ANTI-PHOSPHOLIPID ANTIBODY SYNDROME AND INVASIVE ASPERGILLOSIS IN AN ADULT MALE

P. Yegneswaran1*, V. Pandit1, I. Bairy1

Author address: 

1Manipal University, Kasturba Medical College

Abstract: 

Purpose: A case report Methods: Mr. MJ, 31yr. old, accountant was admitted with h/o fever, rash and polyarthralgia for the past 3 months. Dry cough was recent in onset. Evaluation at other centers showed pancytopenia and he had undergone antibiotic treatment & few days of ATT. On the 2nd day he had two episodes of seizures & restlessness. Treatment was started with IV methyl prednisolone followed by Cyclophosphamide with Piperacillin Tazobactum & Phenytoin. 6 -13 days later he developed breathlessness, cough & hypoxemia with chest infiltrates and noticeable decline in Hb, WBC and Platelet count. This was treated with G-CSF, packed cell & platelet transfusion and antibiotics; Meropenem, Teicoplanin and Oral steroids. During 3rd week patient developed deep vein thrombosis of left leg which was treated with low molecular weight heparin. Oral candidiasis was treated with IV fluconozole. Improvement in hematological parameters noticed with treatment. Fever, breathlessness , cough with moderate expectoration, tachypnea & restlessness persisted. Sputum & Bronchial lavage for fungal culture grew Aspergillus fumigatus. Bronchial brush cytology showed numerous epithelial cells along with acute branching septal fungi suggestive of Aspergillosis. He was treated with Amphotericin B for 4 days, rapidly deteriorated, and succumbed. Results: Final diagnosis was multisystem involved SLE (hematological, neurological, renal) with Antiphospholipid antibody syndrome (venous thrombosis + positive cardiolipin antibody) and invasive pulmonary aspergillosis due to Aspergillus fumigatus. Conclusions: Pulmonary involvement occurs in most patients with SLE, however severe involvement is rare. Differential diagnosis for pulmonary lesions are Pleurisy ± effusion (50%), acute lupus pneumonitis, alveolar hemorrhages, opportunistic infections, bronchiolitis obliterans, chronic interstitial pneumonitis, pulmonary vasculitis & embolism. Leading cause of death in SLE is infection & majority of fatal infections are due to opportunistic organisms. Among fungal infections candida is most common agent followed by nocardia & cryptococcus. Aspergillosis in SLE is rarely described. Risk factors for aspergillosis in SLE are high grade disease activity, neutropenia & prolonged use of immunosuppressives. Case series by Katz et. Al. (Seminars in Arthritis & Rheumatism 1996) reveal that only 23 cases have been reported in literature, of which only one male patient survived. In review of 379 treated aspergillosis cases, by Denning et al, none had SLE. Invasive aspergillosis most commonly presents as pulmonary disease; pneumonia/ lung abscess. The presence of septate, acute, branching hyphae invading the lung tissue, along with culture positive for Aspergillus, is diagnostic of invasive pulmonary aspergillosis. Definitive diagnosis is often delayed since the sputum and blood cultures are usually negative. Unfortunately most of the reported cases are diagnosed at postmortem. Invasive procedures such as bronchoscopy, VATS lung biopsy and open lung biopsy, are often required for early diagnosis. Other recent methods used in the diagnosis are Aspergillus PCR from the fluids and estimation of galactomannan in the serum. At present, Voriconazole is the treatment of choice for invasive aspergillosis. Combination of Capsofungin with lipid formulation of amphotericin B or triazole is also promising. Despite antifungal therapy mortality is high and exceeds 70%. Unique features of the case we presented are Young male complicated by aspergillosis, early in the disease course. Prolonged neutropenia might be the predisposing factor, as he was not treated in the past with immunosuppressives.
2010

abstract No: 

168

Full conference title: 

4th Advances Against Aspergillosis
    • AAA 4th (2010)