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Questions and Answers
What is the most common manifestation in the CNS for multisystem involvement?
What is the most common manifestation in the CNS for multisystem involvement?
- Neurosarcoidosis
- Acute transverse myelitis
- Bilateral lower motor neuron 7th cranial nerve palsy (correct)
- Basilar meningitis
Chronic tubulo interstitial disease rarely affects the glomerulus.
Chronic tubulo interstitial disease rarely affects the glomerulus.
True (A)
Which imaging technique is considered obsolete for identifying multisystem manifestations?
Which imaging technique is considered obsolete for identifying multisystem manifestations?
Ga-67 scan
Pancytopenia with splenomegaly is primarily a manifestation of _____.
Pancytopenia with splenomegaly is primarily a manifestation of _____.
Match the following investigations with their purposes:
Match the following investigations with their purposes:
Which of the following features is pathognomonic for hypocomplementemic urticarial vasculitis?
Which of the following features is pathognomonic for hypocomplementemic urticarial vasculitis?
Behcet's disease is classified as a single organ vasculitis.
Behcet's disease is classified as a single organ vasculitis.
Name one type of drug associated with small vessel vasculitis.
Name one type of drug associated with small vessel vasculitis.
Infections like _____ can lead to immune complex mediated small vessel vasculitis.
Infections like _____ can lead to immune complex mediated small vessel vasculitis.
Match the following conditions to their associated vasculitis types:
Match the following conditions to their associated vasculitis types:
What is the first line treatment for chronic sarcoidosis?
What is the first line treatment for chronic sarcoidosis?
Patients with Mixed Connective Tissue Disease (MCTD) are predominantly male.
Patients with Mixed Connective Tissue Disease (MCTD) are predominantly male.
What type of antibody is positive in 100% of patients with Sarcoidosis?
What type of antibody is positive in 100% of patients with Sarcoidosis?
The second line treatment for chronic sarcoidosis is __________.
The second line treatment for chronic sarcoidosis is __________.
Match the following autoimmune diseases with their associated characteristics:
Match the following autoimmune diseases with their associated characteristics:
Which of the following patterns is associated with sarcoidosis?
Which of the following patterns is associated with sarcoidosis?
Lupus pernio is characterized by erythematous, indurated plaques commonly observed on the central face.
Lupus pernio is characterized by erythematous, indurated plaques commonly observed on the central face.
Which condition is primarily associated with chronic bilateral panuveitis?
Which condition is primarily associated with chronic bilateral panuveitis?
Sarcoidosis is the only rheumatological disease that notably affects the upper lobe of the lung along with __________.
Sarcoidosis is the only rheumatological disease that notably affects the upper lobe of the lung along with __________.
Match the features with their associated conditions.
Match the features with their associated conditions.
What is a common clinical feature of Giant Cell Arteritis (GCA)?
What is a common clinical feature of Giant Cell Arteritis (GCA)?
Visual loss in GCA can be both temporary and permanent.
Visual loss in GCA can be both temporary and permanent.
What is the primary treatment option for PMR?
What is the primary treatment option for PMR?
Patients with GCA usually exhibit an ESR greater than _____ mm/hr.
Patients with GCA usually exhibit an ESR greater than _____ mm/hr.
Match the following clinical features with their corresponding descriptions related to GCA:
Match the following clinical features with their corresponding descriptions related to GCA:
What is the most common clinical manifestation of 2º Raynaud's phenomenon?
What is the most common clinical manifestation of 2º Raynaud's phenomenon?
Interstitial lung disease (ILD) is the least common complication associated with these conditions.
Interstitial lung disease (ILD) is the least common complication associated with these conditions.
What does nail pitting indicate in rheumatology and immunology?
What does nail pitting indicate in rheumatology and immunology?
Patients with 2º Raynaud's phenomenon may exhibit ______ capillaries in capillaroscopy.
Patients with 2º Raynaud's phenomenon may exhibit ______ capillaries in capillaroscopy.
Match the following complications with their descriptions:
Match the following complications with their descriptions:
Which of the following is NOT an atypical presentation of Giant Cell Arteritis?
Which of the following is NOT an atypical presentation of Giant Cell Arteritis?
Temporal artery biopsy is usually negative in cases where Giant Cell Arteritis is strongly suspected.
Temporal artery biopsy is usually negative in cases where Giant Cell Arteritis is strongly suspected.
What is the first-line treatment for Giant Cell Arteritis?
What is the first-line treatment for Giant Cell Arteritis?
An elevated ESR is typically greater than or equal to _______ mm/hr in the diagnosis of Giant Cell Arteritis.
An elevated ESR is typically greater than or equal to _______ mm/hr in the diagnosis of Giant Cell Arteritis.
