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What is a granuloma?
The word ‘Granuloma’ takes origin from the word ‘granule’ meaning well circumscribed lesion and ‘-oma’ denoting localized macrophage laden inflammatory mass (1) It is a focal collection of mononuclear cell rich inflammatory growth (2) Granulomatous means any process in the body that causes this granuloma to form (3) Histologically they are congregate of inflammatory mononuclear cells or modified macrophages surrounded by a rim of circulating immune complexes and other biological mediators (4) They are formed to protect the host calls from constant inflammation related stimuli that if unchecked can cause destructive effects (5)
What are granulomatous disorders of the nose and paranasal sinuses?
Granulomatous disorders of the head and neck region frequently involve the nose and Paranasal sinuses. Trauma, inflammation, autoimmunity, substance use or infections can lead to granulomatous disorders in the nose and sinuses (6)
Infective granulomas:
Bacteria (tuberculosis, non-tuberculous mycobacterial infection, scleroma, leprosy, syphilis and actinomycosis) or Fungi (Rhinosporidiosis, histoplasmosis, cryptococcosis, blastomycosis and paracoccidioidomycosis).
Protozoal (Leishmaniasis)
Non-infective granulomas:
Wegener’s granulomatosis, Churg Strauss syndrome, Sarcoidosis
Neoplastic: T cell lymphomas
What kind of symptoms and signs can one have to denote a granulomatous disease?
They present with symptoms that are non-specific such as nasal discharge, stuffy nose, nasal obstruction, nasal deformity, epistaxis, crusting, ulceration, or mass. As these disorders progress they tend to involve the adjoining structures such as palate, orbit, and even the skull base (7). They tend to present like neoplastic disorders with malformations and degenerative lesions of the nose (8)
How will my doctor diagnose these diseases?
Thorough clinical examination including endoscopic study along with radiological and other investigations specific to the clinical suspicion is carried out. Biopsy of the lesion followed by histological study not only helps in the diagnosis but also rules out malignant counterparts of these conditions (if any). Serological testing specific to certain disorders are also a part of the workup. Due to their nonspecific symptoms and mimic of many other conditions, granulomatous disorders pose a significant clinical challenge for management in a timely and appropriate manner.
Most of these conditions originate at the nasal septum and spread across to the back of the nose (Nasopharynx) and Inferior meatus as these are the areas and, in that order, are most exposed to the external environment.
Imaging using Sinus CT specially in patients with severe involvement such as epistaxis or eye involvement.MRI may be required when there is spread to the Central nervous system. (9)
What are the different types of granulomatous disorders?
Rhinoscleroma: caused by klebsiella rhinoscleromatis. Commonly seen in southern India,sri lanka & Pakistan with male predominant population. Spread through infected cattle or contaminated ponds/cattle. Severe involvement of nose& nasopharynx with friable strawberry appearing mass may be seen. Atypical granuloma with Mikulicz cells noted on histopathology
Aspergillosis: caused by Aspergillus niger/flavis. Seen in bird handlers and captives especially when there is history of compromised immunity or long term steroid treatment. Grayish nasal mucosal false membrane can be seen in the patients and spread of infection to sinus cavities is common. It can be diagnosed by fungal staining and growth tests and 1% gentian violet application for treatment with Amphotericin for severe infections is the gold standard treatment.
Mucormycosis: Fungal pathogenic infection of the nose and paranasal sinuses most commonly seen in immune compromise and was seen in surge post the coronavirus pandemic.Surgical debridement with systemic and long term antifungal therapy is the mainstay of management.
Churg strauss syndrome: Eosinophillic granuloma with asthma and hypereosinophilia make up the necrotizing vasculitis disorder that present in one third of all cases as nasal symproms.. Antinuclear cytoplasmic antibodies (ANCA) presence can be used as a positive indicator for diagnosis.
Wegener’s granulomatosis: Autoimmune necrotising granulomas of the nose, paranasal sinuses and lower respiratory tract with focal glomerulonephritis. c-ANCA or p-ANCA is diagnostic. Immunosuppressive therapy and supportive immunoglobulin are used for management with reconstructive surgeries as and when necessary.
Do all granulomatous diseases require surgical correction?
In early stages, with small lesions, medical management may suffice. Cosmetic corrections may be done in small lesions. Grossly large lesions may require excision with skin grafting or extensive surgical resection if the sinus spaces are involved.
What should I do if I or someone I know has one of these granulomatous disorders?
Awareness regarding the condition with seeking early consultation are key to appropriate management. Medical regimens in early conditions can reduce the disease burden significantly as with time surgical corrections of severe deformity become difficult. For recurrence management, follow ups are essential.
The team of experts at Bangalore Head and Neck Sciences have a thorough comprehensive approach to management of these conditions and have successfully dealt with these cases in their clinical journey. If you or anyone you know have the above set of features or symptoms, seek an appointment at the earliest or reach out to us by any means and we will guide them to a healthy disease-free outcome.
REFERENCES
(1) Mohan H. “Textbook of Pathology”. Sixth Edition. New delhi: Jaypee Brothers Medical Publishers (2015).
(2) James D G. “A clinicopathological classification of granulomatous disorders”. Postgraduate Medicine 76 (2000): 457-465.
(3) Nwawka O K., et al. “Granulomatous Disease in the Head and Neck: Developing a Differential Diagnosis”. 34.5 (2014): 1240- 1256.
(4) Mohanty D., et al. “A Clinico-Pathological Study of Granulomas of the Nose”. Indian Journal of Applied Research 4.5 (2014): 499-502
(5) Hughes R and Drake-Lee A. “Nasal manifestations of granulomatous disease”. Hospital Medicine 62.7 (2001): 417- 421.
(6) Fuchs H and Tanner S. “Granulomatous disorders of the nose and paranasal sinuses”. Current Opinion in Otolaryngology and Head and Neck Surgery 17.1 (2009): 23-27.
(7) N. Cereceda-Monteoliva, M.J. Rouhani, E.F. Maughan, A. Rotman, N. Orban, C. Al Yaghchi, G. Sandhu. Sarcoidosis of the ear, nose and throat: a review of the literature. Clin. Otolaryngol., 46 (2021), pp. 935-940
(8) A. Muller, B. Krause, A. Kerstein-Stahle, S. Comduhr, S. Klapa, S. Ullrich, K. Holl-Ulrich, P. Lamprecht. Granulomatous inflammation in ANCA-associated vasculitis Int. J. Mol. Sci., 22 (2021)
(9) Scadding GK, Durham SR, Mirakian R, et al. BSACI guidelines for the management of rhinosinusitis and nasal polyposis. Clin Exp Allergy 2007; 38:260–275.