From first symptoms to diagnosis of systemic lupus erythematosus: mapping the journey of patients in an observational study.

OBJECTIVES: Although increased awareness for systemic lupus erythematosus (SLE) has reduced diagnostic delay, the average time from symptom onset to diagnosis is still long, potentially resulting in adverse outcomes. We mapped the journey of lupus patients from onset of symptoms to disease diagnosis. METHODS: We carried out an observational study of 275 SLE patients with disease duration <6 years. Data were collected from patient charts, interviews and in-person clinical visits. Total delay was divided in i) time from symptom onset to rst physician visit, ii) time from rst visit to assessment by rheumatologist, and iii) time from initial rheumatologist assessment to nal diagnosis. Early diagnosis was de ned as diagnosis within 6 months from symptom onset. RESULTS: Most common initial symptoms were arthritis/arthralgia (74.5%) and rashes (61.8%). Median (IQR) total delay between symptom onset and SLE diagnosis was 24 (54) months. An "early" diagnosis was achieved only in 28.4% of patients, while 55.6% were diagnosed after 12 months, with patients consulting an average of 3 different physicians before reaching diagnosis. Oral ulcers (OR 3.55; 95% CI 1.45-8.70) and malar rash (OR 1.99; 95% CI 1.00-3.94) as initial symptoms, and rst medical assessment by orthopaedic (OR 5.18; 95% CI 1.47-18.20) were independently associated with a delayed diagnosis. The latter was also associated with increased SDI at the time of diagnosis (OR 2.42; 95% CI 1.03-5.69), attributed mainly to neuropsychiatric and thrombotic events. CONCLUSIONS: Diagnosis of SLE is delayed by more than 6 months in three quarters of patients and is associated with more damage accrual.

First Diagnosis of Systemic Lupus Erythematosus in Hospitalized Patients: Clinical Phenotypes and Pitfalls for the Non-Specialist.

BACKGROUND: Prompt recognition of systemic lupus erythematosus (SLE) in hospitalized patients presenting with severe disease is essential to initiate treatment. We sought to characterize the phenotype of hospitalized patients with new-onset SLE and estimate potential diagnostic delays. METHODS: An observational study of 855 patients ("Attikon" SLE cohort). Clinical phenotype was categorized according to the leading manifestation that led to hospitalization. Disease features, time to diagnosis, classification criteria, and the SLE Risk Probability Index (SLERPI) were recorded for each patient. RESULTS: There were 191 patients (22.3% of the total cohort) hospitalized due to manifestations eventually attributed to SLE. Main causes of admission were neuropsychiatric syndromes (21.4%), cytopenias (17.8%), nephritis (17.2%), and thrombotic events (16.2%). Although 79.5% of patients were diagnosed within 3 months from hospitalization, in 39 patients diagnosis was delayed, particularly in those with hematological manifestations. At hospitalization, a SLERPI >7 (indicating high probability for SLE) was found in 87.4% of patients. Patients missed by the SLERPI had fever, thrombotic or neuropsychiatric manifestations not included in the algorithm. Lowering the SLERPI threshold to 5 in patients with fever or thrombotic events increased the diagnostic rate from 88.8% to 97.9% in this subgroup, while inclusion of all neuropsychiatric events yielded no additional diagnostic value. CONCLUSION: One in five patients with new-onset SLE manifest disease presentations required hospitalization. Although early diagnosis was achieved in the majority of cases, in approximately 20%, diagnosis was delayed. A lower SLERPI cut-off (≥5) in patients with fever or thrombosis could enhance early diagnosis.

Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study.

OBJECTIVES: Interstitial pneumonia with autoimmune features (IPAF) refers to patients with interstitial lung disease and autoimmune features not fulfilling the classification criteria for a specific connective tissue disease. We sought to study the characteristics, disease progression, response to treatment and complications of patients with IPAF in 1-year follow-up period. METHODS: Clinical and laboratory findings, comorbidities, medications, pulmonary function tests (PFTs), chest HRCT and complications during the one-year follow-up period were documented for each of the 39 enrolled patients with IPAF. RESULTS: The mean age at the time of IPAF diagnosis was 63.2 (±11) years, and 62% of patients were female. The most common clinical features were arthritis (82%) and rash (54%-not included in the IPAF criteria). Antinuclear antibodies (ANA) (59%) and non-specific interstitial pneumonia (NSIP-61.5%) were the most prevalent autoantibodies and radiological pattern respectively. PFTs at 12 months from baseline stabilized or improved in 79.5% of patients (p> 0.05). Infections were observed in 23.1% of patients during the first and in 12.8% during the second semester of follow-up. Two patients (5.1%) required hospitalization. All infections occurred in patients with non-usual interstitial pneumonia (UIP) pattern (p=0.02). CONCLUSIONS: Arthritis and rash are among the most common features in IPAF suggesting rash could be included into IPAF criteria. Almost 80% of patients had stable/improved PFTs at the end of follow-up. Infections occurred mainly in the first semester of treatment and in patients with non-UIP radiological pattern probably due to higher doses of corticosteroids used in these patients.