Fifty-Two-Week Results of Clinical and Imaging Assessments of a Patient with Rheumatoid Arthritis Complicated by Systemic Sclerosis with Interstitial Pneumonia and Type 1 Diabetes despite Multiple Disease-Modifying Antirheumatic Drug Therapy That Was Successfully Treated with Baricitinib: A Novel Case ReportRead the full article
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Lupus Never Fails to Deceive US: A Case of Rowell’s Syndrome
Background. Rowell’s syndrome is comprised of the presentation of erythema multiforme- (EM-) like lesions in association with lupus erythematosus (LE), along with serologies of speckled antinuclear antibodies (ANAs), positive rheumatoid factor (RF), positive anti-La/anti-Ro, and the clinical finding of chilblains. As per the redefined criteria by Zeitouni et al., three major criteria in addition to at least 1 minor criterion are necessary for diagnosis. Case Presentation. A 20-year-old male presented with a one-week history of worsening nonpruritic erythematous maculopapular skin rash (resembling EM) which appeared on the face and subsequently spread to the trunk, arms, legs, palms, and soles. There was no mucosal involvement. At the onset of rash, the patient reported headaches, associated with photosensitivity and intermittent fevers. Workup for viral meningitis yielded negative results. Laboratory investigation revealed mild anemia, elevated inflammatory markers, a positive ANA with speckled pattern, a positive anti-Ro/SSA, anti-La/SSB antibodies, and a positive rheumatoid factor (RF). Lupus anticoagulant antibody was positive along with a low-positive anticardiolipin IgM antibody and a negative beta-2 glycoprotein antibody. Anti-dsDNA, anti-Smith, anti-Jo-1, anti-centromere, and anti-Scl-70 antibodies were negative. Hepatitis serologies, herpes simplex virus 1 and 2, mycoplasma, Epstein–Barr virus, HIV, and parvovirus B19 were negative. Left arm skin biopsy demonstrated vacuolar interface dermatitis and positive colloidal iron stain suggestive of dermal mucin deposition, favoring the diagnosis of cutaneous collagen vascular disease. Cutaneous lesions improved with administration of oral prednisolone. Conclusion. Rowell’s syndrome should be considered in patients who present with cutaneous LE and lesions resembling EM. Further serological markers should be pursued in the absence of obvious EM-precipitating factors.
Primary Sjögren’s Syndrome Accompanied by Clinical Features of TAFRO Syndrome
Sjögren’s syndrome (SS) is associated with not only sicca symptoms but also various symptoms caused by extraglandular manifestation. The pathophysiology and comorbidities of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly), which is thought to be a variant of multicentric Castleman’s disease, are not fully understood, and there are few data on the effectiveness of treatments. We report a patient of SS with TAFRO syndrome-like clinical features. A 52-year-old woman was admitted to our hospital because of abdominal distension. Laboratory data showed thrombocytopenia, and image findings showed massive ascites without evidence of malignant disease as confirmed by cytology. She was diagnosed with SS based on dysfunction of salivary secretion and positivity for anti-Ro/SS-A and La/SS-B antibodies, accompanied by clinical features of TAFRO syndrome based on the presence of anasarca and thrombocytopenia. High-dose corticosteroid for inflammation, anasarca, and thrombocytopenia was not effective. Cyclosporine was administered next, but anasarca and thrombocytopenia did not immediately improve until tolvaptan and eltrombopag were added. Although tolvaptan and eltrombopag were used for only a few months, the patient maintained a good condition with cyclosporine and low-dose prednisolone. In SS patients, activation of antigen-specific T lymphocytes is thought to be an important trigger that accelerates the immune response and is followed by hypercytokinemia. Therefore, using cyclosporine to suppress the activity of T lymphocytes is a reasonable treatment for SS accompanied with TAFRO syndrome-like pathophysiology. It might also be useful to administer tolvaptan or eltrombopag before the effects of immunosuppressants appear. If refractory inflammation with anasarca, thrombocytopenia, or lymphadenopathy is observed in an SS patient, complications with TAFRO syndrome-like pathophysiology should be considered.
Development of Arthritis as the Initial Involvement in Adult-Onset Cutaneous Polyarteritis Nodosa: Two Cases and Literature Review
Articular symptoms are commonly present in polyarteritis nodosa (PAN). Meanwhile, they may occur as the initial and main involvement of PAN, raising a concern of a delay in a definitive diagnosis of disease unless the histological evidence is obtained. Herein, we report two cases of cutaneous PAN (c-PAN) in which arthritis developed as the initial clinical episode of disease and we argued, through a review of the literature, the clinical feature of patients presenting with arthritis as the initial symptom of PAN. To our knowledge, only six cases have been reported in the English literature, and all six patients were categorized as having c-PAN. Of those patients, four had arthritis with indicating destructive changes. A median of 9 years elapsed prior to the induction of immunosuppressive treatment despite the fact that the other reviewed cases as well as our two patients, who received treatment significantly earlier, showed no evidence of joint destruction. Taken together, this report suggests that the early induction of therapy based on the definitive diagnosis of PAN may be required for preventing joint disruption even though it is not easy to make a diagnosis of PAN unless biopsied tissue can provide the evidence of necrotizing vasculitis.
