Kimura's Disease: A Literature Review Based on a Clinical Case.

Kimura's disease (KD) was first described in 1937. It is a rare, benign, and chronic immune-mediated inflammatory disorder affecting the subcutaneous tissue, salivary glands, and lymph nodes. The disease is more common in the second to third decades of life in middle-aged Southeast Asian countries. The cause of Kimura's disease remains unknown; some authors believe it is related to an autoimmune or delayed-type hypersensitivity reaction. It commonly presents as a solitary painless lymph node in the head and neck or generalized lymphadenopathy (67%-100%) associated with peripheral eosinophilia and elevated IgE levels. Renal involvement may occur in some patients. Diagnosis is made by histology. A 21-year-old Caucasian man with no relevant medical history presented with a non-tender swelling of the left hemiface without other associated symptoms. Laboratory investigations revealed a leukocyte count with eosinophilia (2.29×10^9/L- 26.5%) and elevated total IgE and IgG4. He had no renal dysfunction. He underwent surgical resection of the lesion in the jugal, infraorbital, and left nasolabial regions, and the anatomopathological examination revealed the characteristics of Kimura's disease. Three months after surgery, an MRI showed an increase in the size of the mass, and he was started on corticosteroids. Six months after surgery, he presented with a slight increase in the size of the mass and was started on Ciclosporin, which allowed a progressive reduction in the dose of corticosteroid with evidence of a progressive reduction in swelling. Ciclosporin was stopped due to toxic serum levels, and he was started on mycophenolate mofetil. The dose was increased because of the increase in facial mass; on mycophenolate mofetil 2500mg/day, the patient remains stable. KD is a chronic disorder of unknown etiology that mainly affects young people in Southeast Asia and is rare in Western countries, as in the case of this young man. Studies have shown no significant differences in region or race, complications, multiplicity, laterality, anatomical distribution, maximum size, eosinophil count, or IgE levels between age groups. There is no consensus on the optimal treatment for KD; several treatments have been used, including surgery, systemic corticosteroids, immunosuppressants, and radiation. Due to the tendency to relapse and the rarity of the disease, there is no consensus on treatment options for relapse.

Methotrexate Pneumonitis After a Low-Dose Medication Error: A Case Report.

Methotrexate is recommended as the first choice of standard drug therapy following the diagnosis of rheumatoid arthritis. Pneumonitis related to methotrexate is a serious, unpredictable adverse event that may become life-threatening. We reported a case of a 68-year-old woman with rheumatoid arthritis that misunderstood the directions for use and took methotrexate daily, instead of weekly, leading to hepatic, hematological, and pulmonary toxicity.Although the histological evaluation was not performed, patient's clinical presentation, in addition to subsequent investigational findings, supported a diagnosis of pneumonitis resulting from MTX exposure. Toxic dosing over a long period of time along with the concomitant taking of pantoprazole and hypoalbuminemia could have increased the incidence of some adverse events. Concerning pneumonitis related to methotrexate, the toxic dose may have accelerated the pulmonary manifestations, but we do not know if correct dose had been taken, this adverse event would occur. This case enlightened two important issues in rheumatoid arthritis treatment: the possibility of medication errors and the rare, but potentially life-threatening, methotrexate-induced pneumonitis. Improving education and warnings when prescribing and dispensing low-dose methotrexate is essential.

Granulomatosis with polyangiitis: An atypical initial presentation.

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small and medium vessels with involvement of the upper and lower respiratory tract and necrotizing pauci-immune glomerulonephritis [1]. This vasculitis has a higher incidence in men in the sixth decade of life and more than 80% of patients have positive anti-neutrophil cytoplasm (ANCA) antibodies [1,2]. We present the case of a 23-year-old man with two weeks of evolution with polyarthralgia, asthenia, and cough with hemoptoic sputum. He did a chest radiography that showed diffuse bilateral alveolar infiltrates, on the second stage. The patient presented a rapid clinical worsening, with moderate hemoptysis and severe respiratory failure requiring invasive mechanical ventilation. The autoimmune study revealed positivity for ANCA PR3 in titer >200, having started pulses of methylprednisolone, plasmapheresis and later cyclophosphamide, with clinical improvement. His high-resolution chest computed tomography (CT) showed areas of diffuse ground glass densification suggesting capillaritis/alveolar hemorrhage and two subpleural nodular areas suggestive of granulomatous vasculitis. CT of the nasal sinuses showing findings compatible with acute inflammatory changes, with histology of the nasal mucosa inconclusive. Thus, this case shows an exuberant and potentially fatal form of diffuse alveolar hemorrhage that culminated in the initial diagnosis of granulomatous vasculitis in a young adult.

Simultaneous Systemic Lupus Erythematosus Flare and Disseminated Tuberculosis: Balancing Anti-Mycobacterial and Autoimmune Treatments.