Match the following diagnostic tools with their relevance to Giant Cell Arteritis:
Match the following diagnostic tools with their relevance to Giant Cell Arteritis:
What is the primary reason for classifying vasculitis based on vessel size?
What is the primary reason for classifying vasculitis based on vessel size?
Temporal/Giant cell arteritis is classified as a small vessel vasculitis.
Temporal/Giant cell arteritis is classified as a small vessel vasculitis.
Name one medium vessel vasculitis.
Name one medium vessel vasculitis.
The initial process involved in vasculitis includes endothelial injury and subsequent ______ formation.
The initial process involved in vasculitis includes endothelial injury and subsequent ______ formation.
Match the vasculitis with the correct vessel classification:
Match the vasculitis with the correct vessel classification:
Which malignancy is NOT typically associated with malignancy-related vasculitis?
Which malignancy is NOT typically associated with malignancy-related vasculitis?
Giant Cell Arteritis predominantly affects the intra-cranial vessels.
Giant Cell Arteritis predominantly affects the intra-cranial vessels.
What is the mean age of diagnosis for Giant Cell Arteritis?
What is the mean age of diagnosis for Giant Cell Arteritis?
The female to male ratio for Giant Cell Arteritis is _____ to 1.
The female to male ratio for Giant Cell Arteritis is _____ to 1.
Match the following features with their associated categories:
Match the following features with their associated categories:
Which artery is the most commonly involved in Takayasu arteritis?
Which artery is the most commonly involved in Takayasu arteritis?
Constitutional symptoms occur in 50% of cases of Takayasu arteritis.
Constitutional symptoms occur in 50% of cases of Takayasu arteritis.
What is the primary symptom experienced in the upper extremities due to Takayasu arteritis?
What is the primary symptom experienced in the upper extremities due to Takayasu arteritis?
Carotidynia refers to the tenderness of the _____.
Carotidynia refers to the tenderness of the _____.
Match the following clinical signs with their descriptions:
Match the following clinical signs with their descriptions:
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Study Notes
Multisystem Manifestations of Sarcoidosis
- Endocrine: Hypercalcemia
- CNS: Bilateral lower motor neuron (LMN) 7th cranial nerve palsy is the most common neurological manifestation.
- Neurosarcoidosis can lead to pituitary mass and stalk effect, resulting in central diabetes insipidus (DI).
- Other possible CNS involvement includes basilar meningitis and acute transverse myelitis.
- CVS: Dilated cardiomyopathy (CMP) is more common than restrictive CMP.
- AV block may also occur.
- Hematology: Pancytopenia with splenomegaly may be present.
- Liver: Asymptomatic rise in alkaline phosphatase (ALP) and intrahepatic cholestasis may occur.
- Renal:
- Acute kidney injury (AKI) from hypercalcemia induced pre-renal failure.
- Chronic kidney disease (CKD) from chronic tubulo interstitial disease (CTID).
- Glomerular involvement is rare, but may present as membranous nephropathy.
- Rheumatological disorders causing CTID: Sjogren's syndrome, 1964 related disease, and sarcoidosis.
- Investigations:
- Gallium 67 scintigraphy
- PET scan to identify nodes for biopsy
- Endobronchial ultrasound (EBUS) and transbronchial biopsy for maximizing yield.
- Blood markers: Angiotensin-converting enzyme (ACE) and soluble interleukin-2 (sIL-2) receptor.
- Bronchoalveolar lavage (BAL): CD4/CD8 ratio greater than 2 is suggestive of sarcoidosis. Normal ratio rules out sarcoidosis.
- A Ga-67 scan, sometimes referred to as the "Panda sign," is obsolete.
Vasculitis
- Hypocomplementemic urticarial vasculitis:
- Associated with anti-C1q antibody.
- May overlap with features of systemic lupus erythematosus (SLE).
- Interface dermatitis is a pathognomonic feature.
- Variable vessel vasculitis:
- Behcet's disease and Cogan syndrome.
- Single organ vasculitis:
- Skin: Cutaneous leukocytoclastic angiitis (capillaries, post-capillary venules) and cutaneous polyarteritis nodosa (PAN) (arterioles).
- CNS: Primary CNS vasculitis.
- Aorta: Isolated aortitis.
- Secondary vasculitis:
- Associated with connective tissue diseases: Immune complex mediated small vessel vasculitis, except for IgG4-related diseases (aortitis), SLE (mesenteric artery, CNS vessels), rheumatoid arthritis (RA), Sjogren's, sarcoidosis, ankylosing spondylitis (large vessel vasculitis).
- Drugs: Small vessel vasculitis associated with penicillins, sulfonamides, and thiazides.