Rapidly Progressive Pulmonary Apical Fibrosis and Parenchymal Destruction in a Patient with Ankylosing Spondylitis
Pulmonary apical fibrosis is a rare complication of ankylosing spondylitis (AS). The essential characteristics of this lesion are its very slow progression and frequently asymptomatic nature. Herein, we are presenting a patient with AS who rapidly developed pulmonary apical fibrosis in a 3-year period despite decreased musculoskeletal pains. The 60-year-old male applied with complaints of progressively increasing cough in the recent two years, dyspnea, and fatigue. He had no chronic disease except AS. He had no continuous medication except nonsteroid anti-inflammatory drugs for 2-3 days monthly since his musculoskeletal pains decreased in the recent years. His physical examination revealed reduced breath sounds in the upper zones of the right lung. Chest X-ray revealed increased diffuse opacity in the upper zones of the right lung. Thoracic high-resolution computed tomography showed a consolidation accompanied with traction bronchiectases compatible with chronic fibrosis in the upper lobe of the right lung. However, thoracic computed tomography of the patient performed 3 years ago did not reveal pulmonary apical fibrosis and parenchymal destruction. Biopsy revealed no finding of malignancy, granulomatous inflammation, or vasculitis. The results of cultures were negative. So, the patient was diagnosed as pulmonary involvement of AS, which developed in a 3-year period. This case has shown that extra-articular complications may continue to develop in patients with AS even if their musculoskeletal complaints have subsided. So, patients with AS should be followed up regularly with systemic examinations.
Acetabula Osteoid Osteoma Mimicking Juvenile Idiopathic Arthritis and Chronic Recurrent Multifocal Osteomyelitis
Osteoid osteoma (OO) is a benign bone tumor that usually presents between 10 and 35 years of age. The metaphysis and diaphysis of the femur and tibia are the typical locations. The diagnosis is usually straightforward when images reveal a radiolucent nidus surrounded by reactive sclerosis. However, the diagnosis is more difficult when it occurs at atypical locations with nonspecific and misleading appearance on images. OO may mimic juvenile idiopathic arthritis (JIA), bone infection, or malignancy. We present a 14-year-old male with a 4-month history of left hip pain. His pain was worse with playing hockey and lacrosse and in the morning and sometimes woke him up at night. His examination was significant for pain with flexion and external rotation of the left hip and for mild limitation of full external rotation. Blood work revealed normal complete blood count, erythrocyte sedimentation rate, and C-reactive protein. Left hip X-ray was unremarkable. Left hip MR arthrogram showed marked edema of the medial and posterior walls of the left acetabulum. CT-guided biopsy of the left acetabulum showed unremarkable flow cytometry and chronic inflammatory component raising concern about chronic recurrent multifocal osteomyelitis (CRMO). Bone scan revealed focal increased uptake in the left acetabulum and no additional abnormality. Repeat MRI with intravenous contrast showed a left hip effusion, focal synovial enhancement in the medial left hip, and acetabula edema. The patient failed treatment for presumed JIA and CRMO with nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, methotrexate, and adalimumab. CT scan of the left hip was performed for further evaluation of the bone and showed 11 × 6 mm low attenuation focus with subtle internal nidus in the posteromedial aspect of the acetabular rim, suggestive of intra-articular OO. Radiofrequency ablation was performed with no complications, and the left hip pain improved. The atypical location resulted in delay of diagnosis for 12 months after presentation. We highlight the diagnostic pitfalls observed in atypical OO locations and the difficulties this creates with making the diagnosis. OO mimicking JIA has previously been described. We submit CRMO as another differential diagnosis which may be mimicked and demonstrate the vital role of CT scan in the diagnosis.
Doxycycline-Induced Antinuclear Antibody and Antineutrophil Cytoplasmic Antibody Associated Vasculitis: A Case Report and Literature Review
Drug-induced antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been increasingly recognized in the literature with numerous medications listed as causative agents in disease pathology. Doxycycline is a commonly prescribed medication within the United States which is a synthetic, broad-spectrum antibiotic with antimicrobial properties and at low doses exhibits anti-inflammatory effects. In this report, we describe a case of doxycycline-induced ANCA-associated vasculitis with laboratory and biopsy findings supporting the diagnosis, which to the best of our knowledge is the first described case of doxycycline-induced AAV in the literature. The patient was started on doxycycline for treatment of potential Lyme disease. She began to develop progressively worsening myasthenia, erythematous macular rash, anorexia, anemia, and fatigue for several weeks following the course of doxycycline with initial concern of a paraneoplastic process. Ultimately, the patient was discovered to be positive for antinuclear antibody (ANA), perinuclear antineutrophil cytoplasmic antibody (pANCA), and myeloperoxidase (MPO) antibody for which she was treated with a course of prednisone leading to complete remission of disease. A brief review of the pathogenesis of ANCA vasculitides will also be discussed within this article.