GPA and Sinusitis Flashcards
(44 cards)
What is GPA?
Granulomatosis with polyangiitis (GPA) is a rare form of vasculitis.
What is the pathophysiology of GPA?
It is thought to be an autoimmune inflammatory process affecting endothelial cells.
It is a multisystem disease which can affect many parts of the body, categorised by the ELK classification: it most commonly presents with lesions in the upper respiratory tract (E indicating ears/nose/throat, almost 100%), lungs (L most patients) and kidneys (K >75%).
Many other areas of the body may also be affected, with joint inflammation occurring in 25-50% of all cases. The sinuses, eyes and skin may also be affected
What are the risk factors for GPA?
GPA has been linked to parvovirus and to chronic nasal carriage of Staphylococcus aureus.
What are the symptoms of GPA?
As a multisystem disease, GPA often presents with nonspecific symptoms and can be difficult to recognise in primary care.
Symptoms may include:
- Fatigue, malaise, abdominal pain and joint pains
- Fever, night sweats
- Weakness, loss of appetite and weight loss
- Rhinorrhoea, sinusitis and hearing loss
- Facial pain
- Hoarseness, cough, dyspnoea, wheezing and chest pain
What are the signs seen in GPA?
The signs found in GPA occur as a result of the inflammation of the small vessels and may affect any part of the body.
The most commonly seen signs relate to the upper and lower airways and the renal tract and may include:
- Ulcers, sores and crusting, in and around the nose, with destruction of nasal cartilage.
- Rhinorrhoea, often bloody.
- Haemoptysis.
- Haematuria.
- Subglottic stenosis (38% in one study) - causing hoarseness, stridor, dyspnoea, or cough.
- Rashes (up to 50%) - often small red/purple raised areas or blister-like lesions, ulcers or nodules
- Conjunctivitis, scleritis and episcleritis.
- Chronic ear infections.
- Mononeuritis multiplex.
- Peritonitis.
What are the differentials of GPA?
Anti-GBM disease Legionella infection SLE Rheumatoid arthritis Churg-Strauss syndrome Renal vein thrombosis with PE
What are the investigations done for GPA?
FBC, ESR.
Serum U&Es.
Blood test for ANCA, of two types: c-ANCA and p-ANCA - detectable in nearly all patients with active severe GPA. However, approximately 1 of 5 patients with active limited disease negative.
Urinalysis for protein, blood and casts.
Nasal endoscopy.
Lung function tests and flow volume loop looking for subglottic stenosis.
CXR looking for cavity formation and pulmonary infiltrates.
Chest CT imaging to exclude lung parenchymal involvement.
Sinus CT scan to exclude sinus disease.
Biopsy of affected tissue, which may include nasal mucosa, lung biopsy, renal biopsy, looking for presence of vasculitis and granulomas.
What is the management of GPA?
Patients who are asymptomatic and have no organ damage may not need immunosuppressive treatment.
Such patients should initially be offered methotrexate to induce remission (with mycophenolate mofetil as an alternative for those intolerant of methotrexate).
Cyclophosphamide should be offered to promote remission in patients who have life-threatening and/or vital organ damage. Due to its serious adverse effects (e.g., renal, haematological and neurological toxicity), it is normally given as pulsed treatment intravenously every 2-4 weeks. Long-term toxicity is dependent on lifetime cumulative dose which should be ≤25 g.
Rituximab if cyclophosphamide is CI,
Prednisolone is given in addition to cyclophosphamide or rituximab, as it helps to increase patient survival and suppress local disease.
Once the patient is in remission, cyclophosphamide should be replaced by azathioprine or methotrexate.
When is surgical treatment indicated for GPA?
Nasal deformity. Subglottic stenosis. Obstruction of lacrimal ducts. Bronchial stenoses. Eustachian dysfunction (insertion of grommets). Acute kidney injury (renal transplant).
What are the complications of GPA?
Acute kidney injury
Respiratory failure
Chronic conjunctivitis
Nasal septum perforation
What are the paranasal sinuses?
The paranasal sinuses refer to the frontal, maxillary, sphenoidal and ethmoidal sinuses.
