Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with various clinical manifestations, including, rarely, a form of interstitial cystitis (lupus cystitis, LC). LC can be asymptomatic and usually has discrete symptoms that improve with conventional therapies available for SLE and/or typical interstitial cystitis. A very severe and refractory form is rarely described. In this study, we present four patients with SLE and a very severe form of noninfectious cystitis refractory to the different forms of treatment described. The clinical descriptions of the cases, demographic factors, manifestations associated with SLE, and clinical and paraclinical manifestations related to cystitis, treatments, and outcomes are provided. A proposal for the pathogenesis of this condition is based on the common findings of these patients, including the fact that three were in SLE remission and all four receiving rituximab as induction and/or maintenance therapy.

Vanessa Ocampo-Piraquive

In&#xe9;s Mondrag&#xf3;n-Lenis

Juan G. De los Rios

Carlos A. Ca&#xf1;as

Very Severe and Refractory Noninfectious Cystitis in Patients with Systemic Lupus Erythematosus: Potential Role of Rituximab Therapy

Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, mucositis, conjunctival changes, and unilateral cervical lymphadenopathy. The most feared complication of Kawasaki disease is development of coronary artery aneurysms. Common laboratory abnormalities include normocytic anemia, thrombocytosis, leukocytosis, and elevated inflammatory markers. Immune-mediated cytopenias such as autoimmune hemolytic anemia and thrombocytopenia are rarely seen at presentation in Kawasaki disease. We describe a unique case of a child presenting with autoimmune hemolytic anemia, who sequentially developed immune thrombocytopenia concerning for Evans&#x2019; syndrome and eventually diagnosed with Kawasaki Disease with coronary artery dilatation. Characteristic clinical findings including extremity edema, cracked lips, and rash developed later in the course. Our patient was treated with IVIG and steroids with significant clinical improvement and complete resolution of cytopenias and coronary aneurysms on long term follow up. Timely administration of IVIG prevents and minimizes the risk of long term cardiac consequences. Hence a high index of suspicion should be maintained for this relatively common pediatric illness, even in absence of more commonly seen laboratory findings.

Chloe Kupelian

Bindu Sathi

Deepika Singh

Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease

Myalgia and mild elevation in muscle enzymes are common side effects of statin therapy. While these symptoms are generally self-limited, in rare cases, statin use is associated with an immune-mediated necrotizing myopathy caused by development of autoantibodies against HMG-CoA reductase. The primary presenting symptom of this condition is progressive symmetric proximal weakness that does not abate or worsens even after cessation of statin therapy and is associated with markedly elevated creatine kinase (CK) levels. To date, no randomized controlled trials have been conducted to identify the most effective treatment for statin-associated autoimmune myopathy. Treatment recommendations involve a combination of steroids and immunosuppressive drugs. This single-center case series highlights the clinicopathologic features diagnostic for statin-associated autoimmune myopathy as well as treatment challenges for the patient population. The series highlights a range of potential presentations, from mildly symptomatic despite highly elevated CK, to severe muscle weakness including dysphagia. Multiple patients required several immunosuppressant medications as well as intravenous immunoglobulin (IVIG) to achieve disease control. In this case series, marked improvement was noted in several diabetic patients with IVIG.

Patrick Webster

Nicholas Wiemer

Abdalhamid Al Harash

Cody Marshall

Nazia Khatoon

Michael Lucke

Challenges in Treating Statin-Associated Necrotizing Myopathy

Drug-induced lupus is an iatrogenic-induced autoimmune disease with common offending agents well documented in the literature. To our knowledge, there are no prior case reports of drug-induced lupus associated with apixaban or any other direct oral anticoagulant. We describe a case of drug-induced lupus with leukocytoclastic vasculitis associated with apixaban started 15 days prior, after a WATCHMAN procedure for atrial fibrillation in an 86-year-old male previously anticoagulated on rivaroxaban.

John Yang

Steffi Lena

Daniel Tarditi

Gopika Banker

Drug-Induced Lupus with Leukocytoclastic Vasculitis Associated with Apixaban

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease, characterized by autoantibody production and immune complex formation, that has the potential to affect virtually any organ. Pleuropulmonary involvement occurs in 50&#x2013;70&#x0025; and commonly manifests as pleuritis and pleural effusion. Diffuse alveolar hemorrhage (DAH) is a rare manifestation of SLE. Most cases of DAH occur in young adults with an underlying autoimmune disease such as systemic vasculitis or Goodpasture syndrome. SLE is typically lower on the list of initial differential diagnoses of DAH due to its rarity compared to other etiologies. We present a case of a patient with dyspnea on exertion, dry coughs, lower extremity edema, and intermittent periorbital edema who ultimately succumbed to respiratory failure secondary to DAH in the setting of SLE. The diagnosis of SLE was suspected clinically and confirmed at autopsy due to her rapid clinical deterioration. DAH requires prompt intervention, and management is guided by the underlying disease process. SLE is a potentially treatable disease; therefore, timely diagnosis is important in order to exclude other noninfectious causes of DAH (reviewed in this report) and to initiate appropriate therapy.

