Case Reports in Rheumatology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Fever, Myositis, and Paralysis: Is This Inflammatory Myopathy or Neuroinvasive Disease? Mon, 28 Mar 2016 11:28:35 +0000 http://www.hindawi.com/journals/crirh/2016/5395249/ 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 http://www.hindawi.com/journals/crirh/2016/4824929/ 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 http://www.hindawi.com/journals/crirh/2016/4232657/ 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 http://www.hindawi.com/journals/crirh/2016/1467583/ 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 http://www.hindawi.com/journals/crirh/2016/6502373/ 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 http://www.hindawi.com/journals/crirh/2016/4507247/ 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 http://www.hindawi.com/journals/crirh/2016/7275860/ 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 http://www.hindawi.com/journals/crirh/2016/4835147/ 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 http://www.hindawi.com/journals/crirh/2016/8957690/ 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 http://www.hindawi.com/journals/crirh/2016/8242597/ 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 http://www.hindawi.com/journals/crirh/2016/6410421/ 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 http://www.hindawi.com/journals/crirh/2016/7367232/ 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 http://www.hindawi.com/journals/crirh/2016/5926327/ 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 http://www.hindawi.com/journals/crirh/2015/765170/ 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 http://www.hindawi.com/journals/crirh/2015/384257/ 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 http://www.hindawi.com/journals/crirh/2015/163952/ 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 http://www.hindawi.com/journals/crirh/2015/517025/ 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 http://www.hindawi.com/journals/crirh/2015/817592/ 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 http://www.hindawi.com/journals/crirh/2015/716138/ 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 http://www.hindawi.com/journals/crirh/2015/598273/ 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 http://www.hindawi.com/journals/crirh/2015/813902/ 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. An Unusual Association: Iliopsoas Bursitis Related to Calcium Pyrophosphate Crystal Arthritis Thu, 15 Oct 2015 12:12:58 +0000 http://www.hindawi.com/journals/crirh/2015/935835/ A 71-year-old man with osteoarthritis and chondrocalcinosis came to our observation developing a swelling in the groin region after a recent left colectomy for adenocarcinoma. The imaging techniques revealed the presence of an iliopsoas bursitis in connection with the hip. The synovial fluid analysis detected the presence of calcium pyrophosphate (CPP) crystals and allowed the final and unusual diagnosis of iliopsoas bursitis related to acute CPP crystal hip arthritis. Marco Di Carlo, Antonella Draghessi, Marina Carotti, and Fausto Salaffi Copyright © 2015 Marco Di Carlo et al. All rights reserved. Effectiveness of Certolizumab Pegol in Treating Rheumatoid Arthritis Patients with Persistent Inflamed Residual Mono- or Oligosynovitis Resistant to Prior TNF-α Inhibitors Mon, 14 Sep 2015 12:30:33 +0000 http://www.hindawi.com/journals/crirh/2015/348614/ We report four cases of successful treatment with certolizumab pegol (CZP) of rheumatoid arthritis (RA) patients with persistent inflamed residual mono- or oligosynovitis resistant to prior TNF-α inhibitors. Although the patients were in a moderate disease activity, a low activity, or a remission of RA, they sustained inflammatory mono-/oligoarthritis even after treatment with prior TNF inhibitors. They were then all treated with CZP and observed in a serial ultrasonography. In all cases, the positive power Doppler signals in the joint have disappeared promptly and all of the patients were able to retain remission in the long term. The treatment of CZP to the refractory mono-/oligoarthritis of inflammatory synovitis in RA patients has not been previously described. The cases suggest that it may be associated with the feature of CZP, possible effective penetration into the site of inflammation. Syuichi Koarada, Masahiko Tsuboi, Mitsunori Komine, Yoshinobu Nakao, Yukiko Tokuda, Yukihide Ono, Satoko Tashiro, Akihito Maruyama, Nobuyuki Ono, Akihide Ohta, and Yoshifumi Tada Copyright © 2015 Syuichi Koarada et al. All rights reserved. A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome Mon, 17 Aug 2015 13:17:20 +0000 http://www.hindawi.com/journals/crirh/2015/528026/ Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG. Helen Chioma Okoh, Sandeep Singh Lubana, Spencer Langevin, Susan Sanelli-Russo, and Adriana Abrudescu Copyright © 2015 Helen Chioma Okoh et al. All rights reserved. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis Tue, 11 Aug 2015 08:12:04 +0000 http://www.hindawi.com/journals/crirh/2015/513602/ Granulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. Robert Ali, Candice Baldeo, Jesse Onyenekwe, Roshan Lala, Cristian Landa, and Anwer Siddiqi Copyright © 2015 Robert Ali et al. All rights reserved. Sarcoidosis and Histoplasmosis: Is One a Consequence of the Other? A Case Report and Review of the Literature Thu, 06 Aug 2015 13:27:54 +0000 http://www.hindawi.com/journals/crirh/2015/108459/ Sarcoidosis involves abnormal collections of inflammatory cells (granulomas) which may form as nodules in multiple organs. 90% of affected patients have respiratory tract abnormalities. We present a 61-year-old male with sarcoidosis who was admitted for respiratory distress. Fibrosing mediastinitis was seen in the chest computograph. Management was conservative and included steroids, antibiotics, and oxygen therapy. Sarcoidosis and fibrosing mediastinitis are rare. Fibrosing mediastinitis is more commonly seen with histoplasmosis. We explore the clinical similarities between histoplasmosis and sarcoidosis. We also explore the potential cause and effect relationship and workup for each disease entity. Anupam Bansal and Rupali Drewek Copyright © 2015 Anupam Bansal and Rupali Drewek. All rights reserved. Glucocorticoid-Responsive Cold Agglutinin Disease in a Patient with Rheumatoid Arthritis Thu, 06 Aug 2015 09:25:24 +0000 http://www.hindawi.com/journals/crirh/2015/823563/ A 57-year-old man with rheumatoid arthritis developed severe anemia during treatment with adalimumab plus methotrexate. Cold agglutinin disease was diagnosed because haptoglobin was undetectable, cold agglutinin was positive (1 : 2048), and the direct Coombs test was positive (only to complement). Although the cold agglutinin titer was normalized (1 : 64) after treatment with prednisolone (0.7 mg/kg/day for two weeks), the patient’s hemoglobin did not increase above 8 g/dL. When cold agglutinins were reexamined using red blood cells suspended in bovine serum albumin, the titer was still positive at 1 : 1024. Furthermore, the cold agglutinin had a wide thermal amplitude, since the titer was 1 : 16 at 30°C and 1 : 1 at 37°C. This suggested that the cold agglutinin would show pathogenicity even at body temperature. After the dose of prednisolone was increased to 1 mg/kg/day, the patient’s hemoglobin rapidly returned to the normal range. The thermal amplitude test using red blood cells suspended in bovine serum albumin is more sensitive than the standard test for detecting pathogenic cold agglutinins. Kyoko Honne, Takao Nagashima, Masahiro Iwamoto, Toyomi Kamesaki, and Seiji Minota Copyright © 2015 Kyoko Honne et al. All rights reserved. Embolic Stroke as the Initial Manifestation of Systemic Lupus Erythematosus Wed, 22 Jul 2015 08:16:34 +0000 http://www.hindawi.com/journals/crirh/2015/373201/ We present a case of a 21-year-old African-American female with no significant medical history, who presented to the emergency department with a one-week history of blurry and double vision. Ophthalmology evaluation revealed bilateral retinal artery occlusion. Further workup with imaging of the brain was consistent with an ischemic stroke. Hereditary hypercoagulable workup was unremarkable and initial testing for antiphospholipid syndrome was positive. She underwent transesophageal echocardiogram (TEE), which showed severe mitral regurgitation and thickening of mitral valve leaflets consistent with Libman-Sacks endocarditis. Autoimmune workup was positive for IF-ANA, anti-RNP, and anti-Smith antibody. She fulfilled 4/11 of the ACR criteria and met 5 of the SLICC (Systemic Lupus International Collaborating Clinics) criteria for lupus (nonscaring alopecia, thrombocytopenia, positive ANA, and positive anti-Smith and positive anti-phospholipid antibodies). This case highlights the importance of early recognition of underlying connective tissue diseases and timely management of these diseases in young patients with no previous manifestations of diseases. Reshma M. Khan, Rajaie Namas, Sachin Parikh, and Bernard Rubin Copyright © 2015 Reshma M. Khan et al. All rights reserved. A Case of Docetaxel Induced Myositis and Review of the Literature Thu, 16 Jul 2015 14:00:29 +0000 http://www.hindawi.com/journals/crirh/2015/795242/ In phase I and II trials taxane chemotherapeutic agents reported side effects, including myelosuppression, peripheral edema, and fluid retention. With further use of these agents, studies in the late 1980s and early 1990s began to report peripheral neuropathy and proximal muscle weakness as common complaints, the later with unexplained pathophysiology. We report a 65-year-old Hispanic woman with estrogen receptor (ER) and progesterone receptor (PR) positive invasive ductal breast carcinoma who presented with right thigh pain and swelling eight days after her third infusion of docetaxel (a taxane chemotherapeutic) and cyclophosphamide. Laboratory findings were notable for elevation in creatine phosphokinase (CPK), aldolase, and erythrocyte sedimentation rate (ESR); a magnetic resonance imaging (MRI) of her lower extremities showed evidence of bilateral muscle edema involving the anterior compartment muscles of the thighs. A workup to rule out other causes of myositis was negative. Docetaxel was not reintroduced and the patient improved with corticosteroids. Since 2005 this is, to our knowledge, the fifth reported case of docetaxel related inflammatory myositis. Taxanes have been noted to cause disabling but transient arthralgias and myalgias; it is important to consider the possibility of inflammatory myopathy as a possible complication in patients undergoing treatment with these agents. Alexandra Perel-Winkler, Regina Belokovskaya, Isabelle Amigues, Melissa Larusso, and Nazia Hussain Copyright © 2015 Alexandra Perel-Winkler et al. All rights reserved. Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis Thu, 09 Jul 2015 08:19:30 +0000 http://www.hindawi.com/journals/crirh/2015/107275/ According to the American Thoracic Society (ATS)/European Respiratory Society consensus classification, idiopathic interstitial pneumonias (IIPs) include several clinic-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF), usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated ILD, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. Ultrasound Lung Comets (ULCs) are an echographic chest-sonography hallmark of pulmonary interstitial fibrosis. We describe the ultrasound (US) findings in the follow-up of a NSIP’s case in rheumatoid arthritis (RA). A. Laria, A. Lurati, and M. Scarpellini Copyright © 2015 A. Laria et al. 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