Case Reports in Rheumatology最新文献

COPA syndrome is a very rare autoinflammatory disorder manifesting with childhood-onset arthritis and pulmonary and renal disease, of which awareness may remain lacking. We present the case of a twenty-year-old male patient seen in the Young Adults with Rheumatic Disease clinic. Initially diagnosed with seropositive polyarticular juvenile idiopathic arthritis, the patient's early childhood complaints of fatiguability, paroxysmal dyspnea, and pneumonia-like episodes were long to be felt unrelated to his arthritis. Upon transition to adult rheumatology care, a thorough review of the patient's history prompted imaging which revealed interstitial lung disease. Restrictive spirometry and genetic testing confirmed the retrospective diagnosis of COPA syndrome.

Objectives: Illustration of a case of systemic mastocytosis mimicking reactive arthritis in the absence of an infectious etiology.

Methods: Review of the patient's medical records.

Results: We report a case of systemic mastocytosis relapse, presenting with pancytopenia accompanied by knee monoarthritis, cystitis, and bilateral conjunctivitis occurring simultaneously at the same time interval within 2-4 days, mimicking reactive arthritis in the absence of an infectious etiology.

Conclusion: Our case demonstrated reactive arthritis features (triad of urethritis, conjunctivitis, and arthritis) without an infectious trigger but rather a relapse of mastocytosis. We should think outside the box when faced with such a clinical scenario in the absence of an infectious etiology. Paraneoplastic reactive arthritis is to be considered after excluding an underlying infection.

Minocycline, a tetracycline antibiotic, is commonly used to treat rosacea and acne vulgaris. A rare adverse reaction of minocycline use is the development of drug-induced lupus. Fortunately, most patients recover from minocycline-induced lupus (MIL) after the drug is discontinued. However, many patients, after recovering from MIL, may desire further treatment for their acne and may consider doxycycline, a close relative of minocycline. Though no cases of doxycycline-induced lupus have been reported, there is little guidance in the medical literature as to whether doxycycline poses a particular risk to patients who have recovered from MIL. We report the long-term follow-up of a patient who recovered from MIL (the diagnosis satisfying clinical and laboratory criteria) and was treated for 8 years with various forms of doxycycline without any untoward effects, suggesting that, at least in some cases, doxycycline can be used safely following MIL.

IgA vasculitis is a common type of vasculitis that is generally triggered by infectious causes. Vaccines have been reported as a trigger as well. Herein, we report a case of a young man who is previously healthy and who developed IgA vasculitis after the first dose of the COVID-19 mRNA vaccine Pfizer-BioNTech. The patient's symptoms were mainly skin and joint without renal or other system involvement. The patient had an excellent outcome with complete resolution after treatment with steroid tapering and azathioprine as a steroid-sparing agent over 6 months.

Takayasu arteritis (TAK) is a rare large-vessel vasculitis that is seen primarily in young females of Asian descent and is infrequently diagnosed in the United States. Pericardial effusion with or without pericarditis as a presenting feature of TAK is rare, with only about five percent of cases of pericarditis attributable to any autoimmune etiology. We present a case of a 22-year-old Caucasian woman who presented with a large, symptomatic pericardial effusion of unclear etiology, who after extensive laboratory workup and imaging to include whole-body positron emission tomography (PET) was diagnosed with TAK. In our patient, the use of whole-body PET showing characteristic hypermetabolism within the aortic arch helped secure our diagnosis while avoiding the need for pericardiocentesis. The patient had rapid symptomatic and radiographic improvement with the use of high-dose oral steroids in addition to colchicine and ibuprofen for her pericarditis and associated pericardial effusion. At follow-up just 1 week after initiation of steroids, only trace effusion was identified on transthoracic echocardiogram.