Here, we report the case of a 53-year-old man with suspected autoimmune arthritis on low-dose corticosteroid therapy. He was recently hospitalized due to presumed bacterial pneumonia and a seizure episode attributed to high fever. His condition deteriorated after discharge, and he presented to our institution with a persistent cough, weight loss, skin rash, arthralgias, fever, and altered mental status. The investigation led to the simultaneous diagnosis of a systemic lupus erythematosus (SLE) flare and disseminated tuberculosis (TB), both pulmonary and intracranial. Proteinuria and peripheral edema were identified, suggesting renal involvement of SLE. Anti-mycobacterial drugs and high-dose corticosteroid therapy were initiated. Given the risk of starting other immunosuppressive drugs in the presence of intracranial TB, in a patient with stable renal function and a significant decrease in proteinuria with corticosteroids and supportive therapy alone, renal biopsy was postponed. Prednisolone was progressively tapered down during the next six months, always maintaining anti-mycobacterial therapy, which resulted in a second SLE flare and the need to increase corticosteroids again. At this time, a renal biopsy was performed, showing class II lupus nephritis and confirming the diagnosis of SLE. After one year of anti-mycobacterial therapy with complete resolution of cerebral and pulmonary TB lesions, we chose to initiate mycophenolate mofetil as an immunosuppressive steroid-sparing agent with increased SLE control, allowing for corticosteroid reduction.

Report of Two Cases of Acquired Idiopathic Haemophilia.

Acquired haemophilia is a rare haemorrhagic dyscrasia caused by autoantibodies against coagulation factors, most commonly factor VIII (FVIII). Even though about half of the cases are classified as idiopathic, acquired haemophilia is more common in the elderly and/or in individuals diagnosed with other immunogenic conditions such as malignancies, autoimmune diseases, or during puerperium. It can be life-threatening, presenting more frequently with major bleeding. We report two cases of acquired haemophilia classified as idiopathic in middle-aged patients with no predisposing factors identified during the diagnostic approach: their disease's progression and complications, choice of treatment, and why and when to change it.

Efficacy of Rituximab in Refractory Inflammatory Myopathy Associated With Coexistence of Behçet's Disease and Antiphospholipid Syndrome.

A 43-year-old Caucasian male initiated myalgias and loss of muscle strength in the upper and lower limbs, but especially at the shoulder and pelvic girdle. Creatinine phosphokinase was elevated seven-fold above the normal reference value and aldolase was slightly elevated. He had a previous diagnosis of Behçet's disease, antiphospholipid syndrome and hypertriglyceridaemia. At this time, he was on azathioprine 150 mg daily, colchicine 1 mg daily, warfarin and fenofibrate 200 mg daily. Fenofibrate was stopped and creatinine phosphokinase re-evaluated 2 months later, but it was higher, with persistent myalgias. By this time, prednisolone was restarted and the azathioprine dose reduced until it was discontinued. Nevertheless, 2 months after stopping azathioprine, the patient remained symptomatic and creatinine phosphokinase was persistently elevated. At this point, the authors requested myositis antibody testing to exclude overlap with a third autoimmune disorder, and Ro52 antibody was positive. Electromyography was normal. Magnetic resonance imaging of lower limb muscles was compatible with polymyositis. Muscular biopsy of the medial gastrocnemius revealed inflammatory myopathy. The authors proposed treatment with rituximab and after 3 months, the patient had clinically and analytically improved, with reduction of creatinine phosphokinase, without adverse reactions. As we can see in this case, rituximab could be a secure treatment for patients with idiopathic inflammatory myopathy without improvement on glucocorticoids plus another immunosuppressive agent. This patient has a rare overlap syndrome, since this is the first case of an association between inflammatory myopathy, Behçet's disease and antiphospholipid syndrome described in the literature. LEARNING POINTS: This is the first case report in the literature of an association between inflammatory myopathy, Behçet's disease and antiphospholipid syndrome.Rituximab could be a secure treatment for refractory idiopathic inflammatory myopathy.This case report highlights the importance of a methodical diagnostic work-up for an accurate diagnosis.

Azathioprine-induced interstitial nephritis in an anti-neutrophil cytoplasmic antibody (ANCA) myeloperoxidase (MPO) vasculitis patient.

Azathioprine (AZA) is used in a wide array of autoimmune diseases, still corresponding to the mainstay maintenance therapy in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although generally well-tolerated, several side effects are recognized. We report the case of a 50-year-old Caucasian man with kidney-limited ANCA myeloperoxidase (MPO) vasculitis who presented with general malaise, fever, worsening renal function, and elevated inflammatory markers 2 weeks after the initiation of therapy with oral AZA. Although a disease relapse was suspected, renal biopsy revealed an eosinophilic infiltrate, suggestive of acute interstitial nephritis. After suspension of AZA, a sustained improvement of renal function and normalization of inflammatory markers was observed. A diagnosis of allergic interstitial nephritis secondary to AZA was established, corresponding to the first biopsy-proven case described in an ANCA MPO vasculitis patient. Although rare, renal toxicity of AZA must be present in the clinician's mind, avoiding the straightforward assumption of disease relapse in the case of worsening renal function.

Plasmodium spp. and Borrelia burgdorferi co-infection associated with antiphospholipid syndrome in a returned traveler: a case report.

The differential diagnosis of fever in a returned traveler is wide and challenging. We present a case of a patient working in Africa, who returned with fever, constitutional symptoms, headache, and blurred vision. An initial diagnosis of malaria was made, and additional workup revealed Borrelia burgdorferi co-infection and antiphospholipid syndrome.