- Infections:
- Rickettsial infections: Immune complex mediated small vessel vasculitis
- Tuberculosis (TB)
- Syphilis
- Hepatitis B virus (HBV): PAN-like
- Hepatitis C virus (HCV): Cryoglobulinemia-like
- ANCA associated vasculitis: May be associated with hydralazine and propylthiouracil.
Fibrosis pattern of Sarcoidosis
- Non-specific interstitial pneumonia (NSIP) of the upper lobe is the most common pattern.
- Thickening of bronchovascular bundles.
- Traction bronchiectasis of the upper lobe is a feature.
Skin Involvement in Sarcoidosis
- Lupus pernio:
- Violaceous or erythematous, indurated, infiltrative plaques.
- Usually located on the central face.
- Less responsive to treatment.
- Associated with bone changes like lytic or cystic joint involvement in sarcoidosis.
Eye Involvement in Sarcoidosis
- Chronic bilateral panuveitis is the most common eye manifestation.
Features Unlikely of Sarcoidosis
- Pulmonary: Pleural involvement, usual interstitial pneumonia (UIP) like pattern (honeycomb, destruction of lung parenchyma).
- Ocular: Episcleritis, scleritis, and sicca.
Treatment of Sarcoidosis
- Chronic:
- First-line: Steroids
- Second-line: Methotrexate
- Third-line: Anti-TNF-α (Adalimumab)
- Acute: No specific treatment.
Therapeutic Paradox of Sarcoidosis
- In patients without sarcoidosis, treatment with TNF-α can lead to the development of skin lesions similar to sarcoidosis. These lesions typically disappear with a reduction in the TNF-α dose.
Overlap Syndrome
- Features of two or more autoimmune systemic connective tissue diseases (CTD): Sjogren's, Systemic Lupus Erythematosus (SLE), Polymyositis (Pm), Dermatomyositis (Dm), Systemic Sclerosis (SSC), and Rheumatoid Arthritis (RA).
- Treatment: Based on the dominant disease.
- Undifferentiated CTD: Patients presenting with some features of CTD but do not meet criteria for classification under a specific disease.
Mixed Connective Tissue Disease (MCTD)
- A specific type of overlap syndrome.
- Unique features and middle-aged females (female: male = 15:1).
Serology in MCTD
- ANA positive in 100%: Coarse speckled pattern.
- Anti-U1 RNP antibody: Positive.
Polymyalgia Rheumatica (PMR)
- Clinical Features:
- Tendinitis, bursitis, and enthesitis.
- Early morning pain and stiffness in the shoulders, hips, and pelvic girdle.
- Elevated ESR (250 mm/hr).
- Pathology:
- IL-6 predominant: Inflammation type.
- IFN-γ mediated: Ischemia type.
- May represent late onset RA.
- Treatment : Low-dose steroids (10 mg/day).
Clinical Features of Giant Cell Arteritis (GCA)
- Headache: New onset, moderate-severe intensity, recurring, unusual type, boring pain.
- ESR: Greater than 100 mm/hr (ACR: >50 mm/hr).
- Visual Loss/Visual Field Abnormalities: Unilateral/bilateral, temporary/permanent, altitudinal hemianopia.
- Associated with AION (Acute Ischemic Optic Neuropathy) which causes pallor of the optic disc, swollen optic nerve head, and dilated tortuous vessels.
- Vessels: Nodular/thickened, decreased pulsations, tenderness. Involved in 50% of cases.
- Jaw Claudication: Due to maxillary artery involvement, especially when accompanied by ophthalmoplegia. 100% positive predictive value for GCA.
- Constitutional Symptoms: Fever, fatigue, weight loss, malaise.
- Associations: Smoking and Varicella zoster virus (VZV) isolation from biopsy. No role of acyclovir. Genetic predisposition (HLA DRBI-04, MICA allele).
Atypical Presentations of Giant Cell Arteritis (GCA)
- Pyrexia of unknown origin.
- Cervical radiculopathy.
- Recurrent throat pain and cough.
Diagnosis of GCA
- Increased ESR and CRP: May be low in 10% of cases (IFN-γ excess).
- Increased IL-6.
- Temporal Artery Biopsy:
- 4-6 cm long segment to overcome skip lesions.
- Repeat biopsy if negative in a case of strong suspicion.
- Color Doppler:
- 70% sensitivity and 80% specificity.
- Halo Sign: Hypoechoic area around the temporal artery in longitudinal and transverse sections.
American College of Rheumatology (ACR) Criteria for GCA Diagnosis
- Age of onset ≥ 50 years.
- New onset or new type of localized headache.
- Temporal artery tenderness to palpation or decreased pulsations.
- ESR ≥ 50 mm/hr.
- Biopsy: Transmural inflammation.
Treatment of GCA
- Steroids: First-line treatment.
- Dosage: 40-60 mg/day (1 mg/kg/day), tapered down.
- Tocilizumab (Anti - IL6):
- Indication: For flares (common at low-dose steroids).
Vasculitis Classification and Large Vessel Vasculitis
- Vasculitis: Inflammation within the vessel wall leading to ischemia and organ damage.
- No gold standard test or diagnostic criteria.
- Vasculopathy: Examples includes systemic sclerosis (SSc). Endothelial injury leads to thrombus formation, capillary hypoxia, myofibroblast activation, TGFβ, and fibrosis.
- ESR and CRP are low.
- Revised Chappell Hill Consensus 2012 Classification:
- Based on predominantly affected vessel size.
- High specificity and low sensitivity.
- Vessel Size and Associated Diseases:
- Large Vessel: Temporal/Giant cell arteritis (most common), Takayasu arteritis.
- Medium Vessel: Polyarteritis nodosa (PAN), Kawasaki disease.
- Small Vessel:
- ANCA associated: Granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), Allergic/Eosinophilic granulomatosis with polyangiitis (AGPA/EGPA), Microscopic Polyangiitis (MPA).
- Immune complex–mediated (further examples): Anti-glomerular basement membrane (GBM) disease (Goodpasture syndrome), Henoch-Schönlein purpura (HSP), Cryoglobulinemia associated vasculitis.
Takayasu Arteritis
-
Synonyms: Aortic arch syndrome, non-specific aortoarteritis, occlusive thrombo aortopathy.
-
Age of Presentation: Aneurysm.
-
Vessels Involved:
- Subclavian artery (most common).
- Aorta (30% of GCA cases).
- Renal artery (renal artery stenosis; accelerated hypertension).
- Pulmonary artery.
- Coronary artery.
-
Clinical Features:
- Constitutional symptoms: Rare (20% cases).
- Claudication pain of the upper extremity (most common symptom).
- Cardiac: Symptoms of aortic regurgitation.
-
Signs:
- Carotidynia (tenderness of carotids).
- Pulse: Inequality.
- BP: Unequal arm blood pressure (>10 mmHg difference between right and left upper limbs).
- Bruits: Abdominal/carotid/aortic/femoral.
Malignancy Related Vasculitis/ Secondary Paraneoplastic Vasculitis
- Small vessels are involved.
- Associated malignancies: Lymphoma, Hairy cell leukemia, bladder cancer (Ca bladder), and renal cell carcinoma (RCC).
Large Vessel Vasculitis
- Similar histology in Takayasu arteritis and Giant Cell Arteritis (GCA):
- Transmural inflammation (involves tunica intima, media, adventitia).
- Cells involved: Lymphocytes, macrophages.
- Granuloma.
- Few giant cells.
- Seen in 50% of cases.
Giant Cell Arteritis (GCA)
- Synonyms: Temporal arteritis, cranial arteritis, granulomatous arteritis.
- Ethnic groups involved: Scandinavians, Northern Europeans.
- Vessels involved: Extracranial vessels with internal elastic lamina (sparing intracranial vessels).
- Most common artery involved: Superficial temporal > vertebral > ophthalmic > posterior ciliary.
- Age of presentation: > 50 years.
- Mean age of diagnosis: 72 years.
- Female to male ratio (F:M): 2:1.
GCA-PMR Syndrome
- 50/20 rule:
- Polymyalgia rheumatica (PMR) symptoms in 50% of GCA cases.
- GCA symptoms in 20% of PMR cases.
Raynaud's Phenomenon (RP)
- 2º RP: Most common manifestation.
- Edema of Hands: Swelling in the hands.
- Nail Pitting: Pitting of the nails.
- Acrosclerosis: Acrosclerosis occurs with RP, MCTD, and SSc.
- Myositis: Muscle inflammation.
- Synovitis: Erosive inflammation of the synovial membranes.
- CNS and Renal System: Usually spared.
Capillaroscopy in Raynaud's Phenomenon (RP)
- Normal Capillaries: Image of normal capillaries.
- Dilated Capillaries: Image of dilated capillaries.
- Tortuous Capillaries and Drop-out: Image of tortuous capillaries and drop-out.
- Microhemorrhage: Image of microhemorrhage.
- Bushy Capillaries: Image of bushy capillaries
- Meandering Capillaries: Image of meandering capillaries.
HLA Association in Raynaud's Phenomenon (RP) & Systemic Sclerosis (SSc)
- HLA DRB04.
Complications of Systemic Sclerosis (SSc)
- **ILD (most common) ** Interstitial lung disease (most common).
- 20% evolve into limited SSc and Pulmonary Artery Hypertension (PAH, most common cause of death): 20% of cases transition to limited systemic sclerosis (SSC) and pulmonary arterial hypertension (PAH), a major cause of mortality.
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