rheumatology serologies were negative, and the patient was started on steroids that dramatically improved her neurological function, with no residual deficits, and led to resolution of the brain lesions. Naveen Raj, Matthew Arkebauer, Barry Waters, and Brucha Dickinson Copyright © 2015 Naveen Raj et al. All rights reserved. A Case of Amyopathic Dermatomyositis with Pneumomediastinum and Subcutaneous Emphysema Sun, 18 Oct 2015 06:38:27 +0000 A 34-year-old man was admitted with dyspnea, cough, and fever. Thorax computed tomography revealed ground glass opacities and pneumomediastinum. The patient was diagnosed as amyopathic dermatomyositis due to skin lesions and radiological findings. Despite immunosuppressive treatment clinical deterioration and radiological progression were observed and the patient died because of severe hypoxemic respiratory failure. The patient presented with extremely rare occurrence of pneumomediastinum and subcutaneous emphysema in amyopathic dermatomyositis with a poor prognosis. Aslıhan Gürün Kaya, Aydın Çiledağ, Orhan Küçükşahin, Özlem Özdemir Kumbasar, and Çetin Atasoy Copyright © 2015 Aslıhan Gürün Kaya et al. All rights reserved.