Invasive fungal infection in ANCA-associated vasculitis: Between the Devil and Deep blue sea. Case series and review of the literature.

INTRODUCTION: Patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are susceptible to opportunistic infections, including invasive fungal infections (IFI). This is due to many factors, including prolonged immunosuppressive therapy. The treatment of AAV with such IFIs is challenging. METHODS: A descriptive analysis of 5 patients with AAV complicated by concomitant invasive fungal infections was performed. We also have done a comprehensive literature review of IFIs in AAV using PubMed and Google Scholar databases. RESULTS: All 5 patients initially received immunosuppressive medication but subsequently acquired IFI. One patient had sphenoid sinus involvement, and four had lung parenchymal involvement. Aspergillus infection was diagnosed in three patients, Cryptococcus infection in one patient and mixed infection with Aspergillus and Mucor infection in one patient. All our patients were on low doses of corticosteroids for several months to years or had received high-dose pulse steroids with cyclophosphamide in the last few weeks before being diagnosed with IFI. It was difficult to distinguish disease activity from IFI in all the cases. Two of the five patients died despite antifungal therapy. The literature review revealed a prevalence of IFIs ranging from 1 to 9.6% (excluding pneumocystis pneumonia). Aspergillosis was the predominant type of IFI, affecting 46 of 86 patients. Most of these patients (40/46) had pulmonary involvement. The prognosis for patients with IFI was consistently poor, as evidenced by 19 deaths out of 29 reported outcomes. CONCLUSION: Overall, IFIs have a poor prognosis in patients with AAV. Differentiating disease activity from IFI is difficult because of similar organ distribution, imaging lesions, and histopathological characteristics. A high suspicion index and good-quality microbiology are needed for early treatment and prevention of mortality.

Rituximab for treatment of pachymeningitis in granulomatosis with polyangiitis.

Pachymeningitis is a rare manifestation of granulomatosis with polyangiitis (GPA), and there is a paucity of literature regarding the efficiency of rituximab (RTX) in such cases. We present a woman in her early 50s with GPA who initially responded adequately to conventional therapy. However, the treatment course was complicated by pulmonary cryptococcosis. The patient later developed non-infectious pachymeningitis, which failed to respond to steroids and methotrexate. However, using RTX upfront led to good clinical response and radiological resolution.

Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre.

Three young males with Hugh-Stovin's syndrome presented with cough, haemoptysis, fever, raised inflammatory markers, and pulmonary artery aneurysm. Only one had recurrent oral ulcers suggestive of Behcet's disease, and none were HLA B51 positive. All responded well to immunosuppression but eventually needed either an endovascular procedure or surgery.

Improved pulmonary function following pirfenidone treatment in a patient with progressive interstitial lung disease associated with systemic sclerosis.

Pirfenidone is an anti-fibrotic drug which has been approved for the management of patients with Idiopathic Pulmonary Fibrosis (IPF). However, its role in interstitial lung disease (ILD) due to other causes such as systemic sclerosis (SSc) is not clear. We present a case of a patient with SSc associated ILD who showed a subjective as well as objective improvement in lung function with pirfenidone.

Diagnosis and management of psoriatic arthritis.

Psoriatic arthritis (PsA) is a multi-faceted disease marked by varying combinations of peripheral arthritis, dactylitis, spondylitis, and enthesitis. Rarely, recurrent uveitis occurs. Skin involvement may or may not exist. However, patients with nail psoriasis have a higher probability of developing PsA. Untreated patients have significant morbidity and mortality. Timely diagnosis and aggressive treatment of the disease can lead to lower morbidity. Drug therapy of PsA includes symptomatic therapy and therapy with disease-modifying anti-rheumatic drugs. Biologics are the only agents that address all the pathological changes, of this chronic condition.