Case Reports in Rheumatology最新文献

A 37-year-old Bangladeshi woman presented with low back and several joints pain and swelling for months together; there was significant morning stiffness for more than two hours. Repeated abortions, dry eye, hair fall, photosensitivity, and oral ulcer were the additional complaints. Clinical examination unveiled asymmetrical peripheral and both sacroiliac joint tenderness, positive modified Schober's test, and limited chest expansion. Schirmer's test was positive. The history of rheumatoid arthritis (RA) and ankylosing spondylitis (AS) among 1st-degree relatives was also significant. Biochemical analysis revealed pancytopenia, raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and mild microscopic proteinuria. The patient was seropositive for rheumatoid factor (RF), antibodies against cyclic citrullinated peptides (anti-CCP), antinuclear antibody (ANA), anti-Sm antibody, anti-Sjögren's-syndrome-related antigen A and B (anti-SSA/SSB), antiphospholipid (aPL-IgG/IgM), and HLA B27; however, serum complement (C3 and C4) levels were normal. Basal cortisol level measured elevated. Besides, X-ray and MRI of lumbosacral spines demonstrated sacroiliitis. There was radiological cardiomegaly, echocardiography unveiled atrial regurgitation, and ascending aorta aneurysm. Based on the abovementioned information, RA, AS, and systemic lupus erythematosus (SLE) have been diagnosed. Moreover, the patient developed Sjogren's syndrome (SS), antiphospholipid lipid syndrome (APS), Cushing syndrome, ascending aorta aneurysm, and atrial regurgitation. Her disease activity score for RA (DAS28), DAS for AS (ASDAS), SLE disease activity index (SLEDAI), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) scores were 3.46, 2.36, 23, and 5, respectively. The patient received hydroxychloroquine (200 mg daily), pulsed cyclophosphamide, prednisolone (20 mg in the morning), and naproxen 500 mg (twice daily). To our best knowledge, this is the first report documenting RA, AS, and SLE with secondary SS and APS.

Granulomatosis with polyangiitis (formerly called Wegener's granulomatosis) is a systemic autoimmune disease, which can lead to necrotizing vasculitis affecting small vessels and cause inflammation of blood vessels in the nose, sinuses, throat, lungs, and kidneys. In rare instances, it has shown involvement of the brain and cranial nerves as well. We are reporting a case of granulomatosis with polyangiitis, complicated by bilateral facial palsy due to lower motor neuron involvement of the facial nerve, which has responded well to immunosuppressive treatment, particularly rituximab. It is prudent to be vigilant in investigating patients with atypical presentation for systemic autoimmune diseases, as this approach would affect the patient morbidity and mortality with early initiation of treatment for the disease.

Primary Sjögren syndrome (SS) is a chronic inflammatory systemic autoimmune disease with a high risk of malignancy development, namely, lymphoproliferative neoplasms. Few studies also reported a high risk of solid cancers; however, the coexistence of primary SS and pancreatic cancer has been rarely described. In this paper, we aim to describe a case of a 59-year-old woman who was an active smoker with sicca symptoms and symmetrical polyarthritis and was diagnosed with primary SS two years before the development of metastatic pancreatic adenocarcinoma. Despite institution of chemotherapy, the patient succumbed to the malignancy. Besides that, we explore the link between primary SS and solid cancers including the main predictors of malignancy and the role of primary SS as a paraneoplastic syndrome. Patients with primary SS should be closely monitored for malignancy, not only for hematological cancer, but also for solid tumors. Further research is necessary to understand which are the predictors of cancer proliferation in primary SS patients.

Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven-Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.

Systemic lupus erythematosus is a systemic autoimmune disease, with presentations that vary within a population and across the lifespan of an individual. The disease afflicts childbearing women more than men and uncommonly presents in the geriatric population. Lupus pneumonitis is rare, with a reported incidence of 1-4%. Herein, we discuss the case report of an elderly gentleman with biopsy-proven acute lupus pneumonitis (ALP) as an initial presentation of lupus. After starting high-dose steroids, the patient initially improved, though unfortunately endured a non-ST elevation myocardial infarction and recurrent gastrointestinal bleeding. Despite multiple interventions and a prolonged hospital course, his gastrointestinal bleeding persisted. He elected to go on home hospice and ultimately passed away due to ongoing gastrointestinal bleeding. As with our patient, elderly patients can pose a diagnostic dilemma with regard to late-onset lupus; multiple comorbidities and growing evidence that late-onset lupus may manifest with distinct clinical patterns from younger cohorts complicate diagnosis in these patients. It is critical to maintain a broad differential, which includes unusual rheumatic manifestations when management of common comorbidities fails to alleviate symptoms for an elderly patient. Failure to do so may result in delayed diagnosis of rheumatic disease and increased side effects related to treatment. Additionally, this case serves as a reminder that due to the complexity of rheumatic disease and the additional challenge of older patients with baseline comorbidities, sometimes palliative care options may be appropriate.

