Background:Primary CNS Vasculitis (PCNSV) is an uncommon inflammatory condition selectively affecting blood vessels of the central nervous system and has diagnostic difficulties. Long term treatment outcomes are unclear.Objective:To elucidate the clinical progression and long-term outcomes of patients with biopsy proven PCNSV.Methods and Material:Clinical data of eight consecutive patients with PCNSV established by meningocortical biopsy were reviewed. Patients had undergone extensive evaluation for secondary causes.Results:The average age at presentation was 33.5 years (25 to 47 years). Three were females. The average follow-up period was 65 months (24 to 108 months). Clinical presentation included acute or subacute focal neurological deficits, seizures, headache and cognitive decline. MRI findings consisted of multifocal gray and white matter signal changes with microhaemorrhages. On contrast multifocal areas of enhancement affecting gray matter, white matter, vessel wall, dura and leptomeningeal areas were observed. Diagnostic yield of DSA was poor. Histopathology showed granulomatous angiitis in six and lymphocytic angiitis in two. Treatment protocols followed were similar for all patients: initial induction with intravenous methylprednisolone and cyclophosphamide, followed by maintenance immunosuppressants (with or without oral steroids). Disease control was reflected by significant improvement on MRI. Multiple clinical relapses occurred over the years (13 relapses, 0 to 4 per patient). Serial MRI studies helped detect subclinical disease activity. Treatment escalation aimed at achieving "no evidence of disease activity" helped in better disease control and remission in patients with active disease. Rituximab was useful in select cases to control disease activity and as rescue therapy (n = 3). Mean MRS score at last follow up was 0 or 1 (unchanged from baseline). Treatment complications included infections and avascular necrosis of hip (n = 1). One patient died due to severe COVID-19 pneumonitis.Conclusion:PCNSV has varied clinical presentation and diagnostic challenges. Immunosuppressive therapy is necessary and is effective but with associated risks. Histopathological confirmation helps to prognosticate and initiate early aggressive treatment for better results. Close clinico-radiological follow up and long-term immunotherapy to ensure disease remission can help manage these patients successfully.
Introduction: Comorbidities frequently accompany psoriasis and psoriatic arthritis (PsA) and add to the disease burden. We aimed to identify the comorbidity burden in patients with PsA and evaluate its impact on the disease activity measures in our geographic region. Methods: This was a multicenter, cross-sectional study involving consecutive PsA patients from 17 rheumatology centers. Their disease variables and comorbidities were recorded. Results: In 549 enrolled patients, the mean age was 39.2 (14.9) years, with male predominance (6:5). The mean duration of PsA was 63.1 (76.3) months and 232 (42.3%) patients had one or more comorbidities. Dyslipidemia was the most prevalent comorbidity, followed by hypertension (HTN) (19.8%) and diabetes (16.6%). About 39% of patients were overweight and 18% were obese. Smoking, ischemic heart disease, hypothyroidism, osteoarthritis, depression, anxiety, and fractures were seen in <5% of the cohort. Increasing age, longer duration of psoriasis, a family history of cardiovascular disease (CVD) or stroke, smoking, alcohol consumption, and higher waist circumference were associated with the presence of one or more comorbidities. Overall, 104 (18.9%) patients needed hospitalization for various comorbidities. Infections accounted for 59 (10.8%), of which skin (23) was the most common site, followed by urinary tract (6) and lung (4). Conclusions: More than 40% of PsA patients have comorbidities. Dyslipidemia, HTN, diabetes, and obesity were most prevalent, putting these patients at risk for CVDs. Active screening for these comorbidities is crucial for providing comprehensive care to these patients.
Introduction: Psoriatic arthritis (PsA) is a chronic inflammatory disease with significant functional impairment. Health Assessment Questionnaire-Disability Index (HAQ-DI) is a reliable and validated outcome measure for a variety of arthritides including PsA. Objective: The objective of this study was to assess the disability as an outcome measure in PsA using the Indian version of HAQ (I-HAQ). Methods: The I-HAQ was administered to PsA patients diagnosed as per the Classification Criteria for PsA. The I-HAQ comprises 12 questions (nine basic and three advanced activities of daily living (ADLs), on the standard HAQ format) relevant to the Indian population. Results: In the 549 participants, the mean I-HAQ was 0.31 (0.45) and 48.2% had mild-to-moderate disability (I-HAQ>0–1). Female gender, older age, higher skin, joint scores, and Disease Activity Index for PsA were associated with some disability (I-HAQ>0). Symmetric polyarthritis (0.34) and spondyloarthritis (0.32) had a significantly higher disability compared to other subsets. Analyzing the individual questions of I-HAQ, squatting in the toilet or sitting cross-legged on the floor (r = 0.78), walking 3 km (r = 0.77), and climbing a flight of stairs (r = 0.74) correlated maximally to the total I-HAQ. ADL which was affected most frequently was “climbing a flight of stairs.” I-HAQ was significantly lower in patients who had been on disease-modifying antirheumatic drugs for 6 months or more (P = 0.0001). Conclusions: The Indian version of HAQ-DI could be efficiently employed to assess outcomes in our cohort. Nearly half of the cohort had mild-to-moderate disability suggesting a high burden of inflammation. Higher joint activity scores are strongly associated with disability.
Angioedema is defined by World Allergy Organization as a transient swelling of the skin or submucosal surface due to increased vascular permeability of small venules. Most common etiologies include IgE-mediated allergic reactions (to food, drug, or environmental triggers), nonsteroidal anti-inflammatory drug (NSAID), and chemically induced histamine release (most commonly opiates, highly cationic antibiotics, and muscle relaxants). Recent publications show a global incidence of anaphylaxis between 50 and 112 episodes per 100 000 person-years while the estimated lifetime prevalence is 0.3-5.1%. Timely intubation and administration of steroids/ antihistaminic agents will prevent a catastrophic cannot intubate cannot ventilate situation. In small, resource limited environment of a field hospital which may be compared to a rural setting in India managing such emergencies is challenging. If available in a case of anticipated difficult airway like this difficult airway cart must contain a whole armamentarium of airway adjuncts and gadgets. History of consumption of fish (Nile Perch) a rare form of allergen causing airway and breathing issues leading to timely intubation saved the life of patient.
