Connective Tissue Diseases and Rheumatology Lecture Review

This set of vocabulary flashcards covers key concepts, diagnostic markers, and clinical manifestations of various connective tissue diseases and rheumatological disorders based on the provided lecture transcript.

Connective tissue diseases (CTDs)

A group of disorders that primarily target structures rich in collagen and elastin, involving loss of immune tolerance and subsequent tissue damage.

Rheumatology

The medical subspecialty dedicated to disorders of the musculoskeletal system and autoimmune and connective tissue diseases.

Systemic Lupus Erythematosus (SLE)

A chronic autoimmune disease characterized by features such as a malar rash, photosensitivity, oral ulcers, serositis, and lupus nephritis.

Lupus nephritis

One of the most serious complications of SLE that directly affects the kidneys.

Rheumatoid arthritis (RA)

A chronic systemic autoimmune disease whose primary pathological target is the synovial membrane lining the joints, often causing symmetric inflammatory arthritis.

Morning stiffness

A classic clinical feature of rheumatoid arthritis that helps differentiate it from osteoarthritis, typically lasting more than $1\,\text{hour}$.

Systemic sclerosis (scleroderma)

A condition characterized by vasculopathy, immune dysregulation, and excessive fibrosis of skin and internal organs.

Raynaud's phenomenon

A common early sign and prominent feature of systemic sclerosis and Mixed Connective Tissue Disease (MCTD) involving vasospasm.

Sjögren's syndrome

An autoimmune disorder characterized by lymphocytic infiltration and dysfunction of the exocrine glands, primarily the salivary and lacrimal glands.

Sicca symptoms

The hallmark symptoms of Sjögren's syndrome, consisting of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).

Dermatomyositis

An inflammatory myopathy characterized by progressive, symmetrical proximal muscle weakness and characteristic cutaneous findings like the heliotrope rash and Gottron's papules.

Polymyositis

An autoimmune condition characterized by progressive, symmetrical weakness of the proximal muscles of the shoulder and hip girdles, without the pathognomonic rashes of dermatomyositis.

Mixed Connective Tissue Disease (MCTD)

An overlap syndrome uniquely characterized by high-titer antibodies against anti-U1 ribonucleoprotein (anti-U1 RNP) and manifestations of SLE, systemic sclerosis, polymyositis/dermatomyositis, and RA.

Antinuclear Antibody (ANA) test

A cornerstone screening tool for CTDs with high sensitivity for SLE ($>95\%$) but low specificity, as it can be positive in healthy individuals.

Anti-dsDNA antibody

An antibody highly specific for SLE whose titer often correlates with disease activity, particularly renal involvement and lupus nephritis.

Anti-CCP (anti-cyclic citrullinated peptide)

The most specific serological marker ($>95\%\text{ specific}$) for predicting erosive disease in patients with Rheumatoid Arthritis.

Anti-SSA (Ro) and anti-SSB (La)

Antibodies associated with Sjögren's syndrome and SLE; they are linked to the risk of congenital heart block in the fetus (neonatal lupus).

Anti-Jo-1 antibody

An antibody associated with antisynthetase syndrome, which includes myositis, interstitial lung disease, Raynaud's, fever, and mechanic's hands.

C-Reactive Protein (CRP) in SLE

A marker that may be normal in active SLE unless there is associated serositis (pleuritis or pericarditis) or concurrent bacterial infection.

High-Resolution Computed Tomography (HRCT)

A critical imaging tool for evaluating pulmonary involvement in CTDs, particularly for detecting interstitial lung disease (ILD).

Schirmer's test

A diagnostic test used to assess lacrimal gland function by measuring tear production, often used in the evaluation of Sjögren's syndrome.

Scleroderma renal crisis

A life-threatening complication of systemic sclerosis characterized by sudden hypertension, severe headache, visual changes, and acute kidney injury.

Pannus

The hyperplastic synovial membrane in rheumatoid arthritis that erodes into cartilage and bone.

Cricoarytenoid arthritis

A laryngeal complication of rheumatoid arthritis that can cause hoarse voice, stridor, and difficulty breathing when lying flat.

Full-house pattern

A characteristic immunofluorescence pattern on renal biopsy for lupus nephritis showing positive staining for IgG, IgA, IgM, C3, and C1q.

Diffuse alveolar hemorrhage (DAH)

A complication in SLE presenting with a triad of hemoptysis, falling hematocrit, and new infiltrates on chest X-ray.

Calcinosis cutis

A painful long-term sequela involving calcium deposits in the skin, most often observed in systemic sclerosis and dermatomyositis.

Nailfold capillaroscopy

An examination tool where megacapillaries and areas of capillary dropout indicate a scleroderma-spectrum disorder like systemic sclerosis or MCTD.

Fibromyalgia

A common non-articular soft tissue condition presenting as widespread pain, fatigue, and tender points, with normal inflammatory and autoantibody labs.

Anti-Smith (anti-Sm) antibody

An antibody highly specific for SLE, though its titer typically does not correlate with renal disease activity or specific disease activity indices.