VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined disorder in which treatment is still unclear. Herein, a patient with VEXAS syndrome who had atypical findings and an interesting treatment course is presented as a case report. He had fatigue, recurrent fever, pulmonary infiltrates, proteinuria, anemia, leucopenia, transient skin rush and increased acute phase reactants.The patient, who could not tolerate corticosteroid tapering, recovered rapidly after diagnostic splenectomy and the pathological examination of the spleen revealed significant findings.
Cardiac involvement (CI) is rare in Behçet syndrome (BS), but the important point is that CI may be the first manifestation of the disease. The presence of CI worsens the prognosis of BS, so early diagnosis and early initiation of immunosuppressive treatment (IST) are vital. Coronary aneurysm may develop spontaneously in these patients, or any vascular intervention may cause aneurysm with a pathergy‐like reaction. The risk of restenosis is high after percutaneous coronary intervention or coronary artery bypass surgery applied without IST. Therefore, it should be kept in mind that IST constitutes the main step of treatment. Herein, we present a young male diagnosed with BS after acute coronary syndrome caused by coronary artery aneurysms and thrombosis.
Patients with systemic lupus erythemasus (SLE) have an increased risk of bacterial, viral, fungal or parasitic infections, especially if they are receiving immunosuppressive therapy. Leishmaniasis is a group of diseases caused by intracellular flagellate protozoan parasites belonging to the genus Leishmania. We present a 48‐year‐old female patient, diagnosed with SLE many years ago, who presented with high fever and pancytopenia. We thought that the patient's hematologic findings were related to SLE hematologic involvement. However, we investigated other possible causes when there was no response to drugs for the treatment of SLE. A second bone marrow biopsy showed Leishmania amastigotes and the patient was diagnosed with leishmaniasis. The patient was treated with liposomal amphotericin‐B (treatment completed at 40 days). She showed rapid clinical improvement and showed no signs of disease after 4 months.
Palmoplantar pustular psoriasis (PPP) is a rare clinical form of psoriasis. It is usually seen on the palms and soles, and affects patients' quality of life. In most cases, topical or systemic treatments are not sufficiently effective, so management of PPP is generally difficult. Hydroxychloroquine (HQ) is an antimalarial drug that is widely used in many autoimmune rheumatic diseases, mainly in systemic lupus erythematosus (SLE).Several reports describe the induction and exacerbation of psoriasis by HQ. Within this report, we aimed to put emphasis on considering possible drug effects by presenting a case of PPP, induced by HQ.
Behcet’s syndrome is a variable vessel vasculitis characterized by recurrent oral and genital ulcers with concomitant skin, ocular, neurologic, gastrointestinal, and joint involvement. Herein, we present a patient who was diagnosed with Behcet’s syndrome, which with magnetic resonance angiography showed occlusion of the right subclavian artery at the level of the thoracic outlet and reverse flow in the right vertebral artery consistent with subclavian steal syndrome. In addition, partial narrowing was noted in the left renal artery. The distribution of arterial involvement resembled Takayasu’s arteritis, although the presence of mucocutaneous lesions, male gender, history of deep vein thrombosis, and HLA-B51 positivity favored a diagnosis of vasculo-Behçet’s syndrome. We treated the patient with methylprednisolone and cyclophosphamide. After the regression of vascular inflammation with immunosuppressive therapy, stenting was performed in the left renal artery.
Vasculitis is the inflammatory changes in vessels of any size that usually have a systemic involvement with a quite variable clinical presentation affecting various organs. Although systemic presentation is more common, in some cases localized inflammation of vasculature of a single organ or limited branches of aorta are reported. Here we present, an isolated vasculitis of bilateral inferior thyroidal arteries in a female patient aged 49 years, who presented with neck pain and was diagnosed with ultrasonography and computed tomographic angiography. The clinical and imaging findings were managed successfully with glucocorticoid induction and addition of methotrexate to the treatment. Localized forms of vasculitis are rarer and the limited size of the affected area makes diagnostic investigations and management more complicated. Non‐invasive imaging modalities rather than conventional angiography provide useful information in a safer and easier way. Isolated vasculitis of thyroidal arteries is an extremely uncommon site and should be excluded in case of unexplained neck pain, even in the presence of normal laboratory examinations, probably because of the size of the involved vessels.
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