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a systemic vasculitis characterized by ANCA positivity and categorized into three main types: microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatous with polyangiitis. Although AAV leads to systemic organ injury, such as of the lungs, kidneys, nerves, and skin, patients with AAV sometimes develop ocular lesions. Here, we report the case of an elderly woman who had been treated for AAV for seven years. She developed scleritis and relapsed twice, with elevation of serum disease markers such as ANCA titer and C-reactive protein. After the decline of these markers due to treatment with additional medication, her scleritis relapsed again and caused a corneal ulcer, which resulted in perforation without obvious marker elevation. She did not present with any symptoms of organ injury, except for ocular lesions. She was treated with surgery, followed by methylprednisolone and rituximab therapy. Subsequently, her ocular lesions and symptoms improved, and she did not relapse. AAV can cause various ocular manifestations. Although C-reactive protein and ANCA titers are useful markers of disease activity and the relapse of AAV complications, including ocular lesions, these markers do not always increase at the time of worsening ocular lesions. Therefore, it is important for clinicians treating patients with AAV to pay careful attention to serum data and physical findings, including the eyes.

Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a rare disease with an unknown entity that affects the skin and the peripheral and/or axial joints. Here, we report on a patient with SAPHO syndrome complicated by lesions of the central nervous system who was successfully treated with brodalumab, an IL-17 receptor blocker. He had been suffering from arthralgia in the wrists and knees as well as axial symptoms such as back pain and assimilation of cervical vertebrae. He had been treated with corticosteroid, salazosulfapyridine, methotrexate, and bisphosphonate; however, his peripheral and axial articular manifestation were intractable. Recently, biologics predominantly targeting TNF-α is employed for difficult-to-treat SAPHO cases; however, he had been complicated with the lesions of the central nervous system resembling multiple sclerosis (MS), an inflammatory demyelinating disorder in the central nervous system, for which application of TNF-α inhibitor is contraindicated. Alternatively, brodalumab was administered , which promptly ameliorated the articular manifestations without aggravating the lesions of the central nervous system. We propose that this type of IL-17 blockade could be an alternative therapy for DMARDs-resistant SAPHO syndrome.

Introduction: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare autoinflammatory condition describing the constellation of inflammatory skin, bone, and joint manifestations which result in diagnostic difficulty and therapeutic challenge.

Case: Here, we present a case of a young male diagnosed with SAPHO syndrome with osteoarticular and cutaneous involvement from an early age in his life. He suffered diagnostic challenges for a long time and was hence inadequately treated. He had minimal response to conventional DMARDs but showed excellent response to TNF inhibitor (adalimumab). Later, he defaulted treatment and presented with acute anterior uveitis which was also dramatically improved with adalimumab and tofacitinib although financial constraint was always an issue for the patient.

Conclusion: The uniqueness of this case was that the patient had a multiorgan involvement including osteoarticular system, skin, and eye. Both TNFi (adalimumab) and JAKinib (tofacitinib) had a good response to all organs with a net improvement in the quality of life of this patient.

We report a patient with catastrophic antiphospholipid syndrome who had significant improvement after corticosteroids, plasmapheresis, argatroban, rituximab, and sirolimus. Argatroban was used instead of heparin due to a history of heparin-induced thrombocytopenia.

Methotrexate, an anchor drug for rheumatoid arthritis, hinders the immunogenicity of mRNA COVID-19 vaccines. Therefore, an optimal vaccine strategy for patients with rheumatoid arthritis receiving methotrexate is vital. We monitored antispike antibody titers after BNT162b2 mRNA COVID-19 vaccination in seven healthcare workers and one methotrexate-treated rheumatoid arthritis patient. The antispike antibody titers of healthcare workers significantly increased immediately after primary vaccination and then continued to decrease, whereas those of the rheumatoid arthritis patient were significantly lower immediately after primary vaccination and then increased. The titers in all participants dramatically increased 1-month postbooster. These changes over time may suggest that in the methotrexate-treated rheumatoid arthritis patient, the generation of short-lived plasma cells was strongly suppressed; in contrast, the generation of long-lived plasma cells and memory B cells was intact. For methotrexate-treated rheumatoid arthritis patients, it is important to complete the primary and booster vaccination series to ensure sufficient immunity against COVID-19.