3 | DISCUSSION
Mucormycosis represents a group of infections caused by the fungi
belonging to the order Mucorales and family
Mucoraceae.1,6 Pulmonary mucormycosis
accounts for ~25% of cases of mucormycosis with more than
50% mortality.2 The most frequent mode of
infection is through inhalation of pervasive spores into the bronchioles
and alveoli, which typically results in the rapid development of
pneumonia or endobronchial disease. Risk factors for mucormycosis
include diabetes mellitus (DM), systemic corticosteroid medication,
neutropenia, hematologic malignancies, stem cell transplant, and other
immunocompromised statuses. Among the risk factors, DM is the most
common predisposing factor. Our patient was diabetic however his glucose
level was well controlled with subcutaneous insulin.
In comparison to bacterial infections, fungal infections in people with
cirrhosis are still relatively rare. In cirrhotic patients, infections
caused by Candida, Cryptococcus, Aspergillus, and Coccidioidomycosis
have all been documented.7 Only a few
instances of invasive mucormycosis in these patients have been
documented.8,9 Neutropenia and
thrombocytopenia are frequently present in cirrhotic individuals and may
act as risk factors for fungus infections.
The presentation of pulmonary Mucormycosis might be acute, subacute, or
chronic. Patients with pulmonary mucormycosis commonly present with
fever, cough, dyspnea, and chest pain. Hemoptysis occurs due to vascular
invasion which is usually fatal. Pancoast syndrome, progressive
subcutaneous emphysema, bronchial perforation, chronic mediastinitis, or
Horner’s syndrome are rare presentations of pulmonary
Mucormycosis.10-14
The radiological evidence of pulmonary mucormycosis is mostly
non-specific. An abnormal chest x-ray is found in more than 80% of
patients.15 Radiological presentations
include infiltrates, consolidation, cavitation, single or multiple
nodules or masses, air crescent sign, halo sign, reversed halo sign,
lymphadenopathy, pleural effusion, pulmonary artery pseudoaneurysms, and
bronchopleural fistula. Cavitations are found in as many as 40% of
patients, but air crescent sign is uncommon. The right lung is more
commonly involved than the left, and there is a predilection for the
involvement of the upper lobes, although the reason for this remains
unknown.16,17
Our patient presented with a fever, cough, and breathlessness for 2
weeks. On examination, cervical lymphadenopathy was present with
features suggestive of CLD. Systemic respiratory system examination was
normal and chest X-ray and HRCT scan of chest findings were suggestive
of the cavitary lesion. Though sputum for Gram staining showed
Klebsiella spp. and fungal stain showed budding yeast cells with
pseudo-hyphae. The patient was treated with injectable ceftriaxone and
oral itraconazole for 2 weeks according to the C/S without any
significant improvement.
The gold standard for the diagnosis of pulmonary mucormycosis is the
demonstration of characteristic hyphae (broad, non-septate, ribbon-like
hyphae, with right-angled branching) and histopathologic changes
(vascular invasion with tissue necrosis and neutrophilic infiltration of
the tissue) in a biopsy specimen.18 As our
patient, the intravenous antibiotics and oral antifungal treatment were
ineffective, then cervical lymph node FNAC was performed that showed
non-caseating granuloma. So, for further evaluation, a CT-guided biopsy
from a left-sided cavitary lesion was performed to confirm the
diagnosis. Histopathological specimens of lung tissue showed features
suggestive of Mucormycosis.
Pulmonary mucormycosis is associated with bacterial pneumonia in 30% of
cases, which can delay the diagnosis of the fungal
infection.19 The sensitivity of microscopic
examination of sputum and sputum culture is low, and the false positive
rate is high. Our patient’s sputum examination showed Klebsiella growth
and we treated the patient according to the culture and sensitivity
without any significant improvement. Polyenes (Amphotericin B and
Liposomal amphotericin B) are the first-line agents for the treatment of
mucormycosis, and Liposomal amphotericin B is the choice of treatment as
it improves the response rates and survival compared to amphotericin B.
In the present case, oral posaconazole was used rather than polyenes due
to financial constrain. Posaconazole is metabolized mainly by the liver
through glucuronidation, and it elevates liver function tests by
approximately 1–5 %. No dose adjustment is necessary in patients with
hepatic impairment.4 Our patient received
itraconazole for a short bridging period of 20 days without significant
improvement and then finally posaconazole was started with a strict
follow-up. During follow up patient improved clinically and
radiologically without any documented side effects during the 3 months
of treatment with posaconazole.
There are approximately 18 case reports of mucormycosis with
decompensated CLD. Among them HCV in 8 patients; HBV in 2; autoimmune
hepatitis in 2; alcoholic liver disease in 4; and unknown etiology
2.20 None of them had lymph node involvement.
This may be the first reported case of pulmonary mucormycosis with CLD
with cervical lymph node involvement. The etiology of CLD of our patient
was non-B non-C (NBNC).
The principal limitation of our report is that we did not obtain
definitive culture confirmation of Mucorales from the biopsy material.
However, the culture yield rate for this organism is relatively low, and
as it can be falsely negative in up to 50% of mucormycosis cases. The
low culture yield rate may be the result of the deleterious effects of
tissue grinding, interactions with other organisms, and/or
less-than-optimal culture temperature.21 Different molecular methods are used for the confirmation of
mucormycosis diagnosis. Among them, PCR of Mucorales can be performed on
tissue biopsy and blood samples. They can confirm diagnosis earlier than
the conventional mycological method. Although serum PCR sensitivity is
lower than in tissue, it is highly
specific.22 We couldn’t perform PCR on tissue
or blood samples due to a lack of facilities.