What is sinusitis?
Sinusitis is an inflammation of the membranous lining of one or more of the sinuses.
Sinusitis is also referred to as rhinosinusitis because inflammation of the nasal mucosa generally accompanies sinusitis.
What is the classification of sinusitis?
Acute: an infection lasting 7-30 days.
Subacute: the inflammation lasts 4-12 weeks.
Recurring: there are >3 significant acute episodes in a year lasting ≥10 days with no intervening symptoms.
Chronic: symptoms persist for >90 days (these may be caused by irreversible changes in the mucosal lining of the sinuses), with or without acute exacerbations.
How can you differentiate between viral and bacterial sinusitis?
Viral disease is said to last less than 10 days, whereas worsening symptoms after 5 days, or symptoms extending beyond 10 days, suggest bacterial infection.
What are the risk factors for sinusitis?
Upper respiratory tract infection.
Allergies and asthma.
Smoking and hormonal status (e.g., pregnancy).
Nasal dryness and DM
Presence of a foreign body and inhalation of irritants (e.g., cocaine).
Iatrogenic (e.g., nasogastric tubes, mechanical ventilation).
Dental problems (e.g., trauma, infection).
Some sporting activities (e.g., swimming, diving, high-altitude climbing).
Mechanical obstruction (e.g., normal anatomical variations, nasal polyps).
Previous history of trauma (nose, cheeks).
Immunocompromise.
What are the rare causes of sinusitis?
Rare causes include cystic fibrosis, neoplasia, as a part of Samter’s triad, sarcoidosis, granulomatosis with polyangiitis (GPA) and immotile cilia syndrome. Sinus surgery can also predispose individuals.
What is the Samter’s triad?
(aspirin sensitivity, rhinitis, asthma)
How do you assess for sinusitis?
In general practice, the most helpful examination technique is simple palpation, as this is quick and easy to perform. Percussion and transillumination are also described, although these are not reliable and diagnosis should not rely on these alone.
Examination of the sinuses should be complemented by assessment of the nose (external and speculum examination) to assess for evidence of related pathology. Thereafter, investigations are guided by clinical suspicion.
What is acute sinusitis?
This is defined as a bacterial or viral infection of the sinuses lasting fewer than four weeks and resolving completely with the appropriate management.
It tends to arise as a result of a viral infection and a diagnosis of acute sinusitis is made if there is sinus drainage obstruction and subsequent secondary bacterial infection.
Are there specific symptoms for acute sinusitis?
No specific clinical symptom or sign is sensitive or specific for acute sinusitis, so the overall clinical impression should be used to guide management.
What is the aetiology of acute sinusitis?
It is commonly caused by Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis.
The latter is more common in children. In adult patients with suspected acute maxillary sinusitis following a viral upper respiratory infection, about one half have been found to have pus or mucopus in the sinus aspirate, and one third had bacterial pathogens growing in culture
What are the symptoms of sinusitis?
Non-resolving cold (>1 week or worsening symptoms over 4-5 days) with a biphasic character: initial viral infection which begins to settle is followed by further malaise related to the sinusitis.
Pain over the affected sinusitis
Pyrexia
Purulent nasal discharge with or without anosmia
How do you diagnose acute sinusitis?
Acute sinusitis is diagnosed if there is:
- Facial discomfort (eg, a feeling of congestion or fullness, often unilateral and worse when bending forwards) or pain.
- Nasal obstruction or (purulent) nasal discharge or postnasal drip.
- Decreased or absent sense of smell.
This may be accompanied by:
-Headache, halitosis, fatigue, dental pain, cough and a feeling of pressure or fullness in the ears.
In children, symptoms of rhinitis predominate ± the additional feature of ear discomfort due to the blockage of the Eustachian tube.
What are the investigations done for acute sinusitis?
Not generally require and investigations usually not helpful.
Possibilities in case of real diagnostic uncertainty include ESR, CRP (although they lack sensitivity), plain X-ray films, ultrasonography, nasendoscopy, CT imaging, MRI scan and sinus puncture.
