Sarcoidosis & Vasculitis - Free MCQ Practice Test with solutions, NEET

• Ankylosing spondylitis
• Behcet's disease
• Drug or hypersensitivity reactions
• Inflammatory bowel disease
• Multiple sclerosis
• Psoriatic arthritis
• Reactive arthritis
• Rheumatic fever
• Sarcoidosis
• Sclerosing cholangitis
• Systemic lupus erythematosus
• Vogt-Koyanagi-Harada syndrome

Cardiac involvement begins with inflammation and the formation of granulomas, which is then followed by scarring. This initial inflammation may provoke ventricular arrhythmias, and the resulting scarring creates a substrate for reentrant monomorphic ventricular tachycardia.

• Spontaneous remission is also observed in those with Lofgren's syndrome, which includes:
• Bilateral hilar lymphadenopathy
• Ankle arthritis
• Erythema nodosum
• Fever
• Myalgia
• Weight loss

Lung involvement occurs in 90% of patients with bilateral hilar lymphadenopathy, peribronchial thickening, and reticulo-nodular alterations.

• Non-caseating granulomas are observed in the eye, parotid gland, lymph nodes, liver, and spleen.
• Cutaneous symptoms differ and can include rashes, nodules, erythema nodosum, granuloma annulare, or lupus pernio.

• involvement of cranial nerves
• basilar meningitis
• myelopathy
• anterior hypothalamic disease, which may lead to diabetes insipidus

Tufting of terminal phalanges is observed in conditions such as:

• Hyperparathyroidism
• Acromegaly
• Gigantism

• The non-caseating granulomas can be observed in the heart.
• This represents the primary cause of non-infectious dilated cardiomyopathy.
• The granulomas generate vitamin D, resulting in hypercalcaemia.

Heerfordt syndrome (also known as uveo-parotid fever) is a type of sarcoidosis that includes:

• Fever
• Parotid gland enlargement
• Facial paralysis
• Ocular involvement

Lofgren's syndrome features:

• Erythema nodosum
• Hilar adenopathy visible on chest radiographs
• Uveitis
• Periarticular arthritis

Lofgren's syndrome is linked to a favourable prognosis, with over 90% of patients achieving disease resolution within two years. A recent suggestion to broaden the definition of Lofgren's syndrome includes periarticular arthritis in the absence of erythema nodosum.

Serum amyloid A is raised and serves as a marker for granulomatous inflammation associated with sarcoidosis. The test is infrequently conducted, and no substrate has been accessible since 1996. There are concerns that specific infections, like bovine spongiform encephalopathy, might be transmitted via a Kveim test. The levels of vitamin D₃ are elevated, resulting in hypercalcemia, which is one of the manifestations of sarcoidosis. The X-ray findings are as follows:

• Stage I: solely hilar adenopathy, frequently accompanied by right paratracheal involvement.
• Stage II: a combination of adenopathy and infiltrates.
• Stage III: infiltrates alone.
• Stage IV: fibrosis.

Lupus pernio is the most distinctive skin manifestation of sarcoidosis. The lesion is generally characterised by:

• Red to purple hues, attributed to enhanced blood vessel formation
• Swelling
• Shiny alterations in the skin

These lesions commonly appear on the nose, cheeks, lips, or ears. They are notably difficult to treat with both surgical and medical interventions.

Several organ systems participate, and the clinicopathologic observations indicate the extent to which the pulmonary arteries are affected in PAN, while involvement of the bronchial arteries is rare.

In classic PAN, the renal pathology consists of arteritis without associated glomerulonephritis.

The presumed pathogenic mechanism for Henoch-Schonlein purpura involves the deposition of immune complexes.

• IgA is the antibody class that is most frequently identified in these immune complexes.
• It has been observed in renal biopsies from affected patients.

Several triggering antigens have been proposed, such as:

• infections of the upper respiratory tract,
• various medications,
• certain foods,
• insect stings,
• and vaccinations.

Types of Takayasu arteritis and their involvement include:

• Type 1 - Aortic arch;
• Type 2 - Descending aorta;
• Type 3 - Aortic arch and descending aorta;
• Type 4 - Involvement of the aorta and pulmonary artery.

Takayasu is marked by segmental inflammatory panarteritis, leading to stenosis of the aorta and its branches, which results in weak peripheral pulses. The Mantoux test is notably positive in cases of Takayasu arteritis. Diagnosis is confirmed through angiography. Treatment typically requires long-term immunosuppression with prednisolone and methotrexate.

As all peripheral pulses are detectable, Takayasu's arteritis can be excluded. The occurrence of gangrene in the digits suggests involvement of small blood vessels.
The existence of hypertension further indicates a vasculitis, which is characteristic of polyarteritis nodosa.

• Wegener's granulomatosis and systemic lupus erythematosus (SLE) are dismissed, as ANCA and dsDNA tests return negative results.
• Polyarteritis nodosa (PAN) can impact digital vessels, resulting in gangrene, while the vasculitis aspect accounts for the hypertension.

Wegener's granulomatosis, also known as granulomatosis with angiitis, is marked by:

• Upper respiratory tract issues, such as maxillary sinusitis.
• Lower respiratory tract symptoms, including hemoptysis.
• Kidney involvement, indicated by hematuria.
• Skin manifestations, characterised by purpura.

Clinical characteristics encompass:

• Chronic sinusitis, which is the most frequent initial complaint.
• Pulmonary infiltrates, along with a persistent cough and hemoptysis.
• Myalgias and arthralgias, typically polyarticular and symmetrical, affecting small and medium joints.
• Crescentic necrotizing glomerulonephritis, with urinary sediment revealing more than 5 RBCs per HPF or erythrocyte casts.
• Nervous system manifestations, including mononeuritis multiplex, sensorimotor polyneuropathy, and cranial nerve palsies.
• CNS involvement, featuring vasculitis of small to medium-sized vessels in the brain or spinal cord, along with granulomatous masses.
• Cutaneous symptoms, such as palpable purpura or skin ulcers, occurring in 45% of cases.

Hypercalcemia is seen with sarcoidosis as the granulomas present in this disease synthesize vitamin D3. This increases the amount of absorption of calcium from the intestine and leads to hypercalcemia.

Garland's triad (also known as the 1-2-3 sign or Pawnbrokers sign) is a lymph node enlargement pattern which has been described in sarcoidosis. It comprises of:

1. Right paratracheal nodes
2. Right hilar nodes
3. Left hilar nodes

Synovial cell hyperplasia and endothelial cell activation are early events in the pathologic process that progresses to uncontrolled inflammation and consequent cartilage and bone destruction. Genetic factors and immune system abnormalities contribute to disease propagation.

Non-palpable purpura is a characteristic of acute idiopathic thrombocytopenic purpura.

Giant cell arteritis, also known as cranial arteritis or temporal arteritis, involves inflammation of medium to large arteries. It typically affects one or more branches of the carotid artery, especially the temporal artery. Nonetheless, it is a systemic condition that can impact arteries in various locations, particularly the aorta and its primary branches.

Giant cell arteritis is significantly linked with polymyalgia rheumatica, which manifests as stiffness, discomfort, and pain in the muscles of the neck, shoulders, lower back, hips, and thighs. While polymyalgia rheumatica usually occurs on its own, it is observed in 40-50% of individuals with giant cell arteritis.

• Moreover, 10-20% of patients who initially display signs of isolated polymyalgia rheumatica eventually develop giant cell arteritis.
• This strong clinical relationship, along with findings from pathophysiological studies, has increasingly indicated that giant cell arteritis and polymyalgia rheumatica are different clinical manifestations of a single disease process.

• Vascular lesions in medium-sized muscular arteries primarily occur at bifurcations and branching points.
• Nodules in PAN are typically found on the lower extremities.
• These nodules represent the least frequent skin manifestation of PAN.

• The 'bead' structures are often larger than the normal arterial lumen.
• In some patients with FMD, aneurysms may be present, which could necessitate treatment.

The diagnosis here is established based on the identification of 3 out of 10 ACR criteria for the diagnosis of PAN:

• Hypertension (BP=160/140 mm Hg),
• Kidney involvement without glomerulonephritis (RBC 10-15/HPF, but no casts observed),
• Muscle weakness or myalgia.

The existence of digital gangrene further supports the diagnosis, indicating small vessel involvement, although the peripheral pulses remain palpable.
The diagnostic criteria for PAN are outlined below (3 out of 10 must be present):

• Weight loss of 4 kg or more,
• Testicular pain or tenderness,
• Myalgia or muscle weakness/tenderness,
• Mononeuropathy or polyneuropathy,
• Diastolic blood pressure exceeding 90 mm/Hg,
• Livedo reticularis,
• Elevated blood urea nitrogen (BUN) or creatinine levels not attributed to dehydration or obstruction.
• Kidney involvement results from vasculitis rather than glomerulonephritis; thus, hematuria is present but without RBC casts,
• Presence of hepatitis B surface antigen or antibody in serum,
• Arteriogram showing aneurysms or occlusions of the visceral arteries,
• Biopsy of a small or medium-sized artery containing polymorphonuclear neutrophils.

The acronym FEBRILE helps to recall the criteria, which include:

• Fever
• Enanthem
• Bulbar conjunctivitis
• Rash
• Internal organ involvement (not part of the criteria)
• Lymphadenopathy
• Extremity changes

The diagnostic criteria set by the American Heart Association (AHA) stipulate that fever lasting more than 5 days (which is an absolute criterion) and 4 out of the 5 primary clinical features must be present, provided that diseases with similar symptoms have been ruled out. The 5 key clinical findings are:

• Changes in the peripheral extremities: Initial redness or swelling of the palms and soles, followed by membranous peeling of the tips of fingers and toes or transverse grooves across the fingernails and toenails (Beau lines).
• Oropharyngeal changes: Redness, cracking, and crusting of the lips; strawberry tongue; widespread mucosal injection of the oropharynx.
• Bilateral, non-exudative, painless bulbar conjunctival injection.
• Acute non-purulent cervical lymphadenopathy with lymph node diameter greater than 1.5 cm, typically unilateral.
• Polymorphous rash (not vesicular): Generally widespread but may be confined to the groin or lower extremities.

• Involvement of the common, external, and internal carotid arteries is usually extracranial; however, proximal intracranial segments are rarely affected.
• Intraorbital branches, particularly the posterior ciliary and ophthalmic arteries, are often involved.
• Vertebral arteries are affected as often as the superficial temporal arteries in fatal cases, although involvement of the basilar artery is uncommon.

The pulmonary artery is not implicated in Polyarteritis Nodosa. This condition is a systemic vasculitis marked by necrotising inflammatory lesions that target medium-sized and small muscular arteries, particularly at vessel bifurcations. This leads to:

• Formation of microaneurysms
• Aneurysmal rupture accompanied by haemorrhage
• Thrombosis
• Subsequent organ ischaemia or infarction

Between 20% and 30% of patients with PAN test positive for the Hepatitis B surface antigen.

Microscopic polyangiitis impacts capillaries, venules, and arterioles, along with influencing pulmonary vessels. In comparison, small vessels and pulmonary vessels are not implicated in polyarteritis nodosa. Takayasu affects the aorta and its primary branches. Giant cell arteritis targets cranial vessels, particularly the temporal artery.

Nonspecific headaches, hearing impairment, and tinnitus frequently arise from skull involvement in Paget's disease. The size of the patient's hat may increase (or, less frequently, decrease) due to skull enlargement or deformation. The most prevalent cranial symptom is hearing loss, which occurs in 30-50% of individuals with skull involvement.

• The leading neurological complication is deafness, resulting from the engagement of the petrous temporal bone.
• Hip pain is most common when the acetabulum and proximal femur are affected, particularly during the sclerotic stage.
• Bowing of the femur and long bones, or protrusion of the acetabulum, leads to pain that intensifies with weightbearing and improves with rest.
• Knee and shoulder discomfort may arise due to altered mechanical forces across the joints caused by deformed bones.

Other individuals with Paget's disease may exhibit a range of symptoms linked to complications, which encompass musculoskeletal, neurological, and cardiovascular issues. Due to heightened osteoblastic activity and bone formation, levels of bone-specific alkaline phosphatase (BSAP) are elevated.

Radiographic findings in the chest are irregular in two-thirds of adults with Wegener's granulomatosis (a term not employed) and are referred to as Granulomatosis with angiitis. The predominant radiological observations include:

• Single or multiple nodules and masses.
• Nodules are generally diffuse, with about 50% exhibiting cavitation.

Henoch-Schonlein purpura, also known as anaphylactoid purpura or non-thrombocytopenic purpura, generally manifests between the ages of 4 to 7 years. It is defined by the deposition of immune complexes containing IgA.

• Palpable purpura on the extensor surfaces
• Polyarthralgia
• Colicky abdominal pain accompanied by nausea and vomiting
• Passage of blood per rectum
• Renal glomerulonephritis with proteinuria and microscopic haematuria

These are typical characteristics associated with the condition.