Secondary cardiac amyloidosis in a patient with mixed connective tissue disease: A case report.

We report the case of a 62-year-old man who presented with shortness of breath, cough, bilateral lower limbs' swelling, and blackish discoloration of multiple fingertips over the past 2 months. Anti-Ribonucleoprotein antibodies were found to be present, and gadolinium-based cardiac MRI showed non-vascular subendocardial enhancement with diffuse symmetrical thickening of the left ventricular wall. A diagnosis of Mixed connective tissue disease with secondary cardiac amyloidosis was thus made, and the patient was successfully managed with intravenous cyclophosphamide, corticosteroids, and other supportive measures. Although extremely rare, this case shows that secondary cardiac amyloidosis should be considered while managing patients with MCTD.

Fatal ascending aortic aneurysm in a patient with systemic lupus erythematosus: A case report.

Aortic aneurysm is a potentially life-threatening condition with higher incidence in patients with systemic lupus erythematosus(SLE). Patients usually present with nonspecific symptoms and diagnosis is typically made incidentally through imaging studies. Management strategies include medical therapy to control inflammation and hypertension, surgical intervention for large or symptomatic aneursyms, and close monitoring for early detection of complications. We present a case of a 49-year female with multiple joint pain and other nonspecific symptoms for 7 years. Anti-ds DNA and ANA titre were significantly high and CT angiogram showed ascending aortic aneurysm measuring 5.5 cm. Conservative management was started with steroids, hydroxychloroquine, and antihypertensives, while awaiting surgery. However she suddenly collapsed, probably due to aneurysm rupture and could not be revived. Our case report therefore emphasizes the importance of close surveillance and timely intervention to minimize the morbidity and mortality in these patients.

Bronchiolitis obliterans following toxic epidermal necrolysis: a case report.

BACKGROUND: Toxic epidermal necrolysis (TEN) is a rare, acute and potentially fatal skin condition usually induced by drugs. Although much attention is focused on the life threatening acute cutaneous and sight threatening ocular manifestations of this disease, chronic pulmonary complications like bronchiolitis obliterans are occasionally encountered. However, little is known about its incidence, pathogenesis, clinical course and outcome in children recovering from TEN. CASE PRESENTATION: We report a five-year-old boy who presented four months after the first manifestation of drug-induced TEN with cough and shortness of breath and was subsequently diagnosed with bronchiolitis obliterans. He was treated with supportive therapy that improved his hypercapnia allowing him to be discharged on domiciliary oxygen, chest physiotherapy and bronchodilators. CONCLUSIONS: This case highlights the need to be vigilant for adverse drug reactions and consider chronic pulmonary complications like Bronchiolitis Obliterans in children recovering from TEN.

Anti-LGI1, anti-GABABR, and Anti-CASPR2 encephalitides in Asia: A systematic review.

AIM: We aim to review the literature to collate and describe features of encephalitides arising from autoantibodies against leucine-rich glioma-inactivated 1 (LGI1), gamma aminobutyric acid receptor (GABABR), and contactin-associated protein-like 2 (CASPR2) in Asian populations and compare them with findings of Western studies. METHODS: Peer-reviewed articles published till 24 May 2020 were searched, and original, full-text studies from Asia with serum/CSF antibody-based diagnosis and at least 2 patients were selected. Twenty-four studies with 263 patients (139 anti-LGI1, 114 anti-GAGABR, and 10 anti-CASPR2) were included. Data were pooled to produce descriptive information on demographics, clinical characteristics, diagnostics, treatments, and outcome. RESULTS: The mean age was 54.2 (anti-LGI1), 55.2 (anti-GABABR), and 47.7 years (anti-CASPR2), with an overall male predominance of 62.0%. Commonest clinical features across all types were seizures (87.5%), memory deficits (80.7%), psychiatric disturbances (75.9%), and altered consciousness (52.9%). Four anti-LGI1, 40 anti-GABABR, and 1 anti-CASPR2 patients had tumors. CSF, MRI, and EEG were abnormal in 33.3%, 54.1%, and 75% patients in anti-LGI1; 60.0%, 49.6%, and 85.7% in anti-GABABR; and 50%, 44.4%, and 100% in anti-CASPR2 patients, respectively. 95.6% patients received first-line therapy alone (steroids/IVIG/Plasma therapy), and 4.4% received second-line therapy (rituximab/cyclophosphamide). 91.7%, 63.6%, and 70% of patients had favorable outcomes (modified Rankin Score 0-2) with mortality rates at 2.5%, 23.2%, and 0% in the three types, respectively. CONCLUSION: Our findings suggest that these disorders present in Asian patients at a relatively young age often with features of seizures, memory deficits, and psychiatric disturbances and usually demonstrate a favorable clinical outcome.