Mia C. Lundgren

Jerry A. Molitor

Benjamin Spilseth

Oyedele Adeyi

A Fatal Case of Diffuse Alveolar Hemorrhage in the Setting of Systemic Lupus Erythematosus: A Case Report and Review of Noninfectious Causes of Acute Pulmonary Hemorrhage in Adults

Biological disease-modifying antirheumatic drugs (bDMARDs) are very effective for treating rheumatoid arthritis (RA). However, they sometimes induce adverse events such as psoriasis-like skin lesions. We describe psoriasis-like skin lesions that developed simultaneously with an RA flare in patient 1 during treatment with abatacept and in patient 2 soon after starting certolizumab pegol. The skin lesions persisted in patient 2 despite stopping certolizumab. Baricitinib was initiated because of RA flare and resulted in immediate beneficial effects on arthritis as well as skin lesions. The RA went into remission in both patients, and the psoriasis-like skin lesions disappeared within four weeks (patient 1) and three months (patient 2).

Yoshifumi Tada

Nobuyuki Ono

Syuichi Koarada

Immediate Effect of Baricitinib on Arthritis and Biological Disease-Modifying Antirheumatic Drug-Induced Psoriasis-Like Skin Lesions in Two Patients with Rheumatoid Arthritis

Background. Hypertrophic osteoarthropathy (HOA) is a rare finding in the setting of metastatic melanoma. A majority of cases of secondary HOA involve lung malignancies. Evaluation of presenting symptoms such as polyarthralgia and clubbing followed by review of imaging studies are diagnostic steps for HOA. Case Presentation. We present a 60-year-old female with a history of metastatic melanoma who presented with bilateral and symmetric polyarthralgia and clubbing. A plain film radiograph demonstrated periosteal thickening involving the metacarpals and proximal phalanges as well as the distal radius and ulna, consistent with HOA. The patient was treated with nonsteroidal anti-inflammatory agents for supported care. Conclusion. HOA may be a secondary manifestation of metastatic melanoma. Recognition and supportive care of this condition may lead to improved quality of life for patients.

Shiva Malaty

Aditya Gupta

Hypertrophic Osteoarthropathy: A Secondary Manifestation of Malignant Melanoma

An overlap of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) is extremely rare: approximately 40 cases have been reported to date. A literature review indicates that they are more common in women in their forties, and simultaneous onset has been reported in 69&#x0025; of cases. In addition, both lupus nephritis and ANCA-associated glomerulonephritis were observed on renal biopsy. This report presents the case of a 35-year-old woman with an 8-month history of polyarthralgia who was admitted to our hospital. She was diagnosed with SLE due to typical clinical presentation of the disease: polyarthritis, lymphocytopenia, hypocomplementemia, presence of antinuclear and anti-dsDNA antibodies, and proteinuria. However, purpura were scattered, and the titer of antimyeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) was high. A skin biopsy revealed leukocytoclastic vasculitis that involved poor immune complex deposition. A renal biopsy showed necrotizing glomerulonephritis with cellular and fibrocellular crescent formation that involved deposition of IgM and C3c only in the mesangial area and the peripheral capillaries. Additionally, no electron-dense deposits were observed under electron microscopy. These pathological findings were consistent with AAV rather than with SLE. Therefore, we finally diagnosed the patient with both SLE and microscopic polyangiitis. After treatment with methylprednisolone and intravenous cyclophosphamide pulse therapies, renal function improved and MPO-ANCA levels decreased. In cases of suspected overlap between SLE and AAV, appropriate diagnosis and treatment are important.

Hiroyuki Hounoki

Koichiro Shinoda

Atsushi Matsui

Maiko Okumura

Satoshi Yamaguchi

Kota Kakeshita

Hidenori Yamazaki

Tsutomu Koike

Teruhiko Makino

Kazuyuki Tobe

A Case of Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis Overlap Syndrome

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Case Reports in Rheumatology publishes case reports and case series on paediatric and adult rheumatological and musculoskeletal conditions, including novel therapies and advances in surgery and imaging.

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Case Reports in Rheumatology