Scleroderma Renal Crisis is Associated with High Mortality: A Real-World Study from India.

INTRODUCTION: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis. Since the use of ACE inhibitors in this condition, there has been a significant reduction of mortality rates. However, there is limited data on characteristics and outcomes of SRC from developing countries. METHOD: This was a single centre, case-control study from India. The records of all patients admitted in the last 5 years were scrutinized, and patients with SRC (as per the updated consensus classification, 2014) were compared with patients of systemic sclerosis who were admitted for other reasons (controls). Disease characteristics, between cases and controls, were compared using chi-squared test, and odds ratios (OR) were calculated. Survival was compared using KaplanMeier statistics. RESULTS: Ninety-four patients of systemic sclerosis admitted over five-years; among them 15 had SRC. As compared to controls, those with SRC had a significantly higher rates of pericardial effusion (OR 11.7, p=0.02), dilated cardiomyopathy (OR 2.5, p=0.04), myopathy (OR 19.3, p=0.001), taking mediumhigh dose glucocorticoids (OR 7.9, p=0.009) and recent disease onset (OR 39.3, p=0.01). Despite aggressive control of hypertension with ACE inhibitors, 10/12 patients with SRC died. Mean (SD) survival in patients with SRC (11.5, 95% CI 5.7 to 17.6 months) was significantly lower than controls (66.2, 95% CI 58.4 to 73.9 months, p<0.001). CONCLUSION: In this single-centre study from a developing country, scleroderma renal crisis was associated with a dismal prognosis, despite the use of ACEI. The recent use of medium-high dose glucocorticoids was associated with SRC.

Fluorodeoxyglucose positron emission tomography/computed tomography in the diagnosis, assessment of disease activity and therapeutic response in relapsing polychondritis.

OBJECTIVE: To evaluate 18F-fluorodeoxyglucose (FDG) PET/CT in the assessment of disease activity, extent of the disease and response to therapy in relapsing polychondritis. METHODS: Twenty-five patients (9 men, 16 women) with a mean age of 38.2 years (s.d. 13.7; range 18-62), diagnosed to have relapsing polychondritis according to Damiani and Levine's modification of McAdam's criteria, who underwent PET/CT examination were included. Ten patients underwent a second PET/CT examination after therapy or during follow-up. Clinical symptoms and auxiliary examination findings were recorded. PET/CT findings were reviewed and correlated with the clinical symptoms. RESULTS: The major symptoms were aural pain (n = 21), nasal pain (n = 10), stridor (n = 5), cough (n = 9), fever (n = 8) and laryngeal tenderness (n = 8). The initial PET/CT was positive in 23/25 patients. PET/CT revealed involvement of auricular (n = 14), nasal (n = 8), laryngeal (n = 7), tracheobronchial (n = 6) and Eustachian (n = 3) cartilages with a mean maximum standardized uptake value (SUVmax) of 4.1 (s.d. 2.5; range 1.7-12.7). Fair correlation of aural/nasal pain/stridor with FDG avidity of cartilage involvement on PET/CT was noted. The key finding was detection of asymptomatic large airway involvement in seven patients (28%). Re-examination PET in 10 patients revealed complete therapeutic response (n = 5), partial response (n = 1), stable disease (n = 1), progressive disease (n = 1) and disease recurrence (n = 2). CONCLUSION: FDG PET/CT is a useful tool for the assessment of the disease activity and extent. It identified activity in clinically inaccessible sites that are of clinical significance. It is also useful in assessing treatment response and finding relapse.

Acute myocardial dysfunction in lupus: outcomes in Asian Indians.

BACKGROUND: Acute myocardial dysfunction is an uncommon but potentially fatal complication in systemic lupus erythematosus (SLE). We describe the outcome in a small series of Asian Indian patients and examine associated factors. METHODS: SLE patients who fulfilled the 2012 SLICC criteria and developed new-onset myocardial dysfunction were included in this retrospective case series. Acute myocardial dysfunction was defined as global hypokinesia and left ventricular ejection fraction (LVEF)<50% on echocardiography (with or without symptoms) in patients with SLE. Survival was assessed using Kaplan-Meier survival analysis and Cox regression. RESULTS: This study included 37 patients with mean age 28.2 ± 11.2 years and median (range) LVEF of 35% (18-48%) at presentation. A majority had active disease, with SLEDAI-2k ≥ 5 in 26 (of 28). All patients received oral corticosteroids and a majority received additional immunosuppression, including pulse methylprednisolone in 28 and cyclophosphamide in 27. Nine patients died during hospitalisation (25%), a majority due to infections. Death was significantly associated with elevated procalcitonin at presentation (p = 0.05), elevated white cell count (p = 0.02) and low complement C3 (p = 0.03). In those who survived, long-term outcomes were good, with complete myocardial recovery in 14 (64%). A higher ejection fraction at presentation was associated with complete recovery. CONCLUSIONS: In this small series of patients of SLE with acute myocardial dysfunction, we report a significant in-hospital mortality due to infections. Many of the patients who died had elevated procalcitonin at presentation. A diligent search for infection seems prudent in lupus patients who present with acute myocardial dysfunction. Key Points • In patients of SLE with acute myocardial dysfunction who were treated with immunosuppression, there was significant short-term mortality due to infections. • This mortality was associated with elevated procalcitonin at baseline and may suggest some of them had pre-existing hidden sepsis. • A prudent search for infections in these patients before immunosuppression may help to decrease short-term mortality.