The diagnosis of giant cell arteritis (GCA) when presenting with atypical features such as stroke is very challenging. Only 0.17% of first-ever strokes are caused by GCA, a life-threatening condition when left untreated. Very few cases have been reported on giant cell arteritis leading to acute stroke due to vertebral artery dissection. We present a case of a 76-year-old female with no medical history who presented with sudden onset right visual loss and left hemiparesis. She had been initially treated for acute stroke and upon further workup was found to have left vertebral artery dissection. She had erythrocyte sedimentation rate (ESR) of 71 mm/h, and bilateral temporal artery biopsy was consistent with giant cell arteritis. Patient received high doses of methylprednisolone which resolved her hemiparesis, but her vision loss did not improve. Stroke in the presence of significant involvement of vertebral arteries should raise suspicion of GCA especially if classic symptoms preceded stroke event. High clinical suspicion is required to prevent delay in diagnosis and treatment.

Introduction. Granulomatosis with polyangiitis (GPA) is a rare disease in pediatric age. We report two cases with distinct presentations. Case Reports. A seventeen-year-old male with prolonged febrile syndrome, cough, and constitutional symptoms. CT-scan showed cavitated lesions of the lung and bronchial biopsy a necrotizing inflammatory process. The remaining investigation revealed hematoproteinuria and positive C-ANCA and anti-PR3. Complications: Bilateral acute pulmonary thromboembolism, splenic infarction, and extensive popliteal and superficial femoral deep vein thrombosis. He was treated with corticosteroids, immunoglobulin, rituximab, and anticoagulation. Rituximab was maintained every six months during the first two years. Control angio-CT was performed with almost complete resolution of previous findings. In a twelve-year-old female with inflammatory signs of the limbs, investigation showed myositis of the thigh and tenosynovitis of the wrist, normocytic normochromic anemia (Hg 9.4 g/dL), mild elevation of inflammatory markers, and high creatine kinase. During hospitalization, she presented an extensive alveolar hemorrhage associated with severe anemia and positive C-ANCA and anti-PR3. Clinical deterioration prompted intravenous methylprednisolone pulses and plasmapheresis. Induction therapy with rituximab and prednisolone showed good results. Rituximab was maintained every six months, for 18 months, with gradual tapering of corticoids. Discussion. GPA is a systemic disease with variable clinical presentation and severity. Pediatric patients have similar clinical manifestations to adults but different frequencies of organ involvement; constitutional symptoms are also more common. We highlight the different presentation of these two cases, as well as the need for an individualized approach. Rituximab has been used for both induction-remission and maintenance therapy, with good results, particularly in young patients.

A 66-year-old man with a history of bronchial asthma and sinusitis was admitted with cholecystitis and peripheral neuropathy. The histopathological findings of the gallbladder revealed necrotic vasculitis and granulomatous inflammation with marked eosinophilic infiltration. Kidney biopsy also showed marked eosinophilic infiltration in the tubulointerstitial area and eosinophilic tubulitis. He was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) and treated with corticosteroids. However, he showed no response. Therefore, he was administered mepolizumab 300 mg, which resulted in clinical improvement, including normalization of the eosinophil and CRP levels. We herein describe the first case of successful induction therapy of EGPA using mepolizumab.

We present a case of a previously healthy adolescent female who developed severe oral mucositis and acute esophagitis as her presenting symptoms of juvenile systemic lupus erythematosus. Mucositis involving the lips is infrequently reported in systemic lupus erythematosus, and to our knowledge, this is the first reported case of acute, non-infectious esophagitis as a presenting symptom in a pediatric systemic lupus erythematosus patient.

Q fever is a rare zoonotic infection caused by Coxiella burnetii. Tumor necrosis factor-alpha (TNF-α) has an important role in the early control of this infection. However, TNF-α blockers increase the risk of infectious diseases. We present herein a patient who developed acute Q fever under anti-TNF-α who had a good evolution after anti-TNF stoppage and treatment with doxycycline.