Scleritis and development of immune-mediated disease: A retrospective chart review.

OBJECTIVE: Scleritis may be idiopathic or caused by trauma, infections, or an immune-mediated condition. Our study aimed to understand the relationship between scleritis and immune-mediated disease, including presenting characteristics, serologies, and treatment course. Understanding these associations may allow clinicians to risk-stratify patients and predict their clinical and treatment course. METHODS: We conducted a retrospective chart review of 341 scleritis patients seen at a tertiary care center between January 1, 2005, and December 31, 2020. Demographics, scleritis characteristics, treatment response, recurrence, and serologic data were compared among patients with idiopathic and immune-mediated disease-associated scleritis. RESULTS: Among scleritis patients seen, 145 patients (43%) had an associated immune-mediated disease, most commonly rheumatoid arthritis (39%), vasculitis (21%), or inflammatory bowel disease (14%). In most cases, the immune-mediated disease diagnosis predated the scleritis presentation (65%), though vasculitis cases were more likely to develop during or after scleritis episodes. There were no significant differences in demographics or treatment failures among scleritis patients with and without associated immune-mediated conditions. Patients with immune-mediated diseases were more likely to have a recurrence of scleritis (62% vs 49%, p=0.02). CONCLUSION: At our ophthalmology center, 43% of patients with scleritis had an associated immunemediated disease, and most patients with immune-mediated disease were symptomatic from this disease prior to scleritis presentation. Rheumatoid arthritis was the most commonly associated condition and typically predated the scleritis, while vasculitis was more likely diagnosed during or after the scleritis episode. Scleritis among immune-mediated disease patients is more likely to recur compared to scleritis that is idiopathic.

Intravenous Immunoglobulin in Idiopathic Inflammatory Myopathies: a Practical Guide for Clinical Use.

PURPOSE OF REVIEW: Idiopathic inflammatory myopathies (IIM) are a complex family of autoimmune systemic disorders which often affect muscle and/or skin. IIM cause significant morbidity and mortality, but optimal treatment is uncertain. This review provides a practical guide for using intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) in the management of IIM, including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myositis (IMNM), and spontaneous inclusion body myositis (IBM), based on relevant recent literature and experience. We summarize pertinent considerations when using IVIG in special circumstances, including myositis-related dysphagia, interstitial lung disease (ILD), calcinosis cutis, and pregnant patients. This review also discusses IVIG safety, available formulations, and costs. RECENT FINDINGS: While IVIG has been used de facto for severe IIM for over 30 years, prior clinical trials of IVIG were notably limited. Recently, however, IVIG has proven safe and effective against IIM in several high-impact publications, including a large prospective, randomized placebo-controlled phase III study in DM. IVIG is useful against both muscular and extra-muscular manifestations in many types of IIM. It can be used as a first-line, steroid-sparring agent or as add-on to other treatments, tailored to specific clinical IIM scenarios. It is generally well-tolerated and has good safety profile, but accessibility and cost still limit its use.

Unusual cases of Anti-SRP necrotizing myopathy with predominant distal leg weakness and atrophy.

Anti-SRP necrotizing myopathy is classically characterized by subacute or chronic, severe, progressive and symmetric myositis which predominantly affects proximal muscles. We report two unusual cases presenting with predominantly distal, asymmetric weakness, with selective involvement of the posterior compartment of the thighs, gastrocnemius, and soleus muscles, in addition to inflammation and edema on STIR or T2-weighted, fat-saturated MRI. In each case, creatine kinase (CK) levels were >10 times normal and myositis panels returned positive for anti-SRP. ANA, ENA, RF, and HMGCR antibody were all negative. Nerve conduction study (NCS) was normal. Electromyography (EMG) confirmed diffuse myopathy with fibrillation potentials and positive sharp waves. Additional work up, including whole exome sequencing (WES), immunohistochemical staining for different types of muscular dystrophy, and western blot for calpain 3 and dysferlin were negative. The strength and CK levels of both patients markedly improved following immunosuppression. Our cases emphasize the importance of considering anti-SRP necrotizing myopathy in patients presenting with recent onset predominant asymmetric distal leg weakness of unclear etiology, and support the usefulness of MRI of the distal legs for early recognition. Given the potential consequences of delays in treatment of this condition, the recognition of this clinical pattern is important and can allow for prompt initiation of aggressive immunotherapies.

Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances.

Cryoglobulins are immunoglobulins that precipitate at temperatures less than 37°C. They occur secondary to infectious, autoimmune, and malignant processes. In the Brouet classification, type I cryoglobulinemia is caused by hyperviscosity, whereas type II and III manifestations are caused by vasculitis in target organs (primarily skin, peripheral nerves, and kidney). New classification criteria were recently proposed that may help with study and treatment of cryoglobulinemic vasculitis (CryoVas). Hepatitis C virus is the most common cause of CryoVas and treatment with antivirals can be curative in mild cases, whereas rituximab is highly effective in treating active vasculitis in more severe cases.

The enigma of vasculitis.

We describe the case of an 86-year-old man presenting with clinical symptoms suggestive of Temporal Arteritis. The evolution of the case prompted extensive work-up, including temporal artery and kidney biopsy. Based on the clinical and pathological findings, a diagnosis of ANCA-negative granulomatous necrotizing vasculitis involving the small-, medium-, and large-size vessels was made. The patient was treated with prednisone and cyclophosphamide, which was later switched to rituximab. The patient remained asymptomatic under this regimen and stabilization of his kidney function was achieved.