Anti-melanoma Differentiation-Associated Gene 5 (Anti-MDA5) Dermatomyositis: A Case Presentation.

We present a case of anti-melanoma differentiation-associated gene 5 (Anti-MDA5) dermatomyositis (DM) in a 30-year-old female. Anti-MDA5 dermatomyositis, previously termed clinically amyopathic dermatomyositis, was first recognized in 2005. Most cases present with varying combinations of cutaneous and oral ulcerations, palmar papules, respiratory symptoms, and minor muscle involvement (most commonly in the shoulders, upper arms, hips, thighs, and neck). This subtype of disease is most notable for its association with an increased risk of rapidly progressive interstitial lung disease. Our patient presented initially with only complaints of cutaneous ulcerations on the dorsal aspect of her hands. Following several months of no true diagnosis, she developed muscle weakness and joint pain. This led to retrieval of a punch biopsy which suggested anti-MDA5 DM at the top of the differential diagnoses. Immunoprecipitation revealed the presence of melanoma differentiation-associated gene 5 (MDA5) antibodies, confirming the diagnosis of anti-MDA5 dermatomyositis. This case demonstrates the importance of pinpointing the diagnosis of this rare disease subtype in a timely manner to prevent a fatal course, and we hope to inform dermatologists, rheumatologists, pulmonologists, and internists alike of the uncommon presentation of anti-MDA5 in an unsuspected, young patient.

Cardiovascular Risk Associated With TNF Alpha Inhibitor Use in Patients With Rheumatoid Arthritis.

Rheumatoid arthritis (RA) is an autoimmune disease characterized by inflammation and pannus formation, with subsequent joint and cartilage degradation. Treatment commonly targets inflammatory cytokines, including tumor necrosis factor (TNF) alpha, which is a potent inflammatory cytokine required for cell signaling, regulation, and apoptosis, as well as for other cellular functions including immune response. TNF alpha inhibitors have demonstrated benefits in improving RA patient outcomes in terms of immune function and symptomatology. While TNF alpha inhibitors are generally beneficial, some studies have demonstrated that TNF alpha inhibitors may increase the risk of adverse cardiovascular events. While this continues to be debated, our study investigates the role of Tumor Necrosis Factor Receptor 1 (TNFR1) and Tumor Necrosis Factor Receptor 2 (TNFR2) in cardiac tissue. TNFR1 is an apoptotic receptor and its inhibition by TNF alpha inhibitors is subsequently cardioprotective. However, TNF alpha inhibitors may be inhibiting TNFR2 receptors even more so than TNFR1 receptors. TNFR2 is primarily a cardioprotective receptor and its greater inhibition results in the cardiovascular morbidity associated with TNF alpha inhibitors.

Giant Cell Arteritis and Cardiac Comorbidity.

Giant cell arteritis (GCA) is a large vessel vasculitis with a pathogenesis that involves two CD4 T-helper cell lineages, Th1 and Th17. The goal of GCA treatment is to achieve clinical remission and prevent complications, especially vision loss. Despite recent advances in treatment and diagnostic modalities for GCA, there continues to be a gap in the medical literature in addressing treatment and follow-up for patients with GCA after clinical remission is achieved. Of the most important issues to address in this patient population by rheumatologists and primary care physicians alike, is that of cardiovascular disease (CVD) risks in GCA patients associated with the vasculitis and its mainstay of treatment with high-dose glucocorticoids over a prolonged period of time. Physicians must be aware of the CVD events that have been observed in a higher proportion compared to the general population in GCA patients, including strokes, thoracic aortic aneurysms and dissections, myocardial infarctions, and peripheral vascular disease. This review will focus on the risk of CVD in GCA patients, with recommendations for management and follow-up.

Repetitive Microtrauma and Negative Ulnar Variance as Possible Culprits of Avascular Necrosis of the Lunate.

Kienböck's disease is characterized by avascular necrosis of the lunate. Its pathophysiology involves a complex interplay of repetitive microtrauma, anatomical and vascular variances. Early diagnosis of this entity is challenging as disease presentation is nonspecific and can mimic common wrist pathologies such as fractured or sprained wrist. Here we report a case presentation of Kienböck's disease Stage IIIA in a 28-year-old healthy male. Initial radiographs of the left wrist were inconclusive and two weeks later the diagnosis was confirmed with a magnetic resonance imaging. The patient was initially treated with cast immobilization for four months but remained symptomatic with no improvements in pain or function. He then elected to participate in left radial shortening osteotomy with a vascularized bone graft from the distal radius. Unfortunately, both conservative and invasive procedures did not prevent end-stage disease characterized by the complete collapse of the lunate. However, 18 months post-surgical follow-up, the patient continues to remain pain-free with no limitations to his daily living activities.

Reconsidering the Use of Minocycline in the Preliminary Treatment Regime of Rheumatoid Arthritis.

Strong epidemiologic, clinical, and basic science studies have identified a number of factors that may lead to rheumatoid arthritis (RA) onset and progression, particularly involving the complex interplay between genomics, environmental risk factors, the breakdown of immune self-tolerance, and microbiome dysbiosis. A chronic state of inflammation established by infectious agents has long been suspected to set the stage for the development of RA. The purpose of this article is to review the contribution of the gut, lung, and oral microbiomes to the pathogenesis of RA and consider the importance of supplementing the preliminary treatment regime of RA patients with antibiotics, in particular, minocycline. Minocycline has been used in the treatment of RA due to its bacteriostatic, as well as immunomodulatory and anti-inflammatory properties. Ultimately, a short course of antibiotic treatment with minocycline may eliminate pathogenic organisms contributing to the development and progression of RA.

Uveitis Sarcoidosis Presumably Initiated After Administration of Shingrix Vaccine.

Uveitis is a form of intraocular inflammation that can occur as a result of infection, injury, vaccination, or as a systemic manifestation of autoimmune or inflammatory diseases including sarcoidosis. Sarcoidosis is an inflammatory disease that involves the formation of abnormal granulomas in multiple organ systems. The hallmark of sarcoidosis is a non-caseating granuloma seen on biopsy. Here, we present a case report of a healthy 56-year-old Caucasian female who presented with uveitis sarcoidosis that was presumably initiated after administration of the Shingrix vaccine. Shingrix is a new attenuated subunit vaccine for Varicella Zoster Virus with an AS01B adjuvant that can result in a potent immune response. The Shingrix vaccine is made using Chinese hamster ovary cells which can contaminate the final vaccination product. Together, through the process of molecular mimicry and strong induction of the immune response, administration of Shingrix may have caused or exasperated this patient's autoimmune etiology.