The patient is a 62-year-old Bangladeshi male with Non-alcoholic Steatohepatitis (NASH) related cirrhosis, sarcoidosis, and poorly controlled type II diabetes who presented with one week of chest pain and five days of cough. The chest pain was non-exertional, pleuritic and localized to the left lateral chest wall. The cough was productive of scant yellow sputum and the patient denied history of hemoptysis. He had no accompanying fevers, chills, night sweats, or recent changes in weight. He immigrated to the United States from Bangladesh in 1991, and reported travel to Saudi Arabia 4 years prior to presentation, and to London 2 weeks prior to presentation. The patient was on no immune-modulating therapy.
CT imaging of the chest revealed bilateral, predominantly subpleural cavitary pulmonary nodules. He underwent IR guided fine needle aspiration of a left lower lobe lung nodule. Microscopy was consistent with zygomycete and fungal cultures from the specimen grew Rhizopus oryzaeon day four of incubation. Maxillofacial CT and MRI imaging, as well mucosal biopsies were negative for signs of paranasal sinus involvement. Surgical debridement was deferred due to the multifocality of the lung involvement, and the patient was initiated on IV liposomal amphotericin B at a dose of 5mg/kg/day. He was transitioned to isavuconazole (372mg q8hr IV for two days, followed by 372mg PO daily) when his renal function began to deteriorate on amphotericin B. CT chest performed after six months of therapy revealed interval improvement in the multifocal cavitary lesions.
Discussion:
Here we present a case of isolated pulmonary mucormycosis in a patient with poorly controlled diabetes, NASH cirrhosis, and sarcoidosis that had not required therapy. The case provides instructive clinical points regarding the presentation of this rare disease entity, and is illustrative of several of the more nuanced treatment decisions confronting clinicians in the management of mucormycosis.
Pulmonary mucormycosis is a rare opportunistic invasive fungal infection of the immunocompromised host. Individuals with diabetes mellitus, hematological malignancies, renal insufficiency, and who are recipients of solid organ transplants are predisposed to infection. Clinical manifestations include fever, cough, hemoptysis, and dyspnea. Radiographic findings are varied, but a reverse halo sign is particularly suggestive of the diagnosis. Direct microscopy of clinical specimens can provide rapid presumptive diagnosis of mucormycosis, and in recent registries, histopathology has yielded diagnosis in between 63 and 66% of cases.
Current guidelines recommend combined pharmacologic and surgical management in addition to correction of any underlying predisposing disorder.
Conclusions:
As was the case here, clinical circumstances may preclude recommended therapy. Liposomal amphotericin B is the currently recommended pharmacotherapy, however a recent single-arm, open-label trial of isavuconazole for treatment of mucormycosis demonstrated efficacy similar to that of amphotericin B after 180 days of therapy when used either as a primary treatment or as a salvage therapy for patients with refractory disease.2While more data is needed to support this approach, it represents a clinically viable alternative when either patients are refractory to liposomal amphotericin B, or cannot tolerate